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PubMed - Adrenal Cancer (100 unread)

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Detection of bronchioloalveolar cancer by means of PET/CT and 18F-fluorocholine, and comparison with 18F-fluorodeoxyglucose.

Fetched: February 15th, 2010, 7:36am CST

Detection of bronchioloalveolar cancer by means of PET/CT and 18F-fluorocholine, and comparison with 18F-fluorodeoxyglucose.

Nucl Med Commun. 2010 Feb 6;

Authors: Balogova S, Huchet V, Kerrou K, Nataf V, Gutman F, Antoine M, Ruppert AM, Prignon A, Lavolée A, Montravers F, Mayaud C, Cadranel J, Talbot JN

AIM: Bronchioloalveolar (BAC) cancer is a source of false-negative F-fluorodeoxyglucose (FDG) PET/computed tomography (CT) results. A few studies reported better diagnostic performances with PET tracers of lipid metabolism, C-choline, or C-acetate, for the detection of well-differentiated adenocarcinoma or BAC. F-fluorocholine (FCH) is a lipid analogue for PET imaging, with advantages in terms of logistics and image resolution. We carried out this prospective pilot study to evaluate whether FCH PET/CT could detect lung cancer with a BAC component and could be more sensitive than FDG in this aim. METHODS: Fifteen patients with a lung nodule or lesion suspected for BAC on CT and/or with a history of BAC had PET/CT 60-90 min after 5 MBq FDG/kg body mass and, on a separate day, 10-20 min after 4 MBq FCH/kg body mass. The standard of truth was histology and a 6-month follow-up. RESULTS: Nine patients (12 lesions) presented BAC or adenocarcinoma with BAC features, two patients presented adenocarcinoma without BAC features (five lesions) and four patients presented benign lesions (15 non-malignant sites). For both FCH and FDG, patient-based sensitivity was 78% for detecting cancer with a BAC component and 82% for detecting malignancy. Site-based sensitivity for detecting malignancy was 76 and 75% for detecting cancer with BAC features, for both radiopharmaceuticals. Specificity was similar for FCH and FDG (site-based 93 vs. 81%, NS). In these early-stage cancers, only one adrenal metastasis was observed that took up FCH and FDG. CONCLUSION: In this population of patients with ground-glass opacities selected on CT suggestive of BAC or with a history of BAC and a recent lung anomaly on CT, FCH detected all malignant lesions with at least a 2.0 cm short axis. However, FDG had similar performance.

PMID: 20145579 [PubMed - as supplied by publisher]

Multiple Endocrine Neoplasms and Serous Cysts of the Pancreas in a Patient with von Hippel-Lindau Disease.

Fetched: February 15th, 2010, 7:36am CST

Multiple Endocrine Neoplasms and Serous Cysts of the Pancreas in a Patient with von Hippel-Lindau Disease.

J Gastrointest Cancer. 2010 Feb 6;

Authors: Matsubayashi H, Uesaka K, Kanemoto H, Sugiura T, Mizuno T, Sasaki K, Ono H, Hruban R

INTRODUCTION: Patients with the von Hippel-Lindau (VHL) syndrome have an increased risk of developing pancreatic endocrine neoplasms. CASE REPORT: A 40-year-old female with a past history of bilateral adrenal pheochromocytomas, whose brother had a history of a malignant pheochromocytoma, was referred to our institution with symptoms of adrenal crisis including general fatigue and insomnia in spite of increasing predonisolone therapy. Screening by ultrasonogram and computed tomography demonstrated multiple well-demarcated, enhancing nodules (maximum, 3 cm) in the head and tail of pancreas and multiple cystic lesions throughout the entire pancreas. Although this patient met the criteria for VHL, magnetic resonance imaging for her brain did not reveal any lesions. Pancreatoduodenectomy with partial distal pancreatectomy was performed. Histologic examination of the resected pancreas revealed multiple well-differentiated endocrine neoplasms accompanied with multiple serous cysts. The patient remains free of disease 5 years after surgery.

PMID: 20140653 [PubMed - as supplied by publisher]

Bone-specific growth inhibition of prostate cancer metastasis by atrasentan.

Fetched: February 15th, 2010, 7:36am CST

Bone-specific growth inhibition of prostate cancer metastasis by atrasentan.

Cancer Biol Ther. 2010 Apr 4;9(8)

Authors: Drake JM, Danke JR, Henry MD

Advanced prostate cancer frequently metastasizes to bone and remains an incurable disease. One recent experimental therapy involves endothelin receptor A (ETA) antagonists (e.g., atrasentan). Clinical results to date have been mixed, with atrasentan not meeting its primary endpoints in a Phase III trial. It remains an open question whether some patients might benefit from this therapy, while others may not. Preclinical data supports the concept that the endothelin signaling axis may be particularly important for tumor growth in bone, but the extent to which it is involved in metastatic colonization of other organ sites in prostate cancer remains unclear. Here we evaluate the efficacy of atrasentan in a mouse model of prostate cancer metastatic colonization. Using bioluminescence imaging, we show that atrasentan does inhibit tumor growth in, but not the initial colonization of, bony sites in intracardially-injected 22Rv1 prostate cancer cells. However, atrasentan shows little efficacy in soft tissues such as adrenal gland or liver. Our studies show that whether atrasentan exhibits significant overall anti-tumor efficacy and survival benefit depends on the presence of bone metastasis evident in that animal. Though in contrast to previous findings, efficacy is apparent in a prostate tumor model that elicits mixed osteoblastic/osteolytic lesions. These data confirm the notion that atrasentan may exhibit selective activity against prostate cancer bone metastasis, mirroring clinical findings, and suggest that the role of endothelin signaling in bone metastasis may be more complex than previously appreciated. The model described here may provide a valuable tool for unraveling this complexity.

PMID: 20139704 [PubMed - as supplied by publisher]

Retrospective Evaluation of the Outcome of Open Versus Laparoscopic Adrenalectomy for Stage I and II Adrenocortical Cancer.

Fetched: February 15th, 2010, 7:36am CST

Retrospective Evaluation of the Outcome of Open Versus Laparoscopic Adrenalectomy for Stage I and II Adrenocortical Cancer.

Eur Urol. 2010 Jan 26;

Authors: Porpiglia F, Fiori C, Daffara F, Zaggia B, Bollito E, Volante M, Berruti A, Terzolo M

BACKGROUND: Although there is consensus that laparoscopy is the standard of care for the resection of benign adrenal tumours, there is controversy regarding the role of laparoscopy for the resection of adrenocortical cancer (ACC). OBJECTIVE: The aim of the present study was to review the ACC database of the San Luigi Hospital to compare the oncologic effectiveness of open adrenalectomy (OA) versus laparoscopic adrenalectomy (LA) in the treatment of patients with stage I and II ACC. DESIGN, SETTING, AND PARTICIPANTS: We performed a retrospective analysis involving 43 patients with stage I and II ACC who had undergone radical surgery. INTERVENTION: The patients were stratified into two groups according to the surgical procedure. The "open group" consisted of patients treated with OA; the "lap group" consisted of patients treated with LA. MEASUREMENTS: Oncologic effectiveness of the procedures was tested comparing the recurrence-free survival of patients treated with OA versus LA. Secondary outcome measures were differences in terms of type of recurrence and overall survival. RESULTS AND LIMITATIONS: The open group consisted of 25 patients and the lap group of 18 patients. The two groups were comparable in terms of demographic data. The median follow-up was 38 mo in the open group and 30 mo in the lap group. Recurrence rate was 64% in the open group and 50% in the lap group. The median recurrence-free survival was 18 mo in the open group and 23 in the lap group (p=0.8). No differences in terms of pattern of recurrences were recorded. During follow-up, 28% of the open group patients and 5% of the lap group patients died. No differences in terms of survival time were noted (p=0.3). CONCLUSIONS: The present findings provide interesting evidence that OA and LA may be comparable in terms of recurrence-free survival for patients with stage I and II ACC when the principles of surgical oncology are respected.

PMID: 20137850 [PubMed - as supplied by publisher]

Metastatic prostatic adenocarcinoma mimicking inflammatory breast carcinoma: a case report.

Fetched: February 15th, 2010, 7:36am CST

Metastatic prostatic adenocarcinoma mimicking inflammatory breast carcinoma: a case report.

Clin Breast Cancer. 2010 Feb 1;10(1):E3-5

Authors: Njiaju UO, Truica CI

Prostate adenocarcinoma can manifest as a fairly indolent tumor or as a very aggressive cancer with significant invasive and metastatic potential. Common metastatic sites include bone, liver, lymph nodes, and adrenal glands. Dermatologic manifestations are rare. We present a case of a man who presented with breast skin changes that mimicked inflammatory breast carcinoma with specialized testing ultimately giving a diagnosis of metastatic prostatic adenocarcinoma. A 78-year-old man presented with left breast redness and swelling. Examination revealed an erythematous rash with subcutaneous edema over the left hemithoracic area. A breast ultrasound showed no focal mass, and a breast core biopsy had no evidence of tumor. A skin biopsy showed metastatic carcinoma in dermal lymphatics, and the tumor was found to have no estrogen or progesterone receptors or HER2 expression. Computed tomography scans, positron emission tomography, and a nuclear bone scan revealed widespread skeletal metastases. The patient received a 3-month course of capecitabine and cyclophosphamide with no improvement in his skin lesions. Subsequent immunohistochemical staining on the tumor specimen was positive for prostate-specific antigen (PSA) and alpha-methyl-CoA-racemase, confirming a diagnosis of metastatic prostatic adenocarcinoma. He received leuprolide and bicalutamide and demonstrated significant improvement with near-complete resolution of his skin lesions and a decrease in his PSA level. Prostatic adenocarcinoma presenting initially as a breast malignancy is a rarely recognizable clinical event. Undoubtedly, increased awareness and recognition of the rare entity described herein will allow for the prompt initiation of specific therapies, which might be of benefit to many patients.

PMID: 20133250 [PubMed - in process]

Adrenal pheochromocytoma with contralateral adrenocortical adenoma in a cat.

Fetched: February 15th, 2010, 7:36am CST

Adrenal pheochromocytoma with contralateral adrenocortical adenoma in a cat.

J Am Anim Hosp Assoc. 2010 Jan-Feb;46(1):36-42

Authors: Calsyn JD, Green RA, Davis GJ, Reilly CM

A 7-year-old, neutered male cat was presented with a 6-month history of progressive polyuria, polydipsia, polyphagia, aggression, and weight gain. Previous blood work, urinalysis, and radiographs did not delineate a cause for the clinical signs. An ultrasound revealed bilateral adrenal gland enlargement. A low-dose dexamethasone suppression test was consistent with hyperadrenocorticism. Based on these findings, bilateral adrenalectomy was attempted and successfully performed. Histopathology was consistent with a cortical adenoma in the right adrenal gland and a pheochromocytoma in the left adrenal gland. This association has never been reported in the cat.

PMID: 20045835 [PubMed - indexed for MEDLINE]

Primary choriocarcinoma of the liver: a clinicopathological study of five cases in males.

Fetched: February 15th, 2010, 7:36am CST

Primary choriocarcinoma of the liver: a clinicopathological study of five cases in males.

Virchows Arch. 2010 Jan;456(1):65-70

Authors: Shi H, Cao D, Wei L, Sun L, Guo A

Primary choriocarcinomas of the liver are rare. Previous reported cases were mostly in infants with only rare adult cases. Here, we presented five adult cases. The patients were all males, with an average age of 41.6 years (from 36 to 48 years). Clinical presentations included right upper abdominal pain or abdominal distension. All patients presented with a large hepatic mass on ultrasonography that measured 11 cm on average in the greatest diameter. Elevated serum human chorionic gonadotropin (HCG) levels were noted in all cases. At presentation, the tumor was confined to the liver in two patients and therefore surgically resected. The other three patients presented with extrahepatic metastases on imaging study and therefore only received chemotherapy. All five patients died from the tumor within 2 to 8 months. Autopsy was performed for all five cases. The autopsy confirmed that the choriocarcinoma was confined to the liver in two surgically resected cases. The other three patients had metastatic choriocarcinoma in the lung (two patients), peritoneum (one patient), adrenal glands (one patient), and brain (one patient). None of the patients had any evidence of a testicular tumor or scar after examination of the entirely submitted testes. No tumor was observed in central nervous system, mediastinum, or other organs other than described above. Grossly, the primary tumors were large, soft, hemorrhagic, and with foci of necrosis. Histologically, the tumors were composed of mononucleated trophoblastic cells with round nuclei, clear cytoplasm, and prominent nucleoli admixed with large, multinucleated syncytiotrophoblastic cells. Immunohistochemically, tumor cells were strongly positive for keratin, HCG, and focally positive for human placental lactogen. Ki-67 proliferation index was high (mean 75%) in the mononucleated trophoblastic cells. Our series is the largest one to document primary hepatic choriocarcinoma in adults. Although these tumors are rare, they behave in a very aggressive fashion.

PMID: 20013345 [PubMed - indexed for MEDLINE]

Telomere biology in neuroblastoma: telomere binding proteins and alternative strengthening of telomeres.

Fetched: February 15th, 2010, 7:36am CST

Telomere biology in neuroblastoma: telomere binding proteins and alternative strengthening of telomeres.

J Pediatr Surg. 2009 Dec;44(12):2258-66

Authors: Onitake Y, Hiyama E, Kamei N, Yamaoka H, Sueda T, Hiyama K

PURPOSE: Neuroblastoma (NBL) shows remarkable biologic heterogeneity, resulting in favorable or unfavorable prognoses. Previously, we reported that most unfavorable NBLs express high telomerase activity to maintain telomere length. Recently, telomere binding proteins (TBPs) and alternative lengthening of telomeres (ALTs) have been identified as key factors of telomere maintenance. METHODS: To evaluate the correlation between telomerase activity, telomere length, and the expression levels of TBPs in NBL, we analyzed and quantified these factors in 121 untreated NBLs. RESULTS: Shortened and elongated telomeres were detected in 21 (17.3%) and 11 cases (9.0%), respectively, and there was a significant correlation between telomere length and the length of the 3'-overhang. The tumors with shortened or elongated telomeres showed significant lower expression of TBPs, except for RAP1. Although telomerase activity did not correlate with telomere length, 16 of 22 cases with high telomerase activity and 5 of 9 cases (ALT tumors) that showed long telomeres without high telomerase activity resulted in death. High-dose chemotherapy did not have much effect on these deceased ALT cases, but their survival periods were more than 2 years and relatively long compared with the deceased cases with nonelongated telomeres, suggesting that chemoresistance in ALT tumors may be related to slow growth rates. CONCLUSIONS: High telomerase activity is a poor prognostic factor in NBL. In the cases without high telomerase activity, those with elongated telomere also showed poor outcomes because of chemoresistance. Therefore, ALT and TBPs may be biomarkers for chemosensitivity in NBL. Thus, a better understanding of telomere biology may help define the characteristics of individual NBLs.

PMID: 20006006 [PubMed - indexed for MEDLINE]

Risks and benefits of ending of mass screening for neuroblastoma at 6 months of age in Japan.

Fetched: February 15th, 2010, 7:36am CST

Risks and benefits of ending of mass screening for neuroblastoma at 6 months of age in Japan.

J Pediatr Surg. 2009 Dec;44(12):2253-7

Authors: Tajiri T, Souzaki R, Kinoshita Y, Tanaka S, Koga Y, Suminoe A, Matsuzaki A, Hara T, Taguchi T

PURPOSE: The mass screening (MS) for neuroblastoma (NB) at 6 months of age in Japan was discontinued in 2004. This study assessed the risks and benefits of MS based on an analysis of NB detected before or after discontinuation of MS in Japan. METHODS: The clinical features and Brodeur's genetic type based on MYCN, DNA ploidy, and other genetic aberrations were assessed in 113 NB patients (20 cases after and 93 cases [55 MS cases] before the discontinuation of MS) older than 6 months treated at one institution since 1985. RESULTS: The 20 patients with NBs detected after MS was discontinued ranged in age from 7 to 67 months, 12 patients were stage 4, and 11 patients would have been detected at 6 months of age if they had undergone MS. The Brodeur's genetic type of these 20 patients showed that 30% (6/20) were type 1 (low risk), 55% (11/20) were type 2A (intermediate risk), and 15% (3/20) were type 2B (high risk). Of 93 patients with NB detected before MS was discontinued, 60% (56/93) were type 1, 18% (17/93) were type 2A, and 22% (20/93) were type 2B. Among the type 2A patients, 82% (9/11) of the patients detected after MS was discontinued showed stage 4, whereas only 50% (9/18) of those diagnosed before MS was discontinued were stage 4. The genetic analysis using single nucleotide polymorphism (SNP) array for type 2A showed that the pattern of genetic aberration was equivalent in those detected either before or after MS was discontinued. CONCLUSIONS: There was a decrease of type 1 and an increase of type 2A NB in patients after MS was discontinued in Japan. These results suggest that most of the type 1 detected by MS has regressed, and most of the type 2A detected by MS has appeared sporadically as advanced NB in patients older than 1 year.

PMID: 20006005 [PubMed - indexed for MEDLINE]

Corrosive injury to the GI tract in adults: a practical approach.

Fetched: February 15th, 2010, 7:36am CST

Corrosive injury to the GI tract in adults: a practical approach.

Expert Rev Gastroenterol Hepatol. 2009 Dec;3(6):701-9

Authors: Atiq M, Kibria RE, Dang S, Patel DH, Ali SA, Beck G, Aduli F

Corrosive injury to the GI tract still poses great challenges with regards to the initial evaluation triage, as well as the optimization of medical management. Although relatively uncommon in the adult population, these injuries can cause significant morbidity and serious sequelae of complications, such as esophageal strictures and cancer. Prompt recognition of the process and aggressive measures towards the stabilization of the patient are key to a favorable outcome.

PMID: 19929589 [PubMed - indexed for MEDLINE]

Simultaneous analysis of neuroendocrine tumor markers by HPLC-electrochemical detection.

Fetched: February 15th, 2010, 7:36am CST

Simultaneous analysis of neuroendocrine tumor markers by HPLC-electrochemical detection.

J Chromatogr B Analyt Technol Biomed Life Sci. 2009 Dec 15;877(32):4140-6

Authors: Manickum T

A validated, high pressure liquid chromatographic (HPLC) method for simultaneous quantitation of urinary catecholic acids 4-hydroxy-3-methoxymandelic acid (HMMA) (vanylmandelic acid) (VMA), 4-hydroxy-3-methoxyphenylacetic acid (HVA) and 5-hydroxyindole-3-acetic acid (5-HIAA) was developed. Sample preparation involved liquid-liquid extraction of acidified urine, containing iso-HMMA (IS) as internal standard, with ether, evaporation of the organic extract, followed by reconstitution of the residue in phosphate buffer at pH 3.3. After reversed-phase HPLC at 35 degrees C and separation on a Licrospher 125mmx4mm C(18) column (5microm particle size) with phosphate buffer (pH 3.5)-acetone (950:50, v/v) as eluent, quantitation is achieved by electrochemical detection using coulometric detection at a potential of +350mV. The method was successfully applied to routine diagnosis of neuroblastoma, carcinoid syndrome and pheochromocytoma.

PMID: 19926540 [PubMed - indexed for MEDLINE]

ACTH-independent Cushing's syndrome with bilateral micronodular adrenal hyperplasia and ectopic adrenocortical adenoma.

Fetched: February 15th, 2010, 7:36am CST

ACTH-independent Cushing's syndrome with bilateral micronodular adrenal hyperplasia and ectopic adrenocortical adenoma.

J Clin Endocrinol Metab. 2010 Jan;95(1):18-24

Authors: Louiset E, Gobet F, Libé R, Horvath A, Renouf S, Cariou J, Rothenbuhler A, Bertherat J, Clauser E, Grise P, Stratakis CA, Kuhn JM, Lefebvre H

CONTEXT: Bilateral micronodular adrenal hyperplasia and ectopic adrenocortical adenoma are two rare causes of ACTH-independent Cushing's syndrome. OBJECTIVE: The aim of the study was to evaluate a 35-yr-old woman with ACTH-independent hypercortisolism associated with both micronodular adrenal hyperplasia and ectopic pararenal adrenocortical adenoma. DESIGN AND SETTING: In vivo and in vitro studies were performed in a University Hospital Department and academic research laboratories. INTERVENTION: Mutations of the PRKAR1A, PDE8B, and PDE11A genes were searched for in leukocytes and adrenocortical tissues. The ability of adrenal and adenoma tissues to synthesize cortisol was investigated by immunohistochemistry, quantitative PCR, and/or cell culture studies. MAIN OUTCOME MEASURE: Detection of 17alpha-hydroxylase and 21-hydroxylase immunoreactivities, quantification of CYP11B1 mRNA in adrenal and adenoma tissues, and measurement of cortisol levels in supernatants by radioimmunological assays were the main outcomes. RESULTS: Histological examination of the adrenals revealed nonpigmented micronodular cortical hyperplasia associated with relative atrophy of internodular cortex. No genomic and/or somatic adrenal mutations of the PRKAR1A, PDE8B, and PDE11A genes were detected. 17alpha-Hydroxylase and 21-hydroxylase immunoreactivities as well as CYP11B1 mRNA were detected in adrenal and adenoma tissues. ACTH and dexamethasone activated cortisol secretion from adenoma cells. The stimulatory action of dexamethasone was mediated by a nongenomic effect involving the protein kinase A pathway. CONCLUSION: This case suggests that unknown molecular defects can favor both micronodular adrenal hyperplasia and ectopic adrenocortical adenoma associated with Cushing's syndrome.

PMID: 19915020 [PubMed - indexed for MEDLINE]

Pathogenicity of DNA variants and double mutations in multiple endocrine neoplasia type 2 and von Hippel-Lindau syndrome.

Fetched: February 15th, 2010, 7:36am CST

Pathogenicity of DNA variants and double mutations in multiple endocrine neoplasia type 2 and von Hippel-Lindau syndrome.

J Clin Endocrinol Metab. 2010 Jan;95(1):308-13

Authors: Erlic Z, Hoffmann MM, Sullivan M, Franke G, Peczkowska M, Harsch I, Schott M, Gabbert HE, Valimäki M, Preuss SF, Hasse-Lazar K, Waligorski D, Robledo M, Januszewicz A, Eng C, Neumann HP

CONTEXT: Cancer genetics is fundamental for preventive medicine, in particular in pheochromocytoma-associated syndromes. Variants in two susceptibility genes, SDHC and RET, were found in a kindred with head and neck paraganglioma. This observation of coincident DNA variants, both reported as pathogenic, in two known susceptibility genes prompted the question of their pathogenic relevance. OBJECTIVE: Our objective was to elucidate the pathogenic role of the detected variants and study the prevalence of such variants. PATIENTS: Patients were registrants from the European-American Pheochromocytoma-Paraganglioma and German von Hippel-Lindau Disease Registries. DESIGN: Analysis of germline mutation screening results for all pheochromocytoma-paraganglioma susceptibility genes, including RET [multiple endocrine neoplasia type 2 (MEN 2)] and VHL [von Hippel-Lindau disease (VHL)]. Cases in which more than one DNA variant was found were clinically reevaluated, and cosegregation of the disease with the variant was analyzed within the registrants' families. A total of 1000 controls were screened for the presence of detected variants, and in silico analyses were performed. RESULTS: Three variants were identified, RET p.Tyr791Phe and p.Ser649Leu and VHL p.Pro81Ser. The frequencies of RET p.Ser649Leu (0.07%) and p.Tyr791Phe (0.9%) compared with controls excluded the two variants' role in the etiology of MEN 2 and VHL. None of the carriers of the RET variants who underwent prophylactic thyroidectomy showed medullary thyroid carcinoma. Clinical reinvestigation of 18 variant carriers excluded MEN 2. VHL variant p.Pro81Ser, also previously described as a mutation, did not segregate with the VHL in one family. In silico analyses for these variants predicted unmodified protein function. CONCLUSIONS: RET p.Tyr791Phe and p.Ser649Leu and VHL p.Pro81Ser are definitely not pathogenic mutations for VHL and MEN 2. Misinterpretation results in irreversible clinical consequences.

PMID: 19906784 [PubMed - indexed for MEDLINE]

Low sensitivity of glucagon provocative testing for diagnosis of pheochromocytoma.

Fetched: February 15th, 2010, 7:36am CST

Low sensitivity of glucagon provocative testing for diagnosis of pheochromocytoma.

J Clin Endocrinol Metab. 2010 Jan;95(1):238-45

Authors: Lenders JW, Pacak K, Huynh TT, Sharabi Y, Mannelli M, Bratslavsky G, Goldstein DS, Bornstein SR, Eisenhofer G

CONTEXT: Pheochromocytomas can usually be confirmed or excluded using currently available biochemical tests of catecholamine excess. Follow-up tests are, nevertheless, often required to distinguish false-positive from true-positive results. The glucagon stimulation test represents one such test; its diagnostic utility is, however, unclear. OBJECTIVE: The aim of the study was to determine the diagnostic power of the glucagon test to exclude or confirm pheochromocytoma. DESIGN, SETTING, AND SUBJECTS: Glucagon stimulation tests were carried out at three specialist referral centers in 64 patients with pheochromocytoma, 38 patients in whom the tumor was excluded, and in a reference group of 36 healthy volunteers. MAIN OUTCOME MEASURES: Plasma concentrations of norepinephrine and epinephrine were measured before and after glucagon administration. Several absolute and relative test criteria were used for calculating diagnostic sensitivity and specificity. Expression of the glucagon receptor was examined in pheochromocytoma tumor tissue from a subset of patients. Results: Larger than 3-fold increases in plasma norepinephrine after glucagon strongly predicted the presence of a pheochromocytoma (100% specificity and positive predictive value). However, irrespective of the various criteria examined, glucagon-provoked increases in plasma catecholamines revealed the presence of the tumor in less than 50% of affected patients. Diagnostic sensitivity was particularly low in patients with pheochromocytomas due to von Hippel-Lindau syndrome. Tumors from these patients showed no significant expression of the glucagon receptor. CONCLUSION: The glucagon stimulation test offers insufficient diagnostic sensitivity for reliable exclusion or confirmation of pheochromocytoma. Because of this and the risk of hypertensive complications, the test should be abandoned in routine clinical practice.

PMID: 19897672 [PubMed - indexed for MEDLINE]

Permalink for 'The glucocorticoid-induced leucine zipper gene (GILZ) expression decreases after successful treatment of patients with endogenous Cushing's syndrome and may play a role in glucocorticoid-induced osteoporosis.'

The glucocorticoid-induced leucine zipper gene (GILZ) expression decreases after successful treatment of patients with endogenous Cushing's syndrome and may play a role in glucocorticoid-induced osteoporosis.

Fetched: February 15th, 2010, 7:36am CST

The glucocorticoid-induced leucine zipper gene (GILZ) expression decreases after successful treatment of patients with endogenous Cushing's syndrome and may play a role in glucocorticoid-induced osteoporosis.

J Clin Endocrinol Metab. 2010 Jan;95(1):246-55

Authors: Lekva T, Bollerslev J, Kristo C, Olstad OK, Ueland T, Jemtland R

CONTEXT: Glucocorticoid-induced bone loss is a serious complication in patients with endogenous Cushing's syndrome. However, the mechanism(s) by which excess glucocorticoids influence bone metabolism is not completely understood. OBJECTIVE: The aim of the study was to investigate the functional role of glucocorticoid-induced leucine zipper (GILZ) in bone remodeling with special focus on glucocorticoid-induced osteoporosis (GIO). PATIENTS: Nine patients with endogenous Cushing's syndrome participated in the study. RESEARCH DESIGN AND METHODS: We analyzed bone biopsies from Cushing's patients before and after treatment to screen for expressional candidate genes with putative roles in GIO. Microarray analysis combined with real-time RT-PCR revealed that the gene encoding GILZ ranked among the topmost regulated genes and was selected for functional characterization in vitro. RESULTS: GILZ mRNA was expressed by human fetal osteoblasts (hFOB), human mesenchymal stem cells (hMSC), osteoblasts differentiated from hMSC, and osteoclasts. GILZ was increased by dexamethasone in a time- and dose-dependent manner in hFOB. Inhibition of GILZ in hFOB cells by small interfering RNA decreased typical osteoblast-related genes, suggesting a physiological role in promoting osteoblast maturation. Our data further support a functional role for GILZ in normal bone remodeling by modulating expression of TNF-(ligand) receptor superfamily/osteoprotegerin in favor of increased ratio in hFOB. Finally, osteoclasts exposed to conditioned media from GILZ-silenced hFOB indicated effects on osteoclast activity. CONCLUSION: Taken together, these results implicate the transcription factor GILZ in the pathophysiology of GIO by regulating osteoblast maturation and bone turnover.

PMID: 19875485 [PubMed - indexed for MEDLINE]

Intra-renal adrenal adenoma: a compelling addition to the differential diagnosis of renal mass.

Fetched: February 15th, 2010, 7:36am CST

Intra-renal adrenal adenoma: a compelling addition to the differential diagnosis of renal mass.

Int J Urol. 2009 Nov;16(11):912-4

Authors: Linder B, Hong Y, Jarrett T

We report a case of an adrenal rest arising in the kidney of a 37-year-old male. An incidental 4.5 cm right upper pole mass with a minimal amount of fat was discovered on computed tomography scan. Laparoscopic renal exploration and biopsy revealed angiomyolipoma on frozen section. However, final pathological analysis of the partial nephrectomy specimen revealed intra-renal adrenal adenoma. The diagnosis was confirmed by immunohistochemistries and transmission electron microscopy. We review the reported literature on intra-renal adrenal adenoma and highlight the aspects that raise the index of suspicion for this entity on the differential diagnosis of renal masses.

PMID: 19863627 [PubMed - indexed for MEDLINE]

Permalink for 'Limited prognostic value of the 2004 International Union Against Cancer staging classification for adrenocortical carcinoma: proposal for a revised TNM classification.'

Limited prognostic value of the 2004 International Union Against Cancer staging classification for adrenocortical carcinoma: proposal for a revised TNM classification.

Fetched: February 15th, 2010, 7:36am CST

Limited prognostic value of the 2004 International Union Against Cancer staging classification for adrenocortical carcinoma: proposal for a revised TNM classification.

Cancer. 2009 Dec 15;115(24):5847; author reply 5848

Authors: Grubbs E, Lee JE

PMID: 19827149 [PubMed - indexed for MEDLINE]

Differential diagnosis and laparoscopic treatment of adrenal pheochromocytoma and ganglioneuroma.

Fetched: February 15th, 2010, 7:36am CST

Differential diagnosis and laparoscopic treatment of adrenal pheochromocytoma and ganglioneuroma.

Chin Med J (Engl). 2009 Aug 5;122(15):1790-3

Authors: Shi BB, Li HZ, Chen C, Rong S, Fan H, Wen J, Li HJ

BACKGROUND: Adrenal ganglioneuroma is a rare adrenal pathogenic disease with difficult differential diagnosis from adrenal pheochromocytoma. Currently, very limited literature is available to allow a differential diagnosis of these two conditions from each other. This study aimed to evaluate the clinical profile, differential diagnosis and surgical treatments of both conditions. METHODS: Clinical characteristics of 36 patients with adrenal pheocheomocytoma and 18 patients with adrenal ganglioneuroma were analyzed. Data from CT scans and surgical treatments from 1999 to 2007 were collected. Endocrine hormone tests and (131)I-metaiodobenzylguanidine (MIBG) were performed. Neither (131)I-MIBG nor endocrine hormone tests were available in 9 cases of asymptomatic adrenal ganglioneuroma with tumor size less than 4 cm and there were negative findings from contrast enhanced CT scans. The level of urine catecholamine of patients was compared by one-way analysis of variance. RESULTS: Te mean age of patients in the adrenal ganglioneuroma group was 41.2 years (16 - 67 year) and in the adrenal pheochromocytoma patients 38 years (17 - 74 year). Contrast enhanced CT showed that the foci were intensified in 5 cases (27.8%) of adrenal ganglioneuroma and there were obvious contrast indications in 30 (83.3%) of the pheochromocytoma. Catecholamine levels in a 24-hour urine sample were normal in 4 patients with adrenal ganglioneuroma and increased in 36 (100%) cases with adrenal pheochromocytoma. (131)I-MIBG nuclear scan showed negative results in 4 patients (100%) with adrenal ganglioneuroma and positive results in 25 (96.2%) with adrenal pheochromocytom. Laparoscopy for adrenal tumors was performed through a transperitoneal or retroperitoneal approach during a follow-up period of (43 +/- 6) months, and all cases survived well. CONCLUSIONS: CT, urinary catecholamine and (131)I-MIBG are standard and efficient tools for differential diagnosis of adrenal ganglioneuroma from pheochromocytoma. Laparoscopic surgery can be performed through a transperitoneal or retroperitoneal approach depending on the finding of CT scans. Open surgery is necessary for patients with blood loss of more than 800 ml and violent fluctuation of intraoperative blood pressure.

PMID: 19781327 [PubMed - indexed for MEDLINE]

Spontaneous intraperitoneal bleeding caused by adrenal pheochromocytoma.

Fetched: February 15th, 2010, 7:36am CST

Spontaneous intraperitoneal bleeding caused by adrenal pheochromocytoma.

Chin Med J (Engl). 2009 Sep 20;122(18):2193-5

Authors: Li C, Xu YM

PMID: 19781311 [PubMed - indexed for MEDLINE]

Piggyback liver transplant techniques in the surgical management of urological tumors with inferior vena cava tumor thrombus.

Fetched: February 15th, 2010, 7:36am CST

Piggyback liver transplant techniques in the surgical management of urological tumors with inferior vena cava tumor thrombus.

Chin Med J (Engl). 2009 Sep 20;122(18):2155-8

Authors: Ji ZG, Xue C, Li HZ, Wang HJ, Xie Y, Liu GH

BACKGROUND: An important characteristic of renal cell carcinomas and adrenal tumors is that these tumors may expand into the renal vein and inferior vena cava, and transform into tumor thrombi. This study was to evaluate the use of piggyback liver transplant techniques for surgical management of urological tumors with inferior vena cava tumor thrombus. METHODS: Nineteen patients with renal cell carcinomas or adrenal tumors with inferior vena cava tumor thrombus were treated from November 1995 to April 2008. Their ages ranged from 29 years to 76 years (mean 54 years). The extent of tumor thrombus was infrahepatic (level I) in 2, retrohepatic (level II) in 7, suprahepatic (level III) in 6, and intra-atrial (level IV) in 4 patients. We used cardiopulmonary bypass with deep hypothermic circulatory arrest to remove the thrombi in 3 cases of level IV and in 2 cases of level III. In all level II, 4 level III, and 2 level IV cases, we used piggyback liver transplant techniques to mobilize the liver off of the inferior vena cava and to separate the inferior vena cava from the posterior abdominal wall. RESULTS: Mean operative time was 5.1 hours, mean estimated blood loss was 2289 ml and mean blood transfusion was 12.84 U. One patient with adrenal cortical carcinoma and level IV thrombus died in the immediate postoperative period. Three patients were lost to follow up, and the other 15 survivors were followed from 5 months to 56 months. Eight of these 15 patients died due to metastasis; however 7 were still alive at the last follow-up. CONCLUSIONS: An aggressive surgical approach is the only hope for curing patients diagnosed with urological tumors combined with inferior vena cava tumor thrombus. The use of piggyback liver transplant techniques to mobilize the liver off of the inferior vena cava provides excellent exposure of the inferior vena cava. Patients with a level II or level III inferior vena cava thrombus may be treated without using cardiopulmonary bypass.

PMID: 19781302 [PubMed - indexed for MEDLINE]

[Alcohol and the liver]

Fetched: February 15th, 2010, 7:36am CST

[Alcohol and the liver]

Gastroenterol Clin Biol. 2009 Aug-Sep;33(8-9):840-9

Authors: Mathurin P

The risk of developing alcohol-induced liver injury occurs above a risk threshold around 25-30g/day. A dose-dependent relationship between alcohol intake and the risk of developing alcohol-induced liver injury is observed in alcoholic subjects with a daily consumption above this threshold. Cirrhosis is the first cause of death related to alcohol. Cirrhosis occurs more frequently in heavy drinkers disclosing alcoholic hepatitis or steatosis. Excess weight is a risk factor for alcoholic liver disease. The role of alcohol in mouth, oropharynx and oesophageal cancers is clearly established. The evidence for the role of alcohol in breast cancer has become clear. Corticosteroids improved the short-term survival of patients with severe alcoholic hepatitis. The Lille model identifies patients who will not benefit from corticosteroids. The benefit of liver transplantation is restricted to alcoholic patients with severe cirrhosis. Only abstainers are considered as candidates for liver transplantation. The recent development of non invasive methods opens up new perspectives for the screening of cirrhosis in subjects with heavy drinking.

PMID: 19729258 [PubMed - indexed for MEDLINE]

$Permalink for 'Safety, tolerability, and pharmacokinetics of the anti-IGF-1R monoclonal antibody figitumumab in patients with refractory adrenocortical carcinoma.'$

Safety, tolerability, and pharmacokinetics of the anti-IGF-1R monoclonal antibody figitumumab in patients with refractory adrenocortical carcinoma.

Fetched: February 15th, 2010, 7:36am CST

Safety, tolerability, and pharmacokinetics of the anti-IGF-1R monoclonal antibody figitumumab in patients with refractory adrenocortical carcinoma.

Cancer Chemother Pharmacol. 2010 Mar;65(4):765-73

Authors: Haluska P, Worden F, Olmos D, Yin D, Schteingart D, Batzel GN, Paccagnella ML, de Bono JS, Gualberto A, Hammer GD

PURPOSE: Insulin-like growth factor 1 receptor signaling through upregulation of the stimulatory ligand IGF-II has been implicated in the pathogenesis of adrenocortical carcinoma. As there is a paucity of effective therapies, this dose expansion cohort of a phase 1 study was undertaken to determine the safety, tolerability, pharmacokinetics, and effects on endocrine markers of figitumumab in patients with adrenocortical carcinoma. METHODS: Figitumumab was administered on day 1 of each 21-day cycle at the maximal feasible dose (20 mg/kg) to a cohort of patients with metastatic, refractory adrenocortical carcinoma. Serum glucose, insulin, and growth hormone were measured pre-study, at cycle 4 and study end. Pharmacokinetic evaluation was performed during cycles 1 and 4. RESULTS: Fourteen patients with adrenocortical carcinoma received 50 cycles of figitumumab at the 20 mg/kg. Treatment-related toxicities were generally mild and included hyperglycemia, nausea, fatigue, and anorexia. Single episodes of grade 4 hyperuricemia, proteinuria, and elevated gamma-glutamyltransferase were observed. Pharmacokinetics of figitumumab was comparable to patients with solid tumors other than adrenocortical carcinoma. Treatment with figitumumab increased serum insulin and growth hormone levels. Eight of 14 patients (57%) had stable disease. CONCLUSIONS: The side effect profile and pharmacokinetics of figitumumab were similar in patients with adrenocortical carcinoma in comparison to patients with other solid tumors. While hyperglycemia was the most common adverse event, no clear patterns predicting severity were observed. The majority of patients receiving protocol therapy with single agent figitumumab experienced stability of disease, warranting further evaluation.

PMID: 19649631 [PubMed - indexed for MEDLINE]

Histologic and immunohistochemical characterization of pheochromocytoma in 6 cotton-top tamarins (Saguinus oedipus).

Fetched: February 15th, 2010, 7:36am CST

Histologic and immunohistochemical characterization of pheochromocytoma in 6 cotton-top tamarins (Saguinus oedipus).

Vet Pathol. 2009 Nov;46(6):1221-9

Authors: Miller AD, Masek-Hammerman K, Dalecki K, Mansfield KG, Westmoreland SV

Pheochromocytomas are uncommon neoplasms of the adrenal medulla that are most frequently reported in rats and select mouse strains. In many cases, especially those in man, pheochromocytoma is associated with familial tumor syndromes, because of inherited mutations in a variety of proto-oncogenes and tumor suppressor genes. Nonhuman primates are valuable animal models for a variety of human diseases because of their similar anatomy and physiology; however, cases of pheochromocytomas have only rarely been reported in nonhuman primates. Herein, we characterize the gross, histologic, and immunohistochemical features of pheochromocytoma in 6 cotton-top tamarins (Saguinus oedipus). Pheochromocytomas represented 6 of 114 of the total causes of death in the studied population (5.3%) and corresponded to 16% of the total number of neoplasms. The average age of affected animals was 17.9 years. On histologic examination, all cases were defined by tight bundles, nests, and cords of neoplastic chromaffin cells. All cases had concurrent myocardial degeneration and fibrosis of varying severity and chronicity. Three of the cases (50%) also had hyalinization and medial thickening of coronary arteries consistent with hypertension. On immunohistochemical examination, 6 of 6 of the cases (100%) stained positively for chromogranin A, synaptophysin, N-CAM (or CD56), and protein gene product 9.5. None of the cases stained for glial fibrillary acidic protein. Pedigree analysis revealed inter-relatedness in 4 of 6 animals, with progenitor animals also affected with pheochromocytomas. The tumors in this population illustrate comparable histologic and immunohistochemical staining patterns with cases in other laboratory animals and humans, and, therefore, may indicate common underlying genetic alterations that precipitate tumor development.

PMID: 19605896 [PubMed - indexed for MEDLINE]

Comparison of laparoscopic adrenalectomy with open surgery for adrenal tumors.

Fetched: February 15th, 2010, 7:36am CST

Comparison of laparoscopic adrenalectomy with open surgery for adrenal tumors.

Kaohsiung J Med Sci. 2009 Aug;25(8):438-44

Authors: Wang HS, Li CC, Chou YH, Wang CJ, Wu WJ, Huang CH

The role of laparoscopy in the management of adrenal tumors is well established. However, there are very few head-to-head comparisons between laparoscopic and open methods at the same institution. We retrospectively evaluated the operative and postoperative parameters of laparoscopic adrenalectomy for adrenal tumors and compared the results with those of traditional open adrenalectomy. Eighty-eight patients with adrenal tumors underwent adrenalectomy between January 1997 and October 2008 at our institute. Clinical data were retrospectively collected after assigning the patients into Group I (n = 51), who underwent the laparoscopic method, and Group II (n = 37), who underwent the traditional open method, by reviewing the patients' charts and related data. Patients in Group I experienced significantly less blood loss (88.6 +/- 93.0 mL vs. 321.4 +/- 265.4 mL, p < 0.01), shorter hospital stay (6.7 +/- 4.3 days vs. 11.3 +/- 5.4 days, p < 0.01) and earlier oral intake (1.5 +/- 0.6 days vs. 2.2 +/- 0.8 days, p < 0.01) postoperatively. In Group I, eight patients had adrenal tumors larger than 6 cm and no statistically significant differences were found compared with the other patients in Group I. Two patients in Group I had malignancies and no local or port site recurrence was found at regular follow-up. There was no mortality in either group. Laparoscopic adrenalectomy is a safe, effective and minimally invasive approach with the advantages of better cosmesis, less blood loss, shorter hospital stay and more rapid recovery. We recommend that laparoscopic adrenalectomy is considered as the gold standard procedure for adrenal tumors, irrespective of whether the tumor is benign or malignant.

PMID: 19605338 [PubMed - indexed for MEDLINE]

Constitutive {beta}-catenin activation induces adrenal hyperplasia and promotes adrenal cancer development.

Fetched: January 31st, 2010, 3:49pm CST

Constitutive {beta}-catenin activation induces adrenal hyperplasia and promotes adrenal cancer development.

Hum Mol Genet. 2010 Jan 27;

Authors: Berthon A, Sahut-Barnola I, Lambert-Langlais S, de Joussineau C, Damon-Soubeyrand C, Louiset E, Taketo MM, Bertherat J, Lefrançois-Martinez AM, Martinez A, Val P

Adrenocortical carcinoma is a rare but aggressive cancer with unknown aetiology. Constitutive activation of beta-catenin is the most frequent alteration in benign and malignant adrenocortical tumours in patients. Here, we show that constitutive activation of beta-catenin in the adrenal cortex of transgenic mice resulted in progressive steroidogenic and undifferentiated spindle-shaped cells hyperplasia as well as dysplasia of the cortex and medulla. Over a 17 months time course, transgenic adrenals developed malignant characteristics such as uncontrolled neovascularisation and loco-regional metastatic invasion. These oncogenic events were accompanied by ectopic differentiation of glomerulosa at the expense of fasciculata cells, which caused primary hyperaldosteronism. Altogether these observations demonstrate that constitutively active beta-catenin is an adrenal oncogene which triggers benign aldosterone-secreting tumour development and promotes malignancy.

PMID: 20106872 [PubMed - as supplied by publisher]

Solitary adrenal metastasis from invasive ductal breast cancer: an uncommon finding.

Fetched: January 31st, 2010, 3:49pm CST

Solitary adrenal metastasis from invasive ductal breast cancer: an uncommon finding.

World J Surg Oncol. 2010 Jan 28;8(1):7

Authors: Liu XJ, Shen P, Wang XF, Sun K, Sun FF

ABSTRACT: BACKGROUND: Invasive ductal carcinoma (IDC) of the breast usually metastasizes to the lungs, liver, bones and brain. Solitary adrenal metastasis is extremely rare. Due to the rarity of this condition, the optimal treatment is unclear. We report the first case of IDC of the breast metastasizing solely to the adrenal gland after a modified radical mastectomy but having a long-term disease-free survival while treated merely by a left adrenalectomy. CASE PRESENTATION: A 64-year-old woman was found a left adrenal mass on a follow- up visit two years after taking a right modified radical mastectomy for the breast cancer. She was subsequently given a left adrenalectomy. Postoperative histopathology findings were compatible with invasive ductal carcinoma (IDC) of the breast. Due to the patient's refusal, no further treatments were offered after the adrenalectomy. The patient now is still alive and has no sign of relapse. Survival time after taking the right modified radical mastectomy and the left adrenalectomy is more than five years and three years, respectively. CONCLUSION: This is the first case of a patient with solitary, metachronous adrenal metastasis from IDC of the breast to be reported. For patients in this condition, complete removal of metastasized organ may translate into survival benefit.

PMID: 20105336 [PubMed - as supplied by publisher]

Incidentally-discovered adrenal masses.

Fetched: January 31st, 2010, 3:49pm CST

Incidentally-discovered adrenal masses.

Discov Med. 2010 Jan;9(44):24-33

Authors: Gross MD, Korobkin M, Bou-Assaly W, Rubello D

Unanticipated adrenal masses are frequently encountered in modern, high resolution diagnostic imaging. Most often, these masses are benign adrenal adenomas, but when detected they necessitate a clinical evaluation sufficient to exclude subclinical endocrine disease, primary adrenal cancer, and remote metastases to the adrenal glands from other malignancies. These "incidentally-discovered" adrenal masses or so-called "adrenal incidentalomas" can be further evaluated with CT, MRI, and nuclear medicine imaging techniques. A substantial literature supports the use of each of these modalities to non-invasively characterize these neoplasms that have been considered by some as a 'disease' of modern imaging technology.

PMID: 20102682 [PubMed - in process]

18F-FDG PET/CT in the evaluation of adrenal masses in lung cancer patients.

Fetched: January 31st, 2010, 3:49pm CST

18F-FDG PET/CT in the evaluation of adrenal masses in lung cancer patients.

Neoplasma. 2010;57(2):129-34

Authors: Lu Y, Xie D, Huang W, Gong H, Yu J

The aim of this study was to assess the usefulness of integrated 18F-FDG PET/CT in differentiating benign from metastatic malignant adrenal masses in patients with lung cancer. <br />One hundred and ten adrenal masses (size range, 0.5 - 6.3 cm, mean size, 1.9 cm) were evaluated retrospectively in 87 lung cancer patients. Integrated PET/CT images were assessed. PET findings were interpreted as positive if the 18F-FDG uptake of the adrenal mass was greater than or equal to that of the liver. PET findings were interpreted as negative if the 18F-FDG uptake of the adrenal mass was less than that of the liver. All studies were reviewed independently by 3 nuclear medicine physicians, and the results were then correlated with clinical follow-up or biopsy results when available.<br />PET/CT findings were positive in 77adrenal masses. Seventy-four of these were eventually considered to be metastatic adrenal disease. In the remaining 3, in the course of follow-up, two underwent percutaneous puncture, and one underwent surgery. In the end, histopathological examinations of the adrenal lesions demonstrated the presence of adenomas. PET/CT findings were negative in 33 adrenal masses, of which 31 eventually proved to be benign. The 2 adrenal masses that were false-negative, underwent PET/CT twice with a two-month interval. At the initial study, the size was 0.5cm, 0.9cm in diameter, respectively. However, at the follow-up study, PET/CT showed both positive result with the size of 1.6cm, and 2.3cm in diameter, respectively. Both adrenal masses were interpreted as metastasis. The sensitivity, specificity, and accuracy for detecting metastatic disease were 97 %( 74 of 76), 94 %( 31 of 34), and 95% (105 of 110), respectively. The positive predictive value was 95 %( 74 of 77), and the negative predictive value was 94% (31 of 33).<br />Integrated 18F-FDG PET-CT is an accurate, noninvasive technique for differentiating benign from metastatic adrenal lesions detected on CT or MRI in patients with lung cancer. It allows early detection and accurate localization of adrenal lesions and differentiation of metastatic nodules from benign lesions, thereby facilitating treatment planning. Keywords: 18F-FDG; PET/CT; lung cancer; adrenal masses; adrenal metastasis.

PMID: 20099976 [PubMed - in process]

The pathophysiology, diagnosis and prognosis of adrenocortical tumors revisited by transcriptome analyses.

Fetched: January 31st, 2010, 3:49pm CST

The pathophysiology, diagnosis and prognosis of adrenocortical tumors revisited by transcriptome analyses.

Trends Endocrinol Metab. 2010 Jan 22;

Authors: Assié G, Guillaud-Bataille M, Ragazzon B, Bertagna X, Bertherat J, Clauser E

Analyzing gene expression (transcriptome) in tissue is now reliable using industrial pangenomic microarrays. Accumulating data on adrenal cortex and adrenocortical tumor transcriptomes have already identified striking transcriptome differences not only between adenoma and carcinoma but also between two sets of carcinoma, which have very different prognoses. These findings result in the development of diagnostic and prognostic molecular predictors, which improve the outcome determination compared with standard clinical and pathological tools. These transcriptome data observing adrenocortical tumor phenotype in great but complex detail, combined with genomic and proteomic information, will function for future research investigating the pathophysiology of their tumorigenesis and hormonal secretion.

PMID: 20097573 [PubMed - as supplied by publisher]

Letrozole.

Fetched: January 31st, 2010, 3:49pm CST

Letrozole.

Expert Opin Drug Metab Toxicol. 2010 Feb;6(2):251-9

Authors: Dellapasqua S, Colleoni M

IMPORTANCE OF THE FIELD: Aromatase is an enzyme which converts adrenal androgens to estrogens in postmenopausal women. Given the importance of estrogens in breast cancer growth, aromatase inhibitors are used to treat breast cancer in different settings. This review focuses on letrozole, a third generation aromatase inhibitor, encompassing pharmacodynamic and pharmacokinetic aspects as well as its clinical efficacy and safety. AREAS COVERED IN THIS REVIEW: A literature search and review of the studies published on letrozole were carried out using the MEDLINE database up to November 2009. WHAT THE READER WILL GAIN: The paper provides the reader with information about pharmacodynamic and pharmacokinetic aspects of letrozole, its preclinical and clinical efficacy and its safety. TAKE HOME MESSAGE: Letrozole is a well-tolerated and effective drug in metastatic breast cancer in postmenopausal women. Two clinical trials conducted in early breast cancer have confirmed its role in the adjuvant setting in postmenopausal women. More recently, data have confirmed its efficacy as neoadjuvant treatment in breast cancer. Future developments include the combination of letrozole with new biologic agents and tailoring of treatment with gene expression profiling studies.

PMID: 20095792 [PubMed - in process]

[The use of aromatase inhibitors in children]

Fetched: January 31st, 2010, 3:49pm CST

[The use of aromatase inhibitors in children]

Harefuah. 2009 Dec;148(12):825-30, 855

Authors: Eyal O, Naugolny L, Weintrob N

Aromatase inhibitors are compounds that block aromatase which converts androgens to estrogens. These compounds have been investigated for many years in a variety of conditions in which estrogen blockade is desired. During the past decade, a third generation of aromatase inhibitors has been developed with a much more potent blockade of the enzyme and less side effects. These new aromatase inhibitors are well absorbed after oral administration and, because of a long half life, are given once daily, thereby increasing compliance. The use of aromatase inhibitors is approved for the treatment of estrogen responsive breast cancer. Estrogens play a major role in bone maturation and growth plate fusion in both sexes. Therefore, inhibition of estrogen production may increase final height in a number of conditions where the final height is compromised. Due to their ability to block estrogen production, a number of clinical studies have been conducted to investigate their efficiency in a range of situations where estrogen blockade is desired. These include: peripheral precocious puberty secondary to congenital adrenal hyperplasia, familial male-dominant precocious puberty, and McCune-Albright syndrome, short stature in boys secondary to growth hormone deficiency, familial/genetic short stature, constitutional growth delay or idiopathic short stature. The use of aromatase inhibitors has been investigated also in pubertal gynecomastia. It is important to emphasize that this treatment, although it seems to be safe and encouraging, is still investigational and is not yet approved for routine usage.

PMID: 20088436 [PubMed - in process]

Image of the month--quiz case. Ganglioneuroma.

Fetched: January 31st, 2010, 3:49pm CST

Image of the month--quiz case. Ganglioneuroma.

Arch Surg. 2010 Jan;145(1):99-100

Authors: Materazzi G, Berti P, Conte M, Faviana P, Miccoli P

PMID: 20083762 [PubMed - indexed for MEDLINE]

[120 laparoscopic adrenalectomies with a harmonic scalpel]

Fetched: January 31st, 2010, 3:49pm CST

[120 laparoscopic adrenalectomies with a harmonic scalpel]

Rozhl Chir. 2009 Aug;88(8):439-43

Authors: Kasalický M, Krsek M, Zelinka T, Hána V, Widimský J

BACKGROUND: Presently the laparoscopic adrenalectomy (LA) becomes most popular since 1992 when it was performed for the first time by laparoscopic method by Gagner. Typical indication for LA is the aldosteron-secreting adenoma, Cushing's syndrome, feochromocytoma or scarcity tumors such as adrenal cyst or myelolipomas. AIM: The evaluation of the laparoscopic adrenalectomy as safe method for adrenals tumout's extirpation to the sizes 10 cm. METHODS: LA is provided with transperitoneal lateral approach most frequently then in back side position. Retroperitoneal approach is used less commonly. RESULTS: Since 2006, firstly at the 1st Surgical department of 1st Medical School of Charles University and General Faculty Hospital and lastly at the Surgical department 2nd Medical School of Charles University and Central Military Hospital, has been performed on the whole 120 LA in 114 patients. The bilateral LA was performed in 6 patients. The conversion from the laparoscopic to open adrenalectomy was necessary by reason of the king size of the tumor (13/14 cm) in two cases. The lateral position and transperitoneal approach was used in all cases. The harmonie scalpel was used with advantage. Average length of the operation was 82 minutes (40-154 min), respective 180 minutes (130-270 min) in the case of the bilateral LA. The median size of the adrenal tumor was 4.9 cm (1.5-12.5 cm) with average weight 44 g (18-421 g). All of the patients after LA were monitored for 24 hours in the Intensive care units. The average time of the hospitalization was 3.7 days (2-6 days). CONCLUSION: Laparoscopic adrenalectomy presently becomes as the "gold standard" for the treatment of adrenal tumors to the size 10 cm namely and in the event of malignancy. The harmonie scalpel is useful and sparing advice for the LA.

PMID: 20055298 [PubMed - indexed for MEDLINE]

[Laparoscopic adrenalectomy]

Fetched: January 31st, 2010, 3:49pm CST

[Laparoscopic adrenalectomy]

Rozhl Chir. 2009 Sep;88(9):514-20

Authors: Stránský P, Hora M, Eret V, Klecka J, Urge T, Grégrová H, Dvoráková E, Hes O, Chudácek Z, Kreuzberg B

OBJECTIVE: Laparoscopy has become the gold standard for the treatment of adrenal tumours in urology. We evaluate our experience with laparoscopic adrenalectomy (LA) in this work. MATERIAL, METHODS: We performed 38 LA between 2003-2008. We use computer tomography (CT) and magnetic resonance imaging (MRI) for the initial evaluation. Indication for procedure is made in cooperation with endocrinologist. We use transperitoneal approach with 3 or 5 ports. RESULTS: Mean age was 57.7 +/- 11.7 year (range 32-74.9 year). Nine LA were made in men (24%), in women 29 (76%). Sixteen tumours (42%) were hormonal active (7 pheochromocytoma, 6 primary hyperaldosteronism, 3 peripheral hypercortisolism). Twenty-two tumours were without hormonal activity. Mean tumour size was 4.1 +/- 2 cm (range 1-10.1 cm), mean operation time was 89 +/- 38 minutes (range 32-220 minutes), mean blood loss was 33 +/- 75 ml (range 0-400 ml), mean hospitalization time was 6.1 days (range 3-12 days). There were histologically 15 cortical adenomas, 5 nodular cortical hyperplasia, 1 calcificated hematoma, 3 cysts, 2 potentional malignant tumours on interface between adenoma and carcinoma, 1 cortical carcinoma and 7 pheochromocytoma. We found 3 metastases of renal carcinoma in adrenal gland and one metastasis mesenchymal chondrosarcoma too. Transperitoneal approach was chosen in 20 patients (53%) after previous abdominal operation (open cholecystectomy, appendectomy, transperitoneal nephrectomy, aortofemoral bypass). Complications were in 3 cases from 38 (8%). It was one perforation of diaphragm, which was resolve with laparoscopic suture, one postoperative delirium with fudge and agitation, one abscess in wound after extraction of specimen. We have got any conversion in our collection. The body mass index was higher than 38 in 3 patients. CONCLUSION: LA is a quick and safe procedure with minimal morbidity and mortality. This procedure requires very experienced surgeon. Patients profit especially from miniinvasivity and short convalescence. Especially benign tumours of smaller size (by 8 cm) are indicated, extensive and especially malignant tumours remain a domain of open approach. Previous operations in abdominal cavity do not have to be a contraindication for LA and operation is possible in patients with monster obesity.

PMID: 20052929 [PubMed - indexed for MEDLINE]

Characterization of adrenal lesions: comparison of 2D and 3D dual gradient-echo MR imaging at 3 T--preliminary results.

Fetched: January 31st, 2010, 3:49pm CST

Characterization of adrenal lesions: comparison of 2D and 3D dual gradient-echo MR imaging at 3 T--preliminary results.

Radiology. 2010 Jan;254(1):179-87

Authors: Marin D, Soher BJ, Dale BM, Boll DT, Youngblood RS, Merkle EM

PURPOSE: To retrospectively compare a two-dimensional (2D) and a three-dimensional (3D) technique for in-phase (IP) and opposed-phase (OP) single-breath-hold 3-T magnetic resonance (MR) imaging in the characterization of adrenal lesions, with histopathologic confirmation, computed tomographic findings, or imaging follow-up for a minimum of 6 months used as the reference standard. MATERIALS AND METHODS: This retrospective HIPAA-compliant study was approved by institutional review board, and a waiver of informed consent was obtained. Thirty-four patients (mean age, 57 years) with 37 adrenal lesions underwent 3-T adrenal MR imaging with both 2D and 3D single-breath-hold dual gradient-echo (GRE) MR sequences. Signal intensity (SI) index and adrenal-to-spleen, adrenal-to-liver, and adrenal-to-muscle SI ratios for each lesion were compared between the two techniques by using repeated-measures analysis of variance. The area under the receiver operating characteristic curve (AUC) for each evaluation method was determined, with retrospective selection of suggested thresholds. RESULTS: For the 2D and 3D techniques, the mean SI index and SI ratios were significantly different between adenomas and nonadenomas (P < .05), except for the adrenal-to-liver SI ratio with the 2D technique and the adrenal-to-muscle SI ratio with both techniques. For all evaluation methods, the AUCs were higher, although not statistically significant, for the 3D technique. The two techniques exploited different suggested thresholds for discriminating adenomas from nonadenomas. CONCLUSION: Adrenal adenomas can be readily differentiated from nonadenomas at 3-T MR imaging with either a 2D or 3D single-breath-hold dual GRE MR technique. Depending on the acquisition technique, different suggested thresholds need to be selected for various evaluation methods.

PMID: 20032151 [PubMed - indexed for MEDLINE]

Permalink for 'Evaluation of response in malignant tumors treated with the multitargeted tyrosine kinase inhibitor sorafenib: a multitechnique imaging assessment.'

Evaluation of response in malignant tumors treated with the multitargeted tyrosine kinase inhibitor sorafenib: a multitechnique imaging assessment.

Fetched: January 31st, 2010, 3:49pm CST

Evaluation of response in malignant tumors treated with the multitargeted tyrosine kinase inhibitor sorafenib: a multitechnique imaging assessment.

AJR Am J Roentgenol. 2010 Jan;194(1):5-14

Authors: Maksimovic O, Schraml C, Hartmann JT, Bitzer M, Claussen CD, Pintoffl J, Horger M

OBJECTIVE: The purpose of this article is to illustrate the characteristic changes induced in different tumor types by the multitargeted tyrosine kinase inhibitor sorafenib. CONCLUSION: Sorafenib reduces tumor perfusion and thereby induces necrosis and often hemorrhage. Malignant tumors treated with sorafenib undergo both morphologic and functional changes; however, the morphologic changes are less frequent and inadequate for early evaluation of response. Therefore, imaging tools accurately assessing hemorrhage and decrease in tumor perfusion with subsequent necrosis should be the mainstay in monitoring targeted therapy agents.

PMID: 20028898 [PubMed - indexed for MEDLINE]

[Paraganglioma of the pancreas metastasized to the adrenal gland: a case report]

Fetched: January 31st, 2010, 3:49pm CST

[Paraganglioma of the pancreas metastasized to the adrenal gland: a case report]

Korean J Gastroenterol. 2009 Dec;54(6):409-12

Authors: Paik KY

Paraganglioma is a rare neuroendocrine tumor arising from the neural crest, which includes tissues such as the adrenal medulla, carotid and aortic body, organs of Zuckerkandl, and other unnamed paraganglia. The head, neck, and retroperitoneum are the most common sites for paraganglioma. However, paraganglioma of the pancreas is extremely rare. We report our experience of this rare disease. A 70-year old female patient admitted for a pancreas tail mass detected by computed tomography (CT) scan checked for vague left flank pain. CT with contrast enhancement showed a 4.2-cm heterogeneously enhanced lesion in the tail of the pancreas. A well defined ovoid shape mass in left adrenal gland was suggested adenoma. Distal pancreatectomy with left adrenalectomy was performed. Any lymph node enlargement was not found. Pancreas mass did not invade adjacent organ. Microscopic examination with pancreas and adrenal gland revealed that the cells were arranged in a characteristic Zellballen pattern. Immunohistochemical staining revealed positivity for neuron-specific enolase, chromogranin A, synaptophysin, and S-100 protein. On the basis of these findings, we diagnosed the tumor as a paraganglioma of the pancreas and adrenal gland. We report the first case of pancreas paraganglioma in Korea.

PMID: 20026898 [PubMed - indexed for MEDLINE]

[Perioperative management of a pheochromocytoma in a pediatric patient: use of transesophageal Doppler echocardiography]

Fetched: January 31st, 2010, 3:49pm CST

[Perioperative management of a pheochromocytoma in a pediatric patient: use of transesophageal Doppler echocardiography]

Rev Esp Anestesiol Reanim. 2009 Oct;56(8):520-1

Authors: Laporta Baez Y, Campo García GD, Palomero Rodríguez MA, Pérez Ferrer A, Goldman Tarvloski L

PMID: 19994626 [PubMed - indexed for MEDLINE]

Symptomatic hypocalcemia in primary hyperaldosteronism: a case report.

Fetched: January 31st, 2010, 3:49pm CST

Symptomatic hypocalcemia in primary hyperaldosteronism: a case report.

J Korean Med Sci. 2009 Dec;24(6):1220-3

Authors: Pai SG, Shivashankara KN, Pandit V, Sheshadri S

The metabolic alterations caused by hyperaldosteronism are being increasingly recognized and have generated considerable interest among the medical fraternity. Hyperaldosteronism is suspected to have a pivotal role in the patho-physiology of congestive cardiac failure where it has been studied extensively. But its effects on calcium metabolism, parathyroid metabolism and renal handling of calcium are less well described. Recent experimental models have shed light into the roles played by previously unknown mechanisms in causing these metabolic alterations. We hereby report a case of primary hyperaldosteronism due to adrenal adenoma (Conn's syndrome) who presented with a myriad of clinical features including symptomatic hypocalcemia, significant weight loss along with uncontrolled hypertension for a prolonged period before eventually detected to have primary hyperaldosteronism. Surgical removal of the causative tumor resulted in prompt disappearance of all the symptoms and signs and regain of lost weight.

PMID: 19949688 [PubMed - indexed for MEDLINE]

Retroperitoneal ectopic pancreas: imaging findings.

Fetched: January 31st, 2010, 3:49pm CST

Retroperitoneal ectopic pancreas: imaging findings.

Br J Radiol. 2009 Dec;82(984):e253-5

Authors: Lin LH, Ko SF, Huang CC, Ng SH, Lin JW, Sheen-Chen SM

Ectopic pancreas is relatively uncommon and usually occurs in the stomach or duodenum. Retroperitoneal ectopic pancreas has not previously been documented. We report the case of a 48-year-old man with retroperitoneal ectopic pancreas that imitated bilateral adrenal tumours on ultrasound and MRI. Subsequent CT-guided biopsies confirmed an ectopic pancreas. The lesions remained stable during follow-up for 7 years. In retrospect, the similarity in signal intensities and enhancement pattern between the retroperitoneal masses and the pancreas may have been a clue to the diagnosis of this rare entity.

PMID: 19934067 [PubMed - indexed for MEDLINE]

Within-patient reproducibility of the aldosterone: renin ratio in primary aldosteronism.

Fetched: January 31st, 2010, 3:49pm CST

Within-patient reproducibility of the aldosterone: renin ratio in primary aldosteronism.

Hypertension. 2010 Jan;55(1):83-9

Authors: Rossi GP, Seccia TM, Palumbo G, Belfiore A, Bernini G, Caridi G, Desideri G, Fabris B, Ferri C, Giacchetti G, Letizia C, Maccario M, Mallamaci F, Mannelli M, Patalano A, Rizzoni D, Rossi E, Pessina AC, Mantero F,

The plasma aldosterone concentration:renin ratio (ARR) is widely used for the screening of primary aldosteronism, but its reproducibility is unknown. We, therefore, investigated the within-patient reproducibility of the ARR in a prospective multicenter study of consecutive hypertensive patients referred to specialized centers for hypertension in Italy. After the patients were carefully prepared from the pharmacological standpoint, the ARR was determined at baseline in 1136 patients and repeated after, on average, 4 weeks in the patients who had initially an ARR > or =40 and in 1 of every 4 of those with an ARR <40. The reproducibility of the ARR was assessed with Passing and Bablok and Deming regression, coefficient of reproducibility, and Bland-Altman and Mountain plots. Within-patient ARR comparison was available in 268 patients, of whom 49 had an aldosterone-producing adenoma, on the basis of the "4-corner criteria." The ARR showed a highly significant within-patient correlation (r=0.69; P<0.0001) and reproducibility. Bland-Altman plot showed no proportional, magnitude-related, or absolute systematic error between the ARR; moreover, only 7% of the values, for example, slightly more than what could be expected by chance, fell out of the 95% CI for the between-test difference. The accuracy of each ARR for pinpointing aldosterone-producing adenoma patients was approximately 80%. Thus, although it was performed under different conditions in a multicenter study, the ARR showed a good within-patient reproducibility. Hence, contrary to previously claimed poor reproducibility of the ARR, these data support its use for the screening of primary aldosteronism.

PMID: 19933925 [PubMed - indexed for MEDLINE]

Permalink for 'Clinicopathological study of a series of 92 adrenocortical carcinomas: from a proposal of simplified diagnostic algorithm to prognostic stratification.'

Clinicopathological study of a series of 92 adrenocortical carcinomas: from a proposal of simplified diagnostic algorithm to prognostic stratification.

Fetched: January 31st, 2010, 3:49pm CST

Clinicopathological study of a series of 92 adrenocortical carcinomas: from a proposal of simplified diagnostic algorithm to prognostic stratification.

Histopathology. 2009 Nov;55(5):535-43

Authors: Volante M, Bollito E, Sperone P, Tavaglione V, Daffara F, Porpiglia F, Terzolo M, Berruti A, Papotti M

AIMS: Pathological diagnosis of adrenocortical carcinoma relies on several microscopic features commonly used in combination in different scoring systems that are sometimes subjective and/or time consuming. The aim was to investigate the impact of individual pathological parameters in the diagnosis and prognosis of adrenocortical carcinoma. METHODS AND RESULTS: The series included 92 malignant cases and a control series of 47 adenomas, all classified according to Weiss score criteria. The presence of disruption of the reticular network, as highlighted by histochemical staining, was present in all adrenocortical carcinomas and the inclusion of at least one of the three following additional parameters - mitotic index >5/50 high-power fields (HPF), presence of necrosis and presence of vascular invasion - gave an algorithm with 100% sensitivity and specificity to recognize malignant tumours according to the Weiss system, with easier and more practical applicability. Moreover, on multivariate analysis, stage III/IV and mitotic count >9/50 HPF showed a strong adverse impact on disease-free and overall survival, leading to the identification of three risk groups affected by a significantly different prognosis. CONCLUSIONS: We have defined an easy-to-perform and highly specific and sensitive algorithm for the diagnosis and prognostic categorization of adrenocortical tumours.

PMID: 19912359 [PubMed - indexed for MEDLINE]

Initial presentation of pheochromocytoma with Takotsubo cardiomyopathy: a brief review of literature.

Fetched: January 31st, 2010, 3:49pm CST

Initial presentation of pheochromocytoma with Takotsubo cardiomyopathy: a brief review of literature.

J Cardiovasc Med (Hagerstown). 2010 Jan;11(1):49-52

Authors: Gujja KR, Aslam AF, Privman V, Tejani F, Vasavada B

Takotsubo cardiomyopathy, or transient left ventricular apical ballooning or broken heart syndrome, is characterized by excessive sympathetic stimulation induced acute coronary vasospasm. A 46-year-old female presented with polyuria and polydypsia and was diagnosed with new-onset diabetes mellitus, treated with insulin and intravenous fluids. During the hospital stay, she complained of an episode of left-sided chest pain and had mildly elevated cardiac enzymes. EKG showed new ST-segment elevation in V2, V3 leads without reciprocal changes. Her coronary angiogram showed no significant coronary artery stenosis, but severe systolic dysfunction and akinesis of the mid-anterior, anteroapical, mid-inferior and inferoapical segments. Further workup was negative except for plasma metanephrine being elevated. MRI of the abdomen showed a right adrenal mass consistent with pheochromocytoma. Surgical resection of the adrenal mass showed evidence of pheochromocytoma and the patient's symptoms were resolved.

PMID: 19797977 [PubMed - indexed for MEDLINE]

Multiple endocrine neoplasia type 2 (MEN 2).

Fetched: January 31st, 2010, 3:49pm CST

Multiple endocrine neoplasia type 2 (MEN 2).

Eur J Cancer. 2009 Sep;45 Suppl 1:267-73

Authors: Frank-Raue K, Raue F

PMID: 19775624 [PubMed - indexed for MEDLINE]

Adjuvant corticosteroid therapy in 2 children with hepatosplenic candidiasis-related IRIS.

Fetched: January 31st, 2010, 3:49pm CST

Adjuvant corticosteroid therapy in 2 children with hepatosplenic candidiasis-related IRIS.

J Pediatr Hematol Oncol. 2009 Oct;31(10):794-6

Authors: Saint-Faust M, Boyer C, Gari-Toussaint M, Deville A, Poiree M, Weintraub M, Sirvent N

First described in HIV-infected patients who recently initiated highly active antiretroviral therapy, the immune reconstitution inflammatory syndrome (IRIS) is best characterized as a collection of inflammatory disorders triggered by rapid resolution of immunosuppression. Treatment of IRIS is a clinical challenge due to the variety of clinical presentations and the presence of multiple pathogens capable of causing the syndrome. Hepatosplenic candidiasis, an uncommon form of invasive Candida species infection, was recently suggested to belong to the spectrum of fungus-related IRIS. We report 2 cases of probable hepatosplenic candidiasis according to the guidelines of the European Organization for Research and Treatment of Cancer and the Mycosis Study Group, occurring in pediatric patients with acute leukemia during rapid neutrophil recovery after cytotoxic chemotherapy. In both cases, abdominal computed tomography scan revealed multiple hepatic micronodules, and liver biopsy showed nonspecific granulomatous lesions. Hepatosplenic candidiasis symptoms (fever, nausea and vomiting, abdominal pain) resolved within 2 days after adjunction of corticosteroid therapy to antifungal treatment. Inflammatory markers and related radiologic abnormalities decreased or disappeared within 1 month. Recovery of neutrophil count in a context of hepatosplenic candidiasis may result in a heightened inflammatory response. Corticosteroid therapy in this setting is associated with prompt resolution of the symptoms.

PMID: 19770685 [PubMed - indexed for MEDLINE]

Bone morphogenetic proteins and zoledronic acid.

Fetched: January 31st, 2010, 3:49pm CST

Bone morphogenetic proteins and zoledronic acid.

Ann Oncol. 2009 Dec;20(12):2019; author reply 2019

Authors: Sahin U, Alkan A, Altundag K

PMID: 19752002 [PubMed - indexed for MEDLINE]

Permalink for 'Characteristics of morphological change in erythrocytes during metaststic adenocarcinoma of the prostate before and after castration.'

Characteristics of morphological change in erythrocytes during metaststic adenocarcinoma of the prostate before and after castration.

Fetched: January 31st, 2010, 3:49pm CST

Characteristics of morphological change in erythrocytes during metaststic adenocarcinoma of the prostate before and after castration.

Georgian Med News. 2009 Jul-Aug;(172-173):10-3

Authors: Veshapidze N, Alibegashvili M, Gabunia N, Chigogidze T, Managadze L

The objective of our study was to establish whether there is a direct link between displayed changes in Erythrocytes and hormonal shifts talking place in organism. For the experimental research we used a blood serum and erythrocytes of 15 men with metastasized adenocarcinoma before castration and a blood serum and erythrocytes of 15 men in 6 months after castration, also a blood serum and erythrocytes of practically healthy men. Age of patients varied from 60-75 years. Structural observation of erythrocytes was conducted by means of light microscope method, and quantitative calculation of steroid hormones was performed by means of the method of imunopherment analysis. Deep morphological changes of erythrocytes were observed in peripheral blood during metastasized adenocarcinoma of prostate gland before and after castration. At the same time during this pathology we detected sharp quantitative change of steroid hormones of testicular and adrenal glands. It was established that the deep morphological changes of erythrocytes in peripheral blood should be related to the cancerous processes occurring in organism (direct way); It was established that during the noted pathology hormonal shifts taking place in organism (indirect way) influence the course of erythropoiesis and cause changes of surface architectonics of erythrocytes, as a result of which pathological erythrocytes -cells with altered form and low functional activity, are formed.

PMID: 19644180 [PubMed - indexed for MEDLINE]

Ectopic Cushing's syndrome due to a pheochromocytoma: a new case in the post-partum and review of literature.

Fetched: January 31st, 2010, 3:49pm CST

Ectopic Cushing's syndrome due to a pheochromocytoma: a new case in the post-partum and review of literature.

Gynecol Endocrinol. 2009 Sep;25(9):624-7

Authors: Cohade C, Broussaud S, Louiset E, Bennet A, Huyghe E, Caron P

Cushing's syndrome due to AdrenoCorticoTropic Hormone (ACTH)-secreting pheochromocytoma has been rarely reported during pregnancy and post-partum. We report the case of a 30-year-old woman who presented 3 months after delivery acute psychiatric signs and rapid progressive features of Cushing's syndrome. Hormonal tests confirmed ACTH-dependant Cushing's syndrome. A computed tomography scan revealed a 25 x 30 mm tumoral mass in the left adrenal gland and octreoscan scintigraphy showed only an uptake of the radiolabelled octreotide by the adrenal tumor. Fractionated 24-h urinary catecholamines and metanephrines were in the normal range, except for slightly increased adrenalin levels. A left laparoscopic adrenalectomy was performed with acute pulmonary oedema following the anesthesia. Histological examination revealed a 3.5 x 2.5 cm adrenal tumor consistent with a pheochromocytoma without signs of malignancy. The tumor cells immunostained for ACTH and diffuse hyperplasia of adrenocortical cells was observed. After surgery and short stay in intensive care unit, clinical and biological signs rapidly improved and both anti-hypertensive treatment and insulin injections were withdrawn. Genetic testing did not reveal germline mutations in RET protooncogene, Von Hippel Lindau and succinate deshydrogenase genes.

PMID: 19591016 [PubMed - indexed for MEDLINE]

Neuroimaging patterns of central nervous system metastases in neuroblastoma: report of 2 recent cases and literature review.

Fetched: January 31st, 2010, 3:49pm CST

Neuroimaging patterns of central nervous system metastases in neuroblastoma: report of 2 recent cases and literature review.

J Child Neurol. 2009 Oct;24(10):1290-3

Authors: Balaji R, Ramachandran K, Kusumakumari P

The authors describe imaging patterns of intracranial metastases in 2 children with grade 4 neuroblastoma. Central nervous system metastases from neuroblastoma are extremely rare and may involve the cerebral parenchyma, leptomeninges, or dura. Cerebral parenchymal metastases can be cystic with mural nodules or solid with hemorrhagic elements. The first patient in our study had multiple cystic parenchymal metastases with calcific mural nodules, while the second patient developed solid hemorrhagic parenchymal metastatic lesions along with extensive leptomeningeal and dural deposits. Central nervous system involvement in both patients occurred within a time span ranging from 12 to 14 months from the time of initial diagnosis.

PMID: 19564645 [PubMed - indexed for MEDLINE]

Recurrent takotsubo cardiomyopathy associated with pheochromocytoma.

Fetched: January 31st, 2010, 3:49pm CST

Recurrent takotsubo cardiomyopathy associated with pheochromocytoma.

Endocr Pract. 2009 Sep-Oct;15(6):560-2

Authors: Rossi AP, Bing-You RG, Thomas LR

OBJECTIVE: To describe a case of recurrent takotsubo cardiomyopathy in a patient with pheochromocytoma. METHODS: We present a case report, including clinical and laboratory data. In addition, the current relevant literature pertaining to pheochromocytoma and takotsubo syndrome is reviewed and summarized. RESULTS: In 2004, an 81-year-old woman with no history of cardiac disease presented with chest discomfort, and takotsubo syndrome was diagnosed. No emotional or physical stressors were identified at that time. Her left ventricular systolic function normalized during that hospitalization. In 2007, the patient was readmitted to the hospital with chest discomfort and ST-segment elevation. Cardiac catheterization demonstrated only minor nonobstructive coronary artery disease. She was again found to have takotsubo syndrome with a classic apical hypokinetic segment. Treatment with a heart failure regimen was initiated, and she was screened for pheochromocytoma as the precipitant for her recurrent takotsubo cardiomyopathy. A 24-hour urine collection showed minimally elevated normetanephrine excretion of 719 microg (reference range, 148 to 560) and vanillylmandelic acid of 8.3 mg (reference range, <8.0). The plasma normetanephrine level was 1.57 pg/mL (reference range, <0.9). Subsequent magnetic resonance imaging revealed a left adrenal mass (2 cm by 1 cm). Ultimately, the patient underwent left adrenalectomy, and the pathology report was consistent with pheochromocytoma. She has been asymptomatic since then, and a repeated echocardiogram demonstrated normal left ventricular systolic function. CONCLUSION: In patients presenting with takotsubo cardiomyopathy, a precipitating factor, such as emotional or physical stress, can often be identified. In some patients (such as our current case), however, pheochromocytoma may be the underlying disease and should be considered.

PMID: 19491077 [PubMed - indexed for MEDLINE]

Gravidity complicated by abdominal neoplastic process.

Fetched: January 31st, 2010, 3:49pm CST

Gravidity complicated by abdominal neoplastic process.

Leg Med (Tokyo). 2009 Apr;11 Suppl 1:S494-5

Authors: Krajcovic J, Macko V, Straka L, Novomeský F

The case of 31-year-old pregnant woman in the 28th week of pregnancy is presented. She was brought in a bad condition to a small hospital by her parents. The case history of only 5 h included e.g. nausea, multiple emesis, cephalea, deteriorated respiration. Shock status was diagnosed in the hospital intensive care unit. After the patient lost her consciousness, resuscitation, intubation and artificial ventilation breath control were realised immediately, the doses of 13 mg of adrenalin were applied.

PMID: 19286412 [PubMed - indexed for MEDLINE]

Evidence of Limited Contributions for Intratumoral Steroidogenesis in Prostate Cancer.

Fetched: January 21st, 2010, 6:02pm CST

Evidence of Limited Contributions for Intratumoral Steroidogenesis in Prostate Cancer.

Cancer Res. 2010 Jan 19;

Authors: Hofland J, van Weerden WM, Dits NF, Steenbergen J, van Leenders GJ, Jenster G, Schröder FH, de Jong FH

Androgen-deprivation therapy for prostate cancer (PC) eventually leads to castration-resistant PC (CRPC). Intratumoral androgen production might contribute to tumor progression despite suppressed serum androgen concentrations. In the present study, we investigated whether PC or CRPC tissue may be capable of intratumoral androgen synthesis. Steroidogenic enzyme mRNAs were quantified in hormonally manipulated human PC cell lines and xenografts as well as in human samples of normal prostate, locally confined and advanced PC, local nonmetastatic CRPC, and lymph node metastases. Overall, the majority of samples showed low or absent mRNA expression of steroidogenic enzymes required for de novo steroid synthesis. Simultaneous but low expression of the enzymes CYP17A1 and HSD3B1, essential for the synthesis of androgens from pregnenolone, could be detected in 19 of 88 patient samples. Of 19 CRPC tissues examined, only 5 samples expressed both enzymes. Enzymes that convert androstenedione to testosterone (AKR1C3) and testosterone to dihydrotestosterone (DHT; SRD5A1) were abundantly expressed. AKR1C3 expression was negatively regulated by androgens in the experimental models and was increased in CRPC samples. Expression of SRD5A1 was upregulated in locally advanced cancer, CRPC, and lymph node metastases. We concluded that intratumoral steroid biosynthesis contributes less than circulating adrenal androgens, implying that blocking androgen production and its intraprostatic conversion into DHT, such as via CYP17A1 inhibition, may represent favorable therapeutic options in patients with CRPC. Cancer Res; 70(3); 1256-64.

PMID: 20086173 [PubMed - as supplied by publisher]

Resorcylic acid lactone L-783,277 inhibits the growth of the human adrenal cancer cell line H295R in vitro.

Fetched: January 20th, 2010, 9:02am CST

Resorcylic acid lactone L-783,277 inhibits the growth of the human adrenal cancer cell line H295R in vitro.

Int J Immunopathol Pharmacol. 2009 Oct-Dec;22(4):889-95

Authors: Lawnicka H, Kowalewicz-Kulbat M, Sicinska P, Altmann KH, Hofmann T, Stepien H

The resorcylic acid lactone L-783,277, isolated from a Phoma sp. (ATCC 74403), is a potent and specific inhibitor of MEK (Map kinase kinase) that exerts very interesting pharmacological activities including anti-neoplastic properties. However, the role of this compound in the regulation of endocrine-related cancer cell growth and tumor progression remains unknown. In the present study we have evaluated the effect of L-783,277 on the viability, proliferation and cell cycle of the human adrenocortical carcinoma cell line H295R. L-783,277 inhibited viability (IC50 of 22 microM) and cell proliferation (IC50 of 21 microM) of H295R. At concentrations of 10-6-10-8M this effect was associated with the accumulation of H295R cells in S-phase, whereas at concentrations of 10-9-10-10M a prolonged G1-phase and reduced transition into S-phase were observed. Our findings demonstrate for the first time the anti-proliferative action of L-783,277 on the human adrenocortical H295R cell line.

PMID: 20074452 [PubMed - in process]

Permalink for 'Investigational New Drug-Directed Toxicology and Pharmacokinetic Study of 4-[3-(2-Nitro-1-Imidazolyl)-Propylamino]-7-Chloroquinoline Hydrochloride (NLCQ-1, NSC 709257) in Beagle Dogs.'

Investigational New Drug-Directed Toxicology and Pharmacokinetic Study of 4-[3-(2-Nitro-1-Imidazolyl)-Propylamino]-7-Chloroquinoline Hydrochloride (NLCQ-1, NSC 709257) in Beagle Dogs.

Fetched: January 20th, 2010, 9:02am CST

Investigational New Drug-Directed Toxicology and Pharmacokinetic Study of 4-[3-(2-Nitro-1-Imidazolyl)-Propylamino]-7-Chloroquinoline Hydrochloride (NLCQ-1, NSC 709257) in Beagle Dogs.

Basic Clin Pharmacol Toxicol. 2010 Jan 14;

Authors: Papadopoulou MV, Bloomer WD, Torti VR, Page JG

4-[3-(2-Nitro-1-imidazolyl)-propylamino]-7-chloroquinoline hydrochloride (NLCQ-1), a 2-nitroimidazole-based hypoxia-selective cytotoxin has been shown to target hypoxic regions of solid tumours. The present study is one of several pre-clinical toxicology studies conducted in support of an 'investigational new drug' (IND) application to test this agent as an adjuvant to radio/chemotherapy for the treatment of cancer in humans. Twenty-four dogs were each assigned to one vehicle control group or to one of three test article-treated groups (three dogs/sex/treatment group). Intravenous (i.v.) doses of 0, 2.74, 5.48 and 10.95 mg/kg/day (54.8, 109.6 or 219 mg/m(2)/day) were administered on a per day x 5 days (qd x 5) schedule. NLCQ-1 was formulated as a solution in sterile saline at 1.5 mg/ml. None of the dogs died during this 33-day study. With few exceptions, most of the clinical signs of toxicity were noted within 2 hr following dosing in the 10.95 mg/kg/day dose group. These observations included aggressive behaviour, ataxia, tachypnea, emesis, hypoactivity, excessive salivation, tremors, and involuntary urination and defecation. Aggressive behaviour was judged to be dose-limiting. No clinical signs of toxicity were noted during the 28-day observation period that followed the 5-day dose period. Findings in a functional observation battery examination were consistent with the clinical observations. No drug-related effects were noted on the body weight or food consumption values, and no drug-related changes were noted during ocular examinations made on these animals prior to scheduled necropsy or during examination of electrocardiogram recordings made at 15 min. and 2 hr after dosing on days 1 and 5. No definitive changes in haematology, clinical chemistry or coagulation values were noted in dogs treated with NLCQ-1. NLCQ-1 was detected in the plasma of treated dogs on days 1 and 5, up to 60 min. after dosing (2.74 and 5.48 mg/kg/day) and up to 8 hr after dosing (10.95 mg/kg/day). There was a dose-related increase in maximum plasma concentration of NLCQ-1 at 5 min. after dosing; comparable concentrations were noted on days 1 and 5. No definitive test article-related lesions were noted during microscopic evaluation of tissues from dogs in this study, although lesions noted at the injection site and in the vascular tissue, lungs, thymus, prostate gland, muscle, adrenal cortex and tongue may have resulted from treatment with this drug. Any drug-related toxicity noted was readily reversible and not cumulative. No sex difference was detected in the susceptibility to NLCQ-1-induced toxicity.

PMID: 20074266 [PubMed - as supplied by publisher]

Permalink for 'Management of Cushing's Syndrome due to Ectopic Adrenocorticotropin Secretion with 1,Ortho-1, Para'-Dichloro-Diphenyl-Dichloro-Ethane: Findings in 23 Patients from a Single Center.'

Management of Cushing's Syndrome due to Ectopic Adrenocorticotropin Secretion with 1,Ortho-1, Para'-Dichloro-Diphenyl-Dichloro-Ethane: Findings in 23 Patients from a Single Center.

Fetched: January 20th, 2010, 9:02am CST

Management of Cushing's Syndrome due to Ectopic Adrenocorticotropin Secretion with 1,Ortho-1, Para'-Dichloro-Diphenyl-Dichloro-Ethane: Findings in 23 Patients from a Single Center.

J Clin Endocrinol Metab. 2010 Jan 8;

Authors: Donadille B, Groussin L, Waintrop C, Abbas H, Tenenbaum F, Dugué MA, Coste J, Bertagna X, Bertherat J

Context: Effective treatment for the ectopic ACTH secretion syndrome (EAS) remains a therapeutic challenge. Immediate curative surgery of the responsible nonpituitary tumor is often not possible. Objective: The objective of the study was to evaluate 1,ortho-1, para'-dichloro-diphenyl-dichloro-ethane (O,p'DDD) therapy in EAS. Design and Patients: Patients included 36 consecutive patients with EAS from a single center treated between 1990 and 2006. Twenty-three of these patients, including 18 women aged 53.7 +/- 12.9 yr (mean +/- SD), were treated with O,p'DDD. Patient follow-up was 8.04 +/- 9.6 yr. Results: A mean daily O,p'DDD dose of 3.3 +/- 1.2 g Lysodren equivalent was given for a mean duration of 1.8 +/- 2.1 yr. Urinary cortisol decreased from 2603 +/- 3443 mug/d before treatment to 79 +/- 169 mug/d at the time of maximal O,p'DDD efficacy. Urinary cortisol was normalized in 21 of the 23 patients. Adrenal insufficiency was observed in 20 patients. This was associated with clinical improvement of Cushing's syndrome manifestations, including diabetes, hypertension, and hypokalemia. O,p'DDD plasma levels were 10.4 +/- 6.5 mug/ml in the 12 patients tested at the time of adrenal insufficiency. Side effects were observed during the first 6 months in seven of 15 patients (46%). National Cancer Institute-Classification Common Toxicity Criteria grade 1 or 2 digestive or neurologic toxicity resolved after withdrawal or reduction of O,p'DDD. Careful monitoring was essential to long-term control, clinical improvement, and good tolerability. Medical control of the disease allowed the subsequent characterization of tumors in eight of 13 patients with initially occult tumors. Conclusion: With close monitoring, O,p'DDD could be a potent medical treatment for long-term control and management of EAS.

PMID: 20061433 [PubMed - as supplied by publisher]

Permalink for 'Multiple Paraganglioma Syndrome type 4 Secondary to Succinate Dehydrogenase B Mutation: The Diagnostic and Therapeutic Challenges of a Skull Base Paraganglioma Masquerading as Nasopharyngeal Cancer.'

Multiple Paraganglioma Syndrome type 4 Secondary to Succinate Dehydrogenase B Mutation: The Diagnostic and Therapeutic Challenges of a Skull Base Paraganglioma Masquerading as Nasopharyngeal Cancer.

Fetched: January 20th, 2010, 9:02am CST

Multiple Paraganglioma Syndrome type 4 Secondary to Succinate Dehydrogenase B Mutation: The Diagnostic and Therapeutic Challenges of a Skull Base Paraganglioma Masquerading as Nasopharyngeal Cancer.

Endocr Pract. 2010 Jan 9;:1-17

Authors: Alzahrani AS, Alshaikh O, Faiyaz-Ul-Haque M, Abalkhail H, Al-Dayel F, Al Hindi H

Context: Multiple paraganglioma syndromes (PGL) are rare and their presentations are variable.Objective: To report a challenging case of PGL and describe the underlying genetic mutation and response to iodine-131-meta-iodobenzylguanidine (MIBG) therapy.Results: A 38-year-old man presented in May 2005 with pseudobulbar palsy and was initially thought to have nasopharyngeal cancer as a CT scan of the head showed a large locally invasive nasopharyngeal tumor. During the staging of the tumor, an abdominal CT scan showed a large locally invasive left adrenal tumor. Urinary normetanephrine was elevated at > 39830.5 ug/day, while metanephrine was normal. MIBG scan was positive at the left adrenal and the skull mass. Biopsy of the nasopharyngeal mass confirmed the diagnosis of paraganglioma. The patient underwent resection of the 13-cm left pheochromocytoma with resection of part of the colon and kidney. Urine normetanephrine dropped to 9338.5 ug/day postoperatively. The nasopharyngeal paraganglioma was inoperable. He was treated with 3 doses of MIBG 201, 190 and 225 mCi in August 2007, January 2008 and January 2009, respectively. Urine normetanephrines normalized and a follow-up MRI showed a 60% reduction in the nasopharyngeal tumor. Genetic testing revealed a C to T transition at nucleotide 268 in exon 3 of succinate dehydrogenase type B (SDHB) resulting in a change in an arginine to a termination codon (R90X) with a truncated SDHB.Conclusion: The case illustrates the diagnostic and therapeutic challenges and the value of genetic testing in PGL. It also shows the effectiveness of MIBG therapy for inoperable paragangliomas.

PMID: 20061288 [PubMed - as supplied by publisher]

Sustained remission with the kinase inhibitor sorafenib in Stage IV metastatic adrenocortical carcinoma.

Fetched: January 20th, 2010, 9:02am CST

Sustained remission with the kinase inhibitor sorafenib in Stage IV metastatic adrenocortical carcinoma.

Endocr Pract. 2009 Nov 26;:1-19

Authors: Butler C, Butler WM, Rizvi AA

Objective: To report our experience with the use of kinase inhibition therapy with sorafenib in a patient with advanced adrenocortical carcinoma.Methods: A 56 year old female presented with rapid development of virilization, cushingoid features, hypertension, weight gain, abdominal distension. An 8 cm left adrenal lesion was found on imaging, removed surgically, and confirmed as adrenal carcinoma on pathology. Postoperative scanning revealed metastases to both lungs and the liver that were confirmed by fine-needle biopsy, thus establishing Stage IV disease.Results: Treatment with the adrenolytic agent mitotane failed to halt disease progression. A trial of sorafenib resulted in regression and eventual resolution of bilateral metastatic lung lesions and reduction in size of hepatic lesion, normalization of androgen hypersecretion, and marked clinical improvement. The radiological and biochemical remission on sorafenib has continued for 28 months.Conclusion: Adrenocortical carcinoma is a rare endocrine malignancy that carries an extremely poor prognosis when discovered in an advanced stage. Traditional medications seldom afford long-term control. Multiple kinase inhibitors such as sorafenib provide targeted oncologic treatment and may be effective in advanced adrenal cancer. The challenge in ongoing and future studies is to identify patients who would have an increased likelihood of a favorable response to these agents.

PMID: 20061282 [PubMed - as supplied by publisher]

Radiating abdominal pain and hypertension.

Fetched: January 20th, 2010, 9:02am CST

Radiating abdominal pain and hypertension.

Am J Med. 2009 Dec;122(12):1103-5

Authors: Ghuman N, Tendler B, Gjertson CK, Kaur M, Moskowitz H, White WB

PMID: 19958888 [PubMed - indexed for MEDLINE]

[Pheochromocytoma with retroperitoneal hemorrhage after abdominal trauma]

Fetched: January 20th, 2010, 9:02am CST

[Pheochromocytoma with retroperitoneal hemorrhage after abdominal trauma]

Hinyokika Kiyo. 2009 Nov;55(11):703-6

Authors: Hayashi T, Nin M, Yamamoto Y, Kamoto A, Ujike T, Nishimura K, Miyoshi S

A 35-year-old man was delivered to the emergency room complaining of right flank pain because of blunt abdominal trauma sustained while playing baseball. Enhanced computed tomography (CT) revealed a right adrenal mass and fluid collection around the mass. We diagnosed the mass as pheochromocytoma by endocrinological examination and radioisotopical imaging test. After absorption of the hematoma three months after the injury, laparoscopic right adrenalectomy was performed. He had an uncomplicated postoperative course without supplementation of catecholamine. Pathological findings were compatible with pheochromocytoma. Eight months after the operation, he had no evidence of recurrence.

PMID: 19946189 [PubMed - indexed for MEDLINE]

Ocular paraneoplastic syndrome: a case of bilateral diffuse uveal melanocytic proliferation.

Fetched: January 20th, 2010, 9:02am CST

Ocular paraneoplastic syndrome: a case of bilateral diffuse uveal melanocytic proliferation.

Retina. 2009 Oct;29(9):1375-6

Authors: Mandelcorn E, McGuire K, Dixon W, Mandelcorn M

PMID: 19934829 [PubMed - indexed for MEDLINE]

[Recent data in adrenocortical tumorigenesis]

Fetched: January 20th, 2010, 9:02am CST

[Recent data in adrenocortical tumorigenesis]

Ann Endocrinol (Paris). 2009 Sep;70 Suppl 1:S20-5

Authors: Crand A, Borson-Chazot F, Brue T

Adrenocortical carcinomas are rare tumors characterized by an aggressive behaviour with a 5-year survival rate below 30%. Until now, surgery is the only curative treatment for tumors confined to the adrenal gland and there is a lack of an effective medical treatment for invasive or metastatic tumors due to the poor knowledge of the mechanisms underlying adrenocortical malignancy. Moreover, histopathology is sometimes insufficient to establish an accurate diagnosis between a benign and a malignant adrenal tumor and a poor indicator of prognosis. In the last decade, the study of rare genetic syndromes associated with adrenocortical carcinomas and the identification of genetic alterations in adrenal tumors has improved our understanding of the pathogenesis of adrenal tumors. The development of molecular predictors of malignancy and of survival could help for histological diagnosis and determination of prognosis. These significant advances are essential to improve adrenocortical carcinoma management. This review summarizes recent advances in the understanding and management of adrenocortical tumors.

PMID: 19878765 [PubMed - indexed for MEDLINE]

A young woman with metastatic solid pseudopapillary tumour of the pancreas.

Fetched: January 20th, 2010, 9:02am CST

A young woman with metastatic solid pseudopapillary tumour of the pancreas.

ANZ J Surg. 2009 Oct;79(10):750-1

Authors: Li WH, Chan C, Cheung MT

PMID: 19878173 [PubMed - indexed for MEDLINE]

Clinical predictors and algorithm for the genetic diagnosis of pheochromocytoma patients.

Fetched: January 20th, 2010, 9:02am CST

Clinical predictors and algorithm for the genetic diagnosis of pheochromocytoma patients.

Clin Cancer Res. 2009 Oct 15;15(20):6378-85

Authors: Erlic Z, Rybicki L, Peczkowska M, Golcher H, Kann PH, Brauckhoff M, Müssig K, Muresan M, Schäffler A, Reisch N, Schott M, Fassnacht M, Opocher G, Klose S, Fottner C, Forrer F, Plöckinger U, Petersenn S, Zabolotny D, Kollukch O, Yaremchuk S, Januszewicz A, Walz MK, Eng C, Neumann HP,

PURPOSE: Six pheochromocytoma susceptibility genes causing distinct syndromes have been identified; approximately one of three of all pheochromocytoma patients carry a predisposing germline mutation. When four major genes (VHL, RET, SDHB, SDHD) are analyzed in a clinical laboratory, costs are approximately $3,400 per patient. The aim of the study is to systematically obtain a robust algorithm to identify who should be genetically tested, and to determine the order in which genes should be tested. EXPERIMENTAL DESIGN: DNA from 989 apparently nonsyndromic patients were scanned for germline mutations in the genes VHL, RET, SDHB, SDHC, and SDHD. Clinical parameters were analyzed as potential predictors for finding mutations by multiple logistic regression, validated by bootstrapping. Cost reduction was calculated between prioritized gene testing compared with that for all genes. RESULTS: Of 989 apparently nonsyndromic pheochromocytoma cases, 187 (19%) harbored germline mutations. Predictors for presence of mutation are age <45 years, multiple pheochromocytoma, extra-adrenal location, and previous head and neck paraganglioma. If we used the presence of any one predictor as indicative of proceeding with gene testing, then 342 (34.6%) patients would be excluded, and only 8 carriers (4.3%) would be missed. We were also able to statistically model the priority of genes to be tested given certain clinical features. E.g., for patients with prior head and neck paraganglioma, the priority would be SDHD>SDHB>RET>VHL. Using the clinical predictor algorithm to prioritize gene testing and order, a 44.7% cost reduction in diagnostic process can be achieved. CONCLUSIONS: Clinical parameters can predict for mutation carriers and help prioritize gene testing to reduce costs in nonsyndromic pheochromocytoma presentations.

PMID: 19825962 [PubMed - indexed for MEDLINE]

A frailty model approach for regression analysis of multivariate current status data.

Fetched: January 20th, 2010, 9:02am CST

A frailty model approach for regression analysis of multivariate current status data.

Stat Med. 2009 Nov 30;28(27):3424-36

Authors: Chen MH, Tong X, Sun J

This paper discusses regression analysis of multivariate current status failure time data (The Statistical Analysis of Interval-censoring Failure Time Data. Springer: New York, 2006), which occur quite often in, for example, tumorigenicity experiments and epidemiologic investigations of the natural history of a disease. For the problem, several marginal approaches have been proposed that model each failure time of interest individually (Biometrics 2000; 56:940-943; Statist. Med. 2002; 21:3715-3726). In this paper, we present a full likelihood approach based on the proportional hazards frailty model. For estimation, an Expectation Maximization (EM) algorithm is developed and simulation studies suggest that the presented approach performs well for practical situations. The approach is applied to a set of bivariate current status data arising from a tumorigenicity experiment.

PMID: 19739240 [PubMed - indexed for MEDLINE]

Suprarenal symplastic leiomyoma of the inferior vena cava.

Fetched: January 20th, 2010, 9:02am CST

Suprarenal symplastic leiomyoma of the inferior vena cava.

Ann Vasc Surg. 2009 Nov-Dec;23(6):786.e11-3

Authors: Kepenekci I, Demirkan A, Sözener U, Cakmak A, Demirer S, Alaçayir I, Ekinci C

We report on a case of a leiomyoma in the inferior vena cava that appeared in the image to be located in the adrenal gland. En bloc excision of the tumor with the right adrenal gland and the involved segment of the vena cava was carried out. Histopathological work-up of the tumor revealed smooth muscle fibers and marked nuclear pleomorphism consistent with symplastic leiomyoma. This case report presents a distinct histological variant of the rarely seen primary smooth muscle tumor of the inferior vena cava.

PMID: 19733033 [PubMed - indexed for MEDLINE]

Permalink for 'Sudden cardiac arrest after minor abdominal trauma: a successful resuscitation in a patient with haemorrhagic phaeochromocytoma.'

Sudden cardiac arrest after minor abdominal trauma: a successful resuscitation in a patient with haemorrhagic phaeochromocytoma.

Fetched: January 20th, 2010, 9:02am CST

Sudden cardiac arrest after minor abdominal trauma: a successful resuscitation in a patient with haemorrhagic phaeochromocytoma.

Resuscitation. 2009 Nov;80(11):1323-4

Authors: Chiu CC, Chen YC, Teng TH, Yang LH, Chen YP, Siao FY

PMID: 19713024 [PubMed - indexed for MEDLINE]

Role of adrenal gland scintigraphy in patients with subclinical hypercortisolism and incidentally discovered adrenal mass.

Fetched: January 20th, 2010, 9:02am CST

Role of adrenal gland scintigraphy in patients with subclinical hypercortisolism and incidentally discovered adrenal mass.

J Endocrinol Invest. 2009 Jul;32(7):576-80

Authors: Donadio F, Morelli V, Salcuni AS, Eller-Vainicher C, Carletto M, Castellani M, Dellavedova L, Scillitani A, Beck-Peccoz P, Chiodini I

BACKGROUND: The role of adrenal scintigraphy (AS) in the diagnosis of subclinical hypercortisolism (SH) in adrenal incidentaloma (AI) is debated. AIM: To evaluate the possible role of AS in identifying SH in AI patients. SUBJECTS AND METHODS: In the retrospective phase (2000-2004), 102 AI patients [71 females (F)/31 males (M)] referred to our center were reevaluated for SH. In the prospective phase (2005-2006), 42 patients (32F/10M) with suspected SH were evaluated performing AS and biochemical assessment of cortisol secretion. We report data of the prospective phase of the study. In these patients AS was performed at baseline; the difference between the uptake of the affected and the controlateral gland [mean Delta uptake (MDeltau)] was calculated. Cortisol secretion was evaluated in 3 different occasions. Patients were considered affected with SH if they presented at least twice 2 of the following criteria: urinary free cortisol >193 nmol/l, cortisol after 1 mg dexamethasone suppression test >83 nmol/l, ACTH levels <2.2 pmol/l. RESULTS: MDeltau was higher in patients with SH (no.=27, 5/22 M/F) than in patients without SH (83.7+/-12.5 vs 54.7+/-24.1%, p<0.001), and directly correlated with UFC (beta=0.387, p=0.015) and was predictive of SH (odds ratio 1.12, 95% confidence interval 1.03-1.22, p=0.009) regardless of age, body mass index, and diameter of the mass. A 75% MDeltau cut-off has 86.7% specificity and 81.5% sensitivity in diagnosing SH. CONCLUSIONS: AS is not recommended as screening test in AI patients, but it can be useful to exclude the presence of a subtle cortisol excess in patients with unclear biochemical diagnosis of SH.

PMID: 19535891 [PubMed - indexed for MEDLINE]

Moderate to severe psoriasis: from topical to biological treatment.

Fetched: January 20th, 2010, 9:02am CST

Moderate to severe psoriasis: from topical to biological treatment.

J Eur Acad Dermatol Venereol. 2009 Nov;23(11):1324-6

Authors: Gardinal I, Ammoury A, Paul C

INTRODUCTION: The simple use of topical corticosteroids in the treatment of severe psoriasis is often inefficient and harmful. The first line of treatment in these cases is based on systemic therapies such as methotrexate, cyclosporin and phototherapy. Later on, biological treatments can be used. OBSERVATIONS: We present three cases of severe psoriasis that have been treated by topical corticosteroids for a long time and with large doses without success and many side-effects. For each one of them, we have introduced either a systemic or a biological treatment with a good efficacy and tolerance. DISCUSSION: Psoriasis has a strong impact on the quality of life and is comparable to patients with major diseases like cancer or depression. Patients who have severe psoriasis are for the most part disappointed and dissatisfied from their treatment. In fact, the misuse of topical corticosteroids for prolonged periods of time may induce local or systemic side-effects without any improvement. Although patients are more pleased with systemic treatments, their use is often limited because physicians are anxious from the systemic side-effects that may occur. CONCLUSION: Systemic treatments are often used too late. Patients are not pleased from the way they are treated. Accordingly, using these treatments earlier may improve patients' quality of life.

PMID: 19470061 [PubMed - indexed for MEDLINE]

SDHB-associated renal oncocytoma suggests a broadening of the renal phenotype in hereditary paragangliomatosis.

Fetched: January 20th, 2010, 9:02am CST

SDHB-associated renal oncocytoma suggests a broadening of the renal phenotype in hereditary paragangliomatosis.

Fam Cancer. 2009;8(3):257-60

Authors: Henderson A, Douglas F, Perros P, Morgan C, Maher ER

Mutations in SDHB are one of the causes of hereditary paraganglioma syndrome. Germline mutations in SDHB predispose to the development of head and neck paragangliomas and phaeochromocytomas. Renal tumours are also increasingly being reported as component tumours in hereditary paragangliomatosis associated with mutations in SDHB. We present the first reported case of a family in whom an individual shown to carry a mutation in SDHB developed a renal oncocytoma. We review other reports of renal tumours associated with SDHB-associated hereditary paragangliomatosis and suggest that various histological subtypes of renal tumours are part of this condition. This observation indicates that SDHB-associated hereditary paragangliomatosis is unlike most tumour predisposition syndromes associated with the development of renal tumours which are usually associated with specific histological sub-types. The increasing recognition of the involvement of renal tumours in SDHB mutation carriers suggests that renal screening is likely to be valuable for these patients. SDHB mutations should also be considered in the context of genetic testing when renal tumours, regardless of histopathology, present in families with other tumours consistent hereditary paraganglioma syndrome.

PMID: 19184535 [PubMed - indexed for MEDLINE]

Targeting DAX-1 in embryonic stem cells and cancer.

Fetched: January 10th, 2010, 2:29pm CST

Targeting DAX-1 in embryonic stem cells and cancer.

Expert Opin Ther Targets. 2010 Feb;14(2):169-77

Authors: Lalli E, Alonso J

IMPORTANCE OF THE FIELD: DAX-1 (NR0B1) is an unusual orphan nuclear receptor whose function is essential for the development of the human adrenal cortex and onset of puberty. Recent data have implicated this transcription factor also in embryonic stem cell and cancer biology. AREAS COVERED IN THIS REVIEW: The role of DAX-1 in the regulation of development and function of the adrenal cortex, reproductive axis, embryonic stem cells and a few types of cancer. WHAT THE READER WILL GAIN: Here we review the past and present milestones in DAX-1 research and try to provide hints about the development and fields of application of DAX-1-targeted drugs in the future. TAKE HOME MESSAGE: The unusual structure and restricted expression pattern of DAX-1 may offer unique opportunities for drug discovery.

PMID: 20055716 [PubMed - in process]

Metastases with hypoadrenalism: diagnostic parallels and diagnostic traps.

Fetched: January 10th, 2010, 2:29pm CST

Metastases with hypoadrenalism: diagnostic parallels and diagnostic traps.

South Med J. 2009 Dec;102(12):1280-1

Authors: Jolobe OM

PMID: 20016446 [PubMed - indexed for MEDLINE]

Virilizing adrenal adenoma in a 20 month-old girl.

Fetched: January 10th, 2010, 2:29pm CST

Virilizing adrenal adenoma in a 20 month-old girl.

J Pediatr Endocrinol Metab. 2009 Sep;22(9):779-80

Authors: Quitos JB, Salas M

PMID: 19960887 [PubMed - indexed for MEDLINE]

Clinical spectrum of pheochromocytoma.

Fetched: January 10th, 2010, 2:29pm CST

Clinical spectrum of pheochromocytoma.

J Am Coll Surg. 2009 Dec;209(6):727-32

Authors: Guerrero MA, Schreinemakers JM, Vriens MR, Suh I, Hwang J, Shen WT, Gosnell J, Clark OH, Duh QY

BACKGROUND: Pheochromocytomas vary in presentation, tumor size, and in catecholamine production. Whether pheochromocytoma size correlates with hormone levels, clinical presentation, and perioperative complications is not known. The goal of this study was to determine if tumor size and hormone level correlate according to the clinical presentation at diagnosis. STUDY DESIGN: We retrospectively analyzed all patients who underwent an adrenalectomy with a diagnosis of a pheochromocytoma from February 1996 to October 2008. We grouped patients according to their clinical presentation at diagnosis (routine biochemical screening, incidentaloma, classic symptoms, pheochromocytoma crisis) and obtained preoperative radiographic tumor size and catecholamine hormone levels. ANOVA was used for the group effects and the Kruskal-Wallis rank test was used for pairwise comparison between groups with the Sidak/Bonferroni method for multiplicity adjustment according to age, tumor size, and hormone level. The Pearson correlation coefficient was then calculated to determine if hormone level correlated with tumor size. RESULTS: Eighty-one of 107 patients had data available for complete analysis. The average age at diagnosis for all patients was 47.1 years, and the average tumor size was 4.9 cm. The average highest hormone ratio among all patients was 27.4. Tumor size and hormone ratio levels differed among all groups (p < or = 0.03). A direct correlation (p = 0.014) was apparent between tumor size and hormone level. Complication rates also differed among the four groups of patients (p < or = 0.02). CONCLUSIONS: Our study showed that tumor size directly correlates with hormone level. Smaller tumors tend to secrete lower levels of catecholamines, but larger tumors have a wider variation in secretory potential. Larger tumors, however, produced the highest hormone ratios.

PMID: 19959041 [PubMed - indexed for MEDLINE]

Permalink for 'Clinical features and treatment outcomes of non-Hodgkin's lymphomas involving rare extranodal sites: a single-center experience.'

Clinical features and treatment outcomes of non-Hodgkin's lymphomas involving rare extranodal sites: a single-center experience.

Fetched: January 10th, 2010, 2:29pm CST

Clinical features and treatment outcomes of non-Hodgkin's lymphomas involving rare extranodal sites: a single-center experience.

Acta Haematol. 2010;123(1):48-54

Authors: Yun J, Kim SJ, Kim JA, Kong JH, Lee SH, Kim K, Ko YH, Kim WS

BACKGROUND: The involvement of certain organs such as the adrenal gland and ovaries is rare in non-Hodgkin's lymphoma (NHL). There are few studies comparing clinical features and prognosis based on the extranodal organ involved. METHODS: We selected patients presenting with predominantly extranodal involvement among patients diagnosed with NHL from 1998 to 2009 at the Samsung Medical Center. RESULTS: Forty-eight patients with NHL involving rare extranodal sites were analyzed. The extranodal sites were as follows: adrenal gland (n = 14), ovary (n = 13), pancreas (n = 11), uterus (n = 4), esophagus (n = 4) and prostate (n = 2). Diffuse large B-cell lymphoma (DLBCL) was the most common (n = 39), and the median overall survival (OS) was 16.63 months. There was no significant difference in OS according to the involved sites. Rituximab plus CHOP failed to provide an additive survival benefit over CHOP alone in 39 DLBCL patients. The OS of DLBCL in rare extranodal sites was worse than that in common sites when compared based on tumor stage. CONCLUSIONS: NHL involving rare extranodal sites had a poor prognosis, and the impact of rituximab on survival was negligible. Thus, more intensive therapeutic strategies should be considered for NHL involving rare extranodal sites.

PMID: 19955711 [PubMed - indexed for MEDLINE]

Anemia and infections in multiple myeloma: supportive therapy.

Fetched: January 10th, 2010, 2:29pm CST

Anemia and infections in multiple myeloma: supportive therapy.

Bol Asoc Med P R. 2009 Apr-Jun;101(2):50-2

Authors: Cáceres W, Santiago K, Paulo L, Román J

Patients with multiple myeloma (MM) have a high rate of infectious complications and symptomatic anemia. Emerging antineoplastic therapies are resulting in a better response, but still quality of life and potentially survival can be significantly affected by these complications. Multiple cytokines and an impaired immunologic system are involved in the development of these complications. Emerging therapies and guidelines will be reviewed taking in consideration the benefits versus risks of those strategies. The beneficial effect of recombinant human erythropoietin (EPO) and darbepoietin alpha in MM patients have been confirmed in several trials. Evidenced-based guidelines for the use of EPO in patients with cancer should be closely followed to ensure correct use of erythropoiesis stimulating agents. Infection, despite being the most common cause of morbidity and mortality, can be controlled by prompt recognition and proper prophylaxis.Supportive care is an important adjunct to cancer therapy. Adequate use of pharmacologic strategies to control anemia and prevent or treat infectious complications will result in benefit to MM patients.

PMID: 19954102 [PubMed - indexed for MEDLINE]

Diffusion-weighted imaging of the renal and adrenal lesions.

Fetched: January 10th, 2010, 2:29pm CST

Diffusion-weighted imaging of the renal and adrenal lesions.

J Comput Assist Tomogr. 2009 Nov-Dec;33(6):828-33

Authors: Kilickesmez O, Inci E, Atilla S, Tasdelen N, Yetimoğlu B, Yencilek F, Gurmen N

OBJECTIVES: The purpose of this study was to calculate the apparent diffusion coefficient (ADC) values of different renal and adrenal lesions to evaluate the ability of diffusion-weighted imaging in characterizing masses and determining malignancy. METHODS: A total of 52 patients consisting of 67 renal lesions and 28 patients with 33 adrenal lesions in addition to 50 healthy controls with normal kidneys were enrolled in the study. Diffusion-weighted imaging was performed with b factors of 0, 500, and 1000 s/mm2, and the ADCs of the normal kidney and renal and adrenal lesions were calculated. RESULTS: The mean (SD) ADCs of the renal cortex and medulla of the control group were 2.08 (0.22) x 10(-3) and 1.94 (0.18) x 10(-3) mm2/s, respectively. Focal renal lesions were as follows: simple cysts (2.94 [0.20] x 10(-3) mm2/s), hemorrhagic cysts (1.71 [0.38] x 10(-3) mm2/s), angiomyolipomas (1.40 [0.21] x 10(-3) mm2/s), renal cell carcinomas (1.06 [0.39] x 10(-3) mm2/s), metastases (1.50 [0.13] x 10(-3) mm2/s), and hydronephrosis (1.54 [0.25] x 10(-3) mm2/s). The mean ADCs of all these pathologies were significantly different when compared with normal parenchyma. Diffusion-weighted imaging was also able to differentiate angiomyolipomas and hemorrhagic cysts from renal cell carcinomas. Adrenal lesions were subgrouped as adenomas (1.41 [0.27] x M10(-3) mm2/s), nonadenomatous solid masses (1.08 [0.28] x 10(-3) mm2/s), and cysts (2.82 [0.24] x 10(-3) mm2/s). The mean ADCs of adenomas were significantly different when compared with nonadenomatous solid masses and cysts. CONCLUSIONS: Our findings show that ADC measurement has a potential ability to differentiate benign and malignant focal renal and adrenal lesions with the guidance of conventional sequences. When used alone, diffusion-weighted imaging may lead to misdiagnoses due to overlapping ADCs of the lesions.

PMID: 19940645 [PubMed - indexed for MEDLINE]

Permalink for 'Coexistence of a giant splenic hemangioma and multiple hepatic hemangiomas mimicking a left adrenal neuroblastoma accompanied with multifocal hepatic metastases: pyrite answer.'

Coexistence of a giant splenic hemangioma and multiple hepatic hemangiomas mimicking a left adrenal neuroblastoma accompanied with multifocal hepatic metastases: pyrite answer.

Fetched: January 10th, 2010, 2:29pm CST

Coexistence of a giant splenic hemangioma and multiple hepatic hemangiomas mimicking a left adrenal neuroblastoma accompanied with multifocal hepatic metastases: pyrite answer.

J Pediatr Hematol Oncol. 2009 Dec;31(12):983-4

Authors: Wang J, Pei G, Yan J, Zhang G

A 4-month girl presenting with a giant mass in left adrenal region and multiple hepatic nodules was hospitalized. On the basis of computed tomography, ultrasound and epidemiology we preferred the diagnosis of a left adrenal neuroblastoma accompanied with multiple hepatic metastases preoperatively. But postoperative diagnosis was hemangiomatosis characterized by synchronous presence of a giant splenic hemangioma and multiple hepatic hemangiomas. We think that it is deservedly reported because of not only its rarity but also pitfalls of preoperative differential diagnosis and management principle.

PMID: 19935098 [PubMed - indexed for MEDLINE]

Pheochromocytoma in pediatric age: a surgical urgency?

Fetched: January 10th, 2010, 2:29pm CST

Pheochromocytoma in pediatric age: a surgical urgency?

Am J Emerg Med. 2009 Nov;27(9):1161

Authors: Mazzei A, Stranieri G, Rubino R, Centonze A, Porta IP, Siani A, Baldassarre E

PMID: 19931771 [PubMed - indexed for MEDLINE]

Outcomes from 3144 adrenalectomies in the United States: which matters more, surgeon volume or specialty?

Fetched: January 10th, 2010, 2:29pm CST

Outcomes from 3144 adrenalectomies in the United States: which matters more, surgeon volume or specialty?

Arch Surg. 2009 Nov;144(11):1060-7

Authors: Park HS, Roman SA, Sosa JA

OBJECTIVE: To assess the effect of surgeon volume and specialty on clinical and economic outcomes after adrenalectomy. DESIGN: Population-based retrospective cohort analysis. SETTING: Healthcare Cost and Utilization Project Nationwide Inpatient Sample. PARTICIPANTS: Adults (>or=18 years) undergoing adrenalectomy in the United States (1999-2005). Patient demographic and clinical characteristics, surgeon specialty (general vs urologist), surgeon adrenalectomy volume, and hospital factors were assessed. MAIN OUTCOME MEASURES: The chi(2) test, analysis of variance, and multivariate linear and logistic regression were used to assess in-hospital complications, mean hospital length of stay (LOS), and total inpatient hospital costs. RESULTS: A total of 3144 adrenalectomies were included. Mean patient age was 53.7 years; 58.8% were women and 77.4% white. A higher proportion of general surgeons were high-volume surgeons compared with urologists (34.1% vs 18.2%, P < .001). Low-volume surgeons had more complications (18.2% vs 11.3%, P < .001) and their patients had longer LOS (5.5 vs 3.9 days, P < .001) than did high-volume surgeons; urologists had more complications (18.4% vs 15.2%, P = .03) and higher costs ($13,168 vs $11,732, P = .02) than did general surgeons. After adjustment for patient and provider characteristics in multivariate analyses, surgeon volume, but not specialty, was an independent predictor of complications (odds ratio = 1.5, P < .002) and LOS (1.0-day difference, P < .001). Hospital volume was associated only with LOS (0.8-day difference, P < .007). Surgeon volume, specialty, and hospital volume were not predictors of costs. CONCLUSION: To optimize outcomes, patients with adrenal disease should be referred to surgeons based on adrenal volume and laparoscopic expertise irrespective of specialty practice.

PMID: 19917944 [PubMed - indexed for MEDLINE]

[How to diagnose and how to treat diseases of the genital mucosa?]

Fetched: January 10th, 2010, 2:29pm CST

[How to diagnose and how to treat diseases of the genital mucosa?]

Rev Med Brux. 2009 Sep;30(4):385-91

Authors: Parent D

The genital area in women is covered by a keratinized squamous stratified epithelium outside the body (vulva), and a non keratinized epithelium inside the body (vagina). These characteristics can have an effect on the clinical aspects of the diseases and/or on the choice of the treatment. Symptoms (itching, pain, vaginal discharge), preferential localisation of skin diseases (psoriasis, lichen planus, lichen sclerosus, atopic dermatitis and allergic contact dermatitis, irritative dermatitis) and the aspect of primary lesions are to be investigated. The implication of this region in sexual activity places it at risk of sexually transmitted diseases (STD's) and dyspareunia. These have numerous causes that have to be sought and taken care of, often by multidisciplinary teams. After a careful history and clinical examination, additional tests allow to exclude infections or confirm a skin condition or neoplasia by a skin biopsy. If contact dermatitis is suspected, specific allergy testing is done. Treatment starts with correction of harmful habits (excessive use of soaps, inappropriate cosmetic products,...) that add to the local irritation. Patients are then reassured of common misconception regarding cancer, STD's and fertility. In the vast majority of cases, the treatment will target an infection (fungal, bacterial, STD's), will relieve irritation by the use of local immunosuppressant drugs (local corticosteroids) and/or relief itching symptoms with anti-histamine drugs.

PMID: 19899385 [PubMed - indexed for MEDLINE]

Life-threatening bilateral adrenal cystic neuroblastoma in an infant.

Fetched: January 10th, 2010, 2:29pm CST

Life-threatening bilateral adrenal cystic neuroblastoma in an infant.

J Pediatr Hematol Oncol. 2009 Dec;31(12):963-4

Authors: Avanzini S, Conte M, Granata C, Zamorani EM, Sementa AR, Garaventa A, Buffa P, Sorrentino S

A case of neonatal bilateral adrenal mass causing severe respiratory distress and requiring an emergency debulking surgical procedure is reported. Histopathology revealed a cystic neuroblastoma stroma poor, poorly differentiated, without MYCN amplification and 1p deletion. During postoperative follow-up, a progression to stage 4s was observed, characterized by liver involvement. According to good prognostic indexes, no further treatment was administered. Both adrenal masses and hepatic nodules showed progressive decrease in size, till complete disappearance. The authors encourage a multidisciplinary approach to develop the best patient-related strategy for cystic neuroblastoma, thus reducing complications rate.

PMID: 19887960 [PubMed - indexed for MEDLINE]

Radiofrequency ablation of metastatic pheochromocytoma.

Fetched: January 10th, 2010, 2:29pm CST

Radiofrequency ablation of metastatic pheochromocytoma.

J Vasc Interv Radiol. 2009 Nov;20(11):1483-90

Authors: Venkatesan AM, Locklin J, Lai EW, Adams KT, Fojo AT, Pacak K, Wood BJ

In the present report on the preliminary safety and effectiveness of radiofrequency (RF) ablation for pheochromocytoma metastases, seven metastases were treated in six patients (mean size, 3.4 cm; range, 2.2-6 cm). alpha- and beta-adrenergic and catecholamine synthesis inhibition and intraprocedural anesthesia monitoring were used. Safety was assessed by recording ablation-related complications. Complete ablation was defined as a lack of enhancement within the ablation zone on follow-up computed tomography. No serious adverse sequelae were observed. Complete ablation was achieved in six of seven metastases (mean follow-up, 12.3 months; range, 2.5-28 months). In conclusion, RF ablation may be safely performed for metastatic pheochromocytoma given careful attention to peri-procedural management.

PMID: 19875067 [PubMed - indexed for MEDLINE]

Permalink for 'Comparison of 18F-fluoro-L-DOPA, 18F-fluoro-deoxyglucose, and 18F-fluorodopamine PET and 123I-MIBG scintigraphy in the localization of pheochromocytoma and paraganglioma.'

Comparison of 18F-fluoro-L-DOPA, 18F-fluoro-deoxyglucose, and 18F-fluorodopamine PET and 123I-MIBG scintigraphy in the localization of pheochromocytoma and paraganglioma.

Fetched: January 10th, 2010, 2:29pm CST

Comparison of 18F-fluoro-L-DOPA, 18F-fluoro-deoxyglucose, and 18F-fluorodopamine PET and 123I-MIBG scintigraphy in the localization of pheochromocytoma and paraganglioma.

J Clin Endocrinol Metab. 2009 Dec;94(12):4757-67

Authors: Timmers HJ, Chen CC, Carrasquillo JA, Whatley M, Ling A, Havekes B, Eisenhofer G, Martiniova L, Adams KT, Pacak K

CONTEXT: Besides (123)I-metaiodobenzylguanidine (MIBG), positron emission tomography (PET) agents are available for the localization of paraganglioma (PGL), including (18)F-3,4-dihydroxyphenylalanine (DOPA), (18)F-fluoro-2-deoxy-D-glucose ((18)F-FDG), and (18)F-fluorodopamine ((18)F-FDA). OBJECTIVE: The objective of the study was to establish the optimal approach to the functional imaging of PGL and examine the link between genotype-specific tumor biology and imaging. DESIGN: This was a prospective observational study. INTERVENTION: There were no interventions. PATIENTS: Fifty-two patients (28 males, 24 females, aged 46.8 +/- 14.2 yr): 20 with nonmetastatic PGL (11 adrenal), 28 with metastatic PGL (13 adrenal), and four in whom PGL was ruled out; 22 PGLs were of the succinate dehydrogenase subunit B (SDHB) genotype. MAIN OUTCOME MEASURES: Sensitivity of (18)F-DOPA, (18)F-FDG, and (18)F-FDA PET, (123)I-MIBG scintigraphy, computed tomography (CT), and magnetic resonance imaging (MRI) for the localization of PGL were measured. RESULTS: Sensitivities for localizing nonmetastatic PGL were 100% for CT and/or MRI, 81% for (18)F-DOPA PET, 88% for (18)F-FDG PET/CT, 78% for (18)F-FDA PET/CT, and 78% for (123)I-MIBG scintigraphy. For metastatic PGL, sensitivity in reference to CT/MRI was 45% for (18)F-DOPA PET, 74% for (18)F-FDG PET/CT, 76% for (18)F-FDA PET/CT, and 57% for (123)I-MIBG scintigraphy. In patients with SDHB metastatic PGL, (18)F-FDA and (18)F-FDG have a higher sensitivity (82 and 83%) than (123)I-MIBG (57%) and (18)F-DOPA (20%). CONCLUSIONS: (18)F-FDA PET/CT is the preferred technique for the localization of the primary PGL and to rule out metastases. Second best, equal alternatives are (18)F-DOPA PET and (123)I-MIBG scintigraphy. For patients with known metastatic PGL, we recommend (18)F-FDA PET in patients with an unknown genotype, (18)F-FDG or (18)F-FDA PET in SDHB mutation carriers, and (18)F-DOPA or (18)F-FDA PET in non-SDHB patients.

PMID: 19864450 [PubMed - indexed for MEDLINE]

Interstitial pneumonia arising in a patient treated with oxaliplatin, 5-fluorouracil, and, leucovorin (FOLFOX).

Fetched: January 10th, 2010, 2:29pm CST

Interstitial pneumonia arising in a patient treated with oxaliplatin, 5-fluorouracil, and, leucovorin (FOLFOX).

Int J Clin Oncol. 2009 Oct;14(5):457-9

Authors: Muneoka K, Shirai Y, Sasaki M, Wakai T, Sakata J, Hatakeyama K

Information concerning the pulmonary toxicity of oxaliplatin with infusional 5-fluorouracil plus leucovorin (FOLFOX) is very limited. We herein report the case of a patient with FOLFOX-induced interstitial pneumonia. An 82-year-old man with unresectable colon cancer liver metastases was referred to our department for chemotherapy with the FOLFOX protocol. After the administration of ten cycles, he visited our outpatient clinic with a 2-week history of coughing and shortness of breath; he was afebrile. A chest radiograph showed reticular shadows with ground-glass opacities mainly involving the middle and lower zones of the right lung. Computed tomography depicted ground-glass opacities with superimposed reticulation in the right lung. A diagnosis of FOLFOX-induced interstitial pneumonia was made based on the clinical course and imaging findings. The symptoms disappeared within 3 days after the cessation of the FOLFOX regimen and the initiation of high-dose corticosteroid treatment. Two months after the initiation of the corticosteroid treatment, complete remission of the radiological abnormalities was confirmed; thereafter, interstitial pneumonia did not recur despite the reintroduction of 5-fluorouracil/leucovorin alone, suggesting that 5-fluorouracil/leucovorin alone was not responsible for the development of the interstitial pneumonia. Thus, oxaliplatin, alone or in combination with 5-fluorouracil/leucovorin, may have caused the interstitial pneumonia in this patient. Once interstitial pneumonia has occurred, cessation of the regimen is mandatory, and high-dose corticosteroid treatment is commonly given to rescue patients from this potentially lethal complication.

PMID: 19856057 [PubMed - indexed for MEDLINE]

The glucocorticoid receptor is overexpressed in malignant adrenocortical tumors.

Fetched: January 10th, 2010, 2:29pm CST

The glucocorticoid receptor is overexpressed in malignant adrenocortical tumors.

J Clin Endocrinol Metab. 2009 Nov;94(11):4591-9

Authors: Tacon LJ, Soon PS, Gill AJ, Chou AS, Clarkson A, Botling J, Stalberg PL, Skogseid BM, Robinson BG, Sidhu SB, Clifton-Bligh RJ

CONTEXT: Adrenocortical carcinoma (ACC) is a rare tumor with a poor prognosis. The Weiss score is the most widely accepted method for distinguishing an ACC from an adrenocortical adenoma (ACA); however, in borderline cases, accurate diagnosis remains problematic. We recently discovered that the glucocorticoid receptor (GR) gene NR3C1 is significantly up-regulated in ACCs compared with ACAs in global gene expression studies. OBJECTIVE: Our objective was to study GR expression in adrenocortical tumors (ACTs) and to assess its utility as an adjunct to the Weiss score. DESIGN: Microarray analysis, real-time quantitative RT-PCR (qPCR), immunohistochemistry, Western blot, and direct sequencing were performed. RESULTS: Analysis of 28 ACTs by microarray and 49 ACTs by qPCR found NR3C1 expression to be up-regulated in ACCs compared with ACAs (P < 0.001). Western blotting and RT-PCR confirmed the presence of the GRalpha isoform in ACCs, and no mutations were detected on direct sequencing. Immunohistochemistry for GR in an overlapping cohort of ACTs demonstrated strongly positive nuclear staining in 31 of 33 ACCs (94%), with negative staining in 40 of 41 ACAs (98%) (P < 0.001). This finding was validated in an external cohort of ACTs, such that 14 of 18 ACCs (78%) demonstrated positive nuclear staining whereas 32 of 33 ACAs (94%) were negative (P < 0.001). CONCLUSIONS: The immunohistochemical finding of nuclear GR staining identified ACCs with high diagnostic accuracy. We propose that GR immunohistochemistry may complement the Weiss score in the diagnosis of ACC in cases that display borderline histology. The possibility that GR is transcriptionally active in these tumors, and may therefore be a therapeutic target, requires further study.

PMID: 19820023 [PubMed - indexed for MEDLINE]

ACTH-producing pheochromocytoma: clinical considerations and concise review of the literature.

Fetched: January 10th, 2010, 2:29pm CST

ACTH-producing pheochromocytoma: clinical considerations and concise review of the literature.

Eur J Intern Med. 2009 Nov;20(7):682-5

Authors: Nijhoff MF, Dekkers OM, Vleming LJ, Smit JW, Romijn JA, Pereira AM

We present a patient with a rare cause of ectopic ACTH-dependent Cushing's syndrome, caused by a pheochromocytoma. The case provides clues for a detailed discussion on the pitfalls and diagnostic difficulties in establishing the correct underlying cause of ACTH-dependent Cushing's syndrome. It clearly demonstrates that the relative contributions of clinical, biochemical, and radiological clues in establishing the correct underlying cause of Cushing's syndrome may differ considerably between Cushing's disease and Cushing's syndrome due to ectopic overproduction of ACTH. In addition, the literature concerning ACTH-producing pheochromocytomas is reviewed.

PMID: 19818286 [PubMed - indexed for MEDLINE]

Permalink for 'Post-surgical hypocortisolism after removal of an adrenal incidentaloma: is it predictable by an accurate endocrinological work-up before surgery?'

Post-surgical hypocortisolism after removal of an adrenal incidentaloma: is it predictable by an accurate endocrinological work-up before surgery?

Fetched: January 10th, 2010, 2:29pm CST

Post-surgical hypocortisolism after removal of an adrenal incidentaloma: is it predictable by an accurate endocrinological work-up before surgery?

Eur J Endocrinol. 2010 Jan;162(1):91-9

Authors: Eller-Vainicher C, Morelli V, Salcuni AS, Torlontano M, Coletti F, Iorio L, Cuttitta A, Ambrosio A, Vicentini L, Carnevale V, Beck-Peccoz P, Arosio M, Ambrosi B, Scillitani A, Chiodini I

OBJECTIVE: Few data are available regarding the need of steroid substitutive therapy after unilateral adrenalectomy for adrenal incidentaloma (AI). It is unknown whether, before surgery, the hypothalamic-pituitary-adrenal (HPA) axis secretion parameters can predict post-surgical hypocortisolism. AIM: This study aimed to evaluate whether, in AI patients undergoing unilateral adrenalectomy, post-surgical hypocortisolism could be predicted by the parameters of HPA axis function. DESIGN: Prospective, multicenter. METHODS: A total of 60 patients underwent surgical removal of AI (surgical indication: 29 subclinical hypercortisolism (SH); 31 AI dimension). Before surgery, SH was diagnosed in patients presenting at least three criteria out of urinary free cortisol (UFC) levels>60 microg/24 h, cortisol after 1-mg dexamethasone suppression test (1 mg-DST)>3.0 microg/dl, ACTH levels<10 pg/ml, midnight serum cortisol (MSC)>5.4 microg/dl. Two months after surgery, HPA axis function was assessed by low dose ACTH stimulation test or insulin tolerance test when needed: 39 patients were affected (Group B) and 21 were not affected (Group A) with hypocortisolism. The accuracy in predicting hypocortisolism of pre-surgical HPA axis parameters or their combinations was evaluated. RESULTS: The presence of >2 alterations among 1 mg-DST>5.0 microg/dl, ACTH<10 pg/ml, elevated UFC and MSC has the highest odds ratio (OR) for predicting post-surgical hypocortisolism (OR 10.45, 95% confidence interval, CI 2.54-42.95, P=0.001). Post-surgical hypocortisolism was predicted with 100% probability by elevated UFC plus MSC levels, but not ruled out even in the presence of the normality of all HPA axis parameters. CONCLUSION: Post-surgical hypocortisolism cannot be pre-surgically ruled out. A steroid substitutive therapy is indicated after unilateral adrenalectomy for SH or size of the adenoma.

PMID: 19797503 [PubMed - indexed for MEDLINE]

Maffucci syndrome and adrenal cortex tumor.

Fetched: January 10th, 2010, 2:29pm CST

Maffucci syndrome and adrenal cortex tumor.

Joint Bone Spine. 2009 Oct;76(5):556-8

Authors: Puech-Bret N, Bret J, Bennet A, Huyghe E, Mazerolles C, Zabraniecki L, Fournie B

We report the second known case of Maffucci syndrome associated with an adrenal cortex tumor. Endocrine tumors have been reported in patients with multiple enchondromas, although the underlying mechanism of this combination is unknown. Maffucci syndrome is characterized by abnormalities of several mesodermal derivates. Therefore, routine evaluation for involvement of the adrenal cortex may be warranted to improve our knowledge of this syndrome and of its pathophysiology.

PMID: 19782627 [PubMed - indexed for MEDLINE]

Radio-frequency ablation of solitary adrenal gland metastasis from renal cell carcinoma.

Fetched: January 10th, 2010, 2:29pm CST

Radio-frequency ablation of solitary adrenal gland metastasis from renal cell carcinoma.

Urology. 2009 Dec;74(6):1341-3

Authors: Mouracade P, Dettloff H, Schneider M, Debras B, Jung JL

OBJECTIVES: To evaluate the early clinical experience associated with radio-frequency (RF) ablation in patients with solitary adrenal gland metastasis of renal cell carcinoma (RCC). METHODS: A total of 5 patients were treated with RF ablation of adrenal gland metastasis from RCC under computed tomography (CT) guidance between 2006 and 2007. Radiotherapeutics generator (Boston Scientific, Natick, MA) and Radiotherapeutics RF ablation probe with "umbrella" array tip (Boston Scientific, Natick, MA) were used. These patients were contraindicated for surgery due to several associated morbidities. All patients underwent a tumor biopsy before RF. The access was percutaneous in all patients. Tumor control was defined as the absence of contrast enhancement in the tumor on CT. Data were collected in a retrospective manner. RESULTS: Five patients were treated with a RF ablation of the adrenal metastasis. The average age of the patients was 69.4 +/- 11.1 years (57-87), the average time between the radical nephrectomy and the onset of adrenal metastasis was 5.2 +/- 1.3 years (4-7), and average diameter of the tumor was 3.9 +/- 1.4 cm (2-6). A year after RF, four patients had a total necrosis of their tumor on CT and 1 had an active tumor for which the patient received a second course of RF. An abscess of the adrenal gland had occurred in 1 case requiring a percutaneous drainage. CONCLUSIONS: The treatment of metastatic adrenal gland with RF broadens the range of treatment of metastatic RCC. The efficacy of this therapeutic modality must be confirmed by larger series with the longest follow-up.

PMID: 19781749 [PubMed - indexed for MEDLINE]

Ventricular fibrillation as the presenting manifestation of adrenocortical carcinoma.

Fetched: January 10th, 2010, 2:29pm CST

Ventricular fibrillation as the presenting manifestation of adrenocortical carcinoma.

Am Heart Hosp J. 2009;7(1):65-6

Authors: Shimony A, Bereza S, Shalev A, Gilutz H, Ilia R, Zahger D

We describe a case of a young adult in whom sudden cardiac death due to ventricular fibrillation was the presenting manifestation of an adrenocortical carcinoma. The arrhythmia was precipitated by severe hypokalemia induced by the aldosterone-secreting tumor. Sudden death has not been previously described as a manifestation of this adrenal neoplasm. Unexplained persistent hypokalemia after resuscitated sudden death (especially when combined with hypertension( should prompt investigation for an underlying secondary hypertension, particularly adrenal pathology. Adrenocortical carcinoma should be considered in the differential diagnosis of unexplained sudden death associated with unexplained hypokalemia.

PMID: 19742438 [PubMed - indexed for MEDLINE]

Permalink for 'p53 Tetramerization domain mutations: germline R342X and R342P, and somatic R337G identified in pediatric patients with Li-Fraumeni syndrome and a child with adrenocortical carcinoma.'

p53 Tetramerization domain mutations: germline R342X and R342P, and somatic R337G identified in pediatric patients with Li-Fraumeni syndrome and a child with adrenocortical carcinoma.

Fetched: January 10th, 2010, 2:29pm CST

p53 Tetramerization domain mutations: germline R342X and R342P, and somatic R337G identified in pediatric patients with Li-Fraumeni syndrome and a child with adrenocortical carcinoma.

Fam Cancer. 2009;8(4):541-6

Authors: Fiszer-Maliszewska L, Kazanowska B, Padzik J,

Germline p53 mutations are associated with Li-Fraumeni syndrome (LFS) and other familial cancer phenotypes not fulfilling the definition for LFS. The majority of germline p53 mutations cluster in exons 5-8, corresponding to a DNA binding domain. We report the identification of two germline mutations and a somatic mutation in a tetramerization domain (TD), a rare site for mutations. The germline mutation, R342X (16915C>T), and the novel mutation, R342P (16916G>C), were found in a child with adrenocortical carcinoma and in a LFS pediatric patient with multiple primaries. The novel somatic mutation, R337G (16900C>G), was discovered in myelodysplastic syndrome with transformation to acute myeloblastic leukemia, developing as the third primary in the LFS child. These findings add further information on p53 TD mutations and TD contribution to tumorigenesis.

PMID: 19714490 [PubMed - indexed for MEDLINE]

Candidate genes associated with malignant pheochromocytomas by genome-wide expression profiling.

Fetched: January 10th, 2010, 2:29pm CST

Candidate genes associated with malignant pheochromocytomas by genome-wide expression profiling.

Ann Surg. 2009 Dec;250(6):983-90

Authors: Suh I, Shibru D, Eisenhofer G, Pacak K, Duh QY, Clark OH, Kebebew E

OBJECTIVE: To improve our understanding of the molecular mechanisms involved in malignant pheochromocytoma by examining differences in the gene expression profile between benign and malignant tumors. BACKGROUND: The molecular events involved in the malignant transformation of pheochromocytoma are poorly understood. There are also no reliable and uniformly accepted histopathologic criteria to distinguish benign from malignant pheochromocytoma. METHODS: We performed genome-wide expression profiling of 58 pheochromocytomas (29 benign and sporadic, 16 benign and hereditary, 13 malignant) with technical and biologic replication. RESULTS: Unsupervised cluster analysis showed 3 main clusters of tumors that did not have complete concordance with the clinical and pathologic groupings of pheochromocytomas. Supervised cluster analysis showed almost completely separate clustering between benign and malignant tumors. The differentially expressed genes with known function belonged to 8 biologic process categories; signal transduction, transcription, protein transport, protein synthesis, smooth muscle contraction, ion transport, chemotaxis, and electron transport. Gene set enrichment analysis revealed significant correlation between the microarray profiles of malignant pheochromocytomas and several known molecular pathways associated with carcinogenesis and dedifferentiation. Ten differentially expressed genes had high diagnostic accuracy, and 5 of these genes (CFC1, FAM62B, HOMER1, LRRN3, TBX3, ADAMTS) in combination had an area under the receiver operating characteristic (ROC) curve of 0.96 for distinguishing benign versus malignant tumors. CONCLUSIONS: Differentially expressed genes between benign and malignant pheochromocytomas distinguish between these tumors with high diagnostic accuracy. Our findings provide new insight into the genes and molecular pathways that may be involved in malignant pheochromocytomas.

PMID: 19661783 [PubMed - indexed for MEDLINE]

Permalink for 'Mutation screening of VHL gene in a family with malignant bilateral pheochromocytoma: from isolated familial pheochromocytoma to von Hippel-Lindau disease.'

Mutation screening of VHL gene in a family with malignant bilateral pheochromocytoma: from isolated familial pheochromocytoma to von Hippel-Lindau disease.

Fetched: January 10th, 2010, 2:29pm CST

Mutation screening of VHL gene in a family with malignant bilateral pheochromocytoma: from isolated familial pheochromocytoma to von Hippel-Lindau disease.

Fam Cancer. 2009;8(4):465-71

Authors: Hasani-Ranjbar S, Amoli MM, Ebrahim-Habibi A, Haghpanah V, Hejazi M, Soltani A, Larijani B

von Hippel-Lindau (vHL) disease is an inherited, autosomal dominant syndrome manifested by a variety of benign and malignant tumors. More than 300 germline VHL mutations have been identified that are involved in VHL disease. A large family (four generations) was evaluated. In this paper we report the presence of a single nucleotide mutation in exon 3 of VHL gene c499 C>T causing substitution of Arginine by Tryptophan at position 167 (R 167 W). It was detected in a family with bilateral malignant pheochromocytoma who has been followed for at least 9 years as RET negative isolated familial pheochromocytoma, finally diagnosed as von Hipple-Lindau disease according to retinal angioma and VHL gene mutation. VHL type 2 presenting with both pheochromocytoma and retinal angioma in this family found to be associated with the new missense mutation (c499 C>T) of VHL gene.

PMID: 19649731 [PubMed - indexed for MEDLINE]

Characterization of the largest kindred with MEN2A due to a Cys609Ser RET mutation.

Fetched: January 10th, 2010, 2:29pm CST

Characterization of the largest kindred with MEN2A due to a Cys609Ser RET mutation.

Fam Cancer. 2009;8(4):379-82

Authors: Mian C, Barollo S, Zambonin L, Pennelli G, Bernante P, Pelizzo MR, Nacamulli D, Mantero F, Girelli ME, Opocher G

RET codon 609 point mutations are rare and may predispose to aggressive medullary thyroid carcinoma (MTC). In a kindred with 15 carriers of the Cys609Ser RET mutation we observed no MTC before 17 years of age, no lymph node metastases before 30 years and no distant metastases before 60 years. Two patients developed pheochromocytoma and one had primary hyperparathyroidism as the first sign of the syndrome. In conclusion, at variance from what already known, in this large kindred the Cys609Ser RET mutation predispose to a scarcely aggressive, highly penetrant MTC and a low penetrance of pheochromocytoma and primary hyperparathyroidism.

PMID: 19475497 [PubMed - indexed for MEDLINE]

Adrenocortical adenoma expressing glucocorticoid in an 8-month-old female.

Fetched: January 10th, 2010, 2:29pm CST

Adrenocortical adenoma expressing glucocorticoid in an 8-month-old female.

Eur J Pediatr Surg. 2009 Aug;19(4):265-7

Authors: Kim E, Aguayo P, St Peter SD, Holcomb GW

PMID: 19197833 [PubMed - indexed for MEDLINE]

Permalink for 'The European Network for the Study of Adrenal Tumors staging system is prognostically superior to the international union against cancer-staging system: A North American validation.'

The European Network for the Study of Adrenal Tumors staging system is prognostically superior to the international union against cancer-staging system: A North American validation.

Fetched: January 3rd, 2010, 8:23pm CST

The European Network for the Study of Adrenal Tumors staging system is prognostically superior to the international union against cancer-staging system: A North American validation.

Eur J Cancer. 2009 Dec 29;

Authors: Lughezzani G, Sun M, Perrotte P, Jeldres C, Alasker A, Isbarn H, Budäus L, Shariat SF, Guazzoni G, Montorsi F, Karakiewicz PI

BACKGROUND: A reclassification of the International Union Against Cancer (UICC) staging system for adrenocortical carcinoma (ACC) patients has recently been proposed by the European Network for the Study of Adrenal Tumors (ENSAT) to better discriminate between cancer-specific mortality (CSM) risk strata. We formally tested the validity of the modified staging system in a large North American population-based cohort. METHODS: Kaplan-Meier survival curves depicted CSM rates in the overall population and after stratification according to the 2004 UICC or the 2008 ENSAT-staging system. Cox regression models addressing CSM tested the prognostic value of respectively the UICC or the ENSAT-staging system. Harrell's concordance index quantified the accuracy of the standard versus the modified staging system. RESULTS: In the overall population (n=573), the CSM-free survival rates at 1, 3, and 5 years were, respectively, 62.9%, 47.0%, and 38.1%. No statistically significant differences in survival were recorded between 2004 UICC stages II and III patients (p=0.1). Conversely, a statistically significant difference was observed between 2008 ENSAT stage II and stage III patients (p<0.001). The 2008 ENSAT-staging system showed higher accuracy (83.0%) in predicting 3-year CSM rates, relative to the 2004 UICC-staging system (79.5%) (p<0.001). CONCLUSION: Our study corroborates the superior accuracy of the ENSAT-staging system for ACC relative to the 2004 UICC-staging system. In consequence, the 2008 ENSAT-staging system may warrant consideration in the next update of staging manuals.

PMID: 20044246 [PubMed - as supplied by publisher]

Synthesis and Characterization of Iodinated Tetrahydroquinolines Targeting the G Protein-Coupled Estrogen Receptor GPR30.

Fetched: January 1st, 2010, 4:56pm CST

Synthesis and Characterization of Iodinated Tetrahydroquinolines Targeting the G Protein-Coupled Estrogen Receptor GPR30.

J Med Chem. 2009 Dec 30;

Authors: Ramesh C, Nayak TK, Burai R, Dennis MK, Hathaway HJ, Sklar LA, Prossnitz ER, Arterburn JB

A series of iodo-substituted tetrahydro-3H-cyclopenta[c]quinolines was synthesized as potential targeted imaging agents for the G protein-coupled estrogen receptor GPR30. The affinity and specificity of binding to GPR30 versus the classical estrogen receptors ERalpha/beta and functional responses associated with ligand-binding were determined. Selected iodo-substituted tetrahydro-3H-cyclopenta[c]quinolines exhibited IC(50) values lower than 20 nM in competitive binding studies with GPR30-expressing human endometrial cancer cells. These compounds functioned as antagonists of GPR30 and blocked estrogen-induced PI3K activation and calcium mobilization. The tributylstannyl precursors of selected compounds were radiolabeled with (125)I using the iodogen method. In vivo biodistribution studies in female ovariectomized athymic (NCr) nu/nu mice bearing GPR30-expressing human endometrial tumors revealed GPR30-mediated uptake of the radiotracer ligands in tumor, adrenal, and reproductive organs. Biodistribution and quantitative SPECT/CT studies revealed structurally related differences in the pharmacokinetic profiles, target tissue uptake, and metabolism of the radiolabeled compounds as well as differences in susceptibility to deiodination. The high lipophilicity of the compounds adversely affects the in vivo biodistribution and clearance of these radioligands and suggests that further optimization of this parameter may lead to improved targeting characteristics.

PMID: 20041667 [PubMed - as supplied by publisher]

[Radiofrequency ablation for postoperative recurrent of metastatic lesions of esophageal cancer]

Fetched: January 1st, 2010, 4:56pm CST

[Radiofrequency ablation for postoperative recurrent of metastatic lesions of esophageal cancer]

Gan To Kagaku Ryoho. 2009 Nov;36(12):2459-61

Authors: Todo M, Fujiwara H, Suchi K, Okamura S, Okamura H, Umehara S, Kubota T, Okamoto K, Kikuchi S, Ichikawa D, Sakakura C, Kokuba Y, Sonoyama T, Otsuji E

Radiofrequency ablation (RFA) was performed for the postoperative recurrent of metastatic lesions of esophageal cancer in 6 patients. All patients were males, and the median age was 59. Surgical curativities were A (3 cases), B (2) and C (1). The recurrent sites were intramediastinal omentum of gastric tube (2 cases), rt lung (2), rt adrenal grand (1) and liver (1). Four cases had a single recurrent lesion and the two had multiple lesions consisted of a single lesion as RFA target, and the lesions in a different site that were simultaneously treated by other therapeutic modalities. The median time of recurrence was 12 months after esophagectomy. RFA was performed once in the 3 cases, and twice in the other 3 cases. Therapeutic effect evaluated by CT was CR (2 cases), PR (3) and SD (1). No serious complications associated with RFA procedure were observed. Three patients died due to cancer recurrence within 7 months after RFA. However, RFA-treated lesions were well controlled to the end. RFA are safe and minimally invasive, thus, can be repeatedly performed technique that can induce a good local control of the target lesion equivalent to surgical resection. RFA is applicable as an effective local therapy for the recurrent or metastatic lesions of esophageal cancer.

PMID: 20037455 [PubMed - in process]

[A case report of combined hepatocellular-cholangiocarcinoma whose lymph node recurrence effectively treated with UFT]

Fetched: January 1st, 2010, 4:56pm CST

[A case report of combined hepatocellular-cholangiocarcinoma whose lymph node recurrence effectively treated with UFT]

Gan To Kagaku Ryoho. 2009 Nov;36(12):2380-2

Authors: Shimizu J, Hayashi S, Dono K, Yasumoto T, Zenitani M, Munakata K, Watanabe N, Takamoto K, Kagawa Y, Hata T, Kawanishi K, Ikeda K, Fujita J, Akagi K, Kitada M, Shimano T

Primary liver cancer with lymph node metastasis was recognized as poor prognosis. We herein report a case of post operative lymph node recurrence treated with UFT. A 74-year-old man with a huge mass lesion in the right liver with para Aortic lymph node metastasis admitted our hospital in April 2007. Extended right lobe hepatectomy and lymph node dissection were performed in May. A histological examination of the resected specimen showed a combined hepatocellular-cholangiocarcinoma with three lymph node metastasis. Computed tomography(CT)revealed intra hepatic metastasis (S3) and right adrenal grand metastasis 5 months after surgery. Transarterial embolization (TAE) and right adrenalectomy were performed for each metastasis. CT revealed a lymph node metastasis at the right side of infra vena cava 1 year after surgery. He was treated by oral administration of UFT (200 mg/day). The AFP and PIVKA-II value gradually decreased after administration of UFT. The size of lymph node metastasis became small confirmed by CT. But the AFP and PIVKA-II value increased 1 year and 7 months after surgery. TAE was performed against lymph node metastasis 1 year and 9 months after surgery. This case suggests UFT is useful for suppressing the growth of the lymph node metastasis.

PMID: 20037429 [PubMed - in process]

Biopsy of pheochromocytomas and paragangliomas: potential for disaster.

Fetched: January 1st, 2010, 4:56pm CST

Biopsy of pheochromocytomas and paragangliomas: potential for disaster.

Surgery. 2009 Dec;146(6):1158-66

Authors: Vanderveen KA, Thompson SM, Callstrom MR, Young WF, Grant CS, Farley DR, Richards ML, Thompson GB

BACKGROUND: Pheochromocytomas and paragangliomas are highly vascular neuroendocrine neoplasms that often secrete catecholamines. Percutaneous biopsy has been associated with life-threatening hemorrhage, hypertensive crisis, capsular disruption with tumor implantation, and death. We sought to determine the outcomes of biopsy in 20 consecutive patients. METHODS: We reviewed retrospectively the medical records of patients with biopsied pheochromocytomas and paragangliomas referred to our Endocrine Division for subsequent management between 1995 and 2005. Biopsy complications, operative findings, and outcomes were reviewed. RESULTS: Twenty patients (14 pheochromocytomas and 6 paragangliomas) were biopsied percutaneously prior to referral. Mean tumor diameter was 6.4 cm (range, 1-15). Pre-biopsy biochemical testing was not performed in 90% of patients, and was negative in the remainder. Fourteen patients (70%) developed a biopsy-related complication, including: increased difficulty of the definitive operation in 7 of 17 (41%) operative cases with 1 patient requiring conversion to an open procedure; severe hypertension (15%); hematoma (30%); incorrect or inadequate biopsy (25%); severe pain (25%); and delay in surgical treatment (15%). Mean follow-up was 58 months, with 4 tumor-related deaths and 2 recurrences. CONCLUSION: Biochemical testing prior to biopsy of adrenal or suspicious retroperitoneal masses is critical to exclude a catecholamine-secreting neoplasm because serious complications and increased operative difficulty can result.

PMID: 19958944 [PubMed - indexed for MEDLINE]

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PubMed - Adrenal Cancer (100 unread)

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