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Hemangioendothelioma (33 unread)

PubMed - Hemangioendothelioma

Primary epithelioid haemangioendothelioma of the eyelid.

Fetched: February 15th, 2010, 7:37am CST

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Primary epithelioid haemangioendothelioma of the eyelid.

Br J Ophthalmol. 2010 Feb;94(2):261-2

Authors: Tsuji H, Kanda H, Kashiwagi H, Mimura T

PMID: 20139294 [PubMed - in process]
Infantile hepatic hemangioendothelioma: an 8-month old infant successfully treated with a corticosteroid.

Fetched: February 15th, 2010, 7:37am CST

Infantile hepatic hemangioendothelioma: an 8-month old infant successfully treated with a corticosteroid.

Hematol Oncol Stem Cell Ther. 2009;2(3):422-5

Authors: Al-Tonbary Y, Fouda A

Infantile hemangioendothelioma is a rare benign vascular tumor of the liver. We report a case of hepatic hemangioendothelioma in an 8- month-old female infant who presented with hepatomegaly and respiratory distress, which was successfully treated with oral prednisolone for six months.

PMID: 20139057 [PubMed - in process]
[Epitheliod hemangioendothelioma of the lung]

Fetched: February 15th, 2010, 7:37am CST

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[Epitheliod hemangioendothelioma of the lung]

Arkh Patol. 2009 Nov-Dec;71(6):39-41

Authors:

The authors give a morphological and immunohistochemical description of epitheliod hemangioendothelioma of the lung in two women aged 62 and 74 years. Evident neovascularization capacity, a significant tumor cell intracytoplasmic lumen frequently packed with red blood cells, and positive immunohistochemical reactions to endothelial antigens are of prime importance in making its diagnosis.

PMID: 20131506 [PubMed - in process]
Hepatic epithelioid hemangioendothelioma: review of three cases.

Fetched: February 15th, 2010, 7:37am CST

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Hepatic epithelioid hemangioendothelioma: review of three cases.

Clin Imaging. 2010 Jan-Feb;34(1):79

Authors: Askri A, Mannai S, Landolsi S, Ben Farhart L, Said W, Dali N, Khalfallah MT, Hendaoni L

PMID: 20122537 [PubMed - in process]
Eccrine angiomatous hamartoma: a rare multifocal variant with features suggesting trauma.

Fetched: February 15th, 2010, 7:37am CST

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Eccrine angiomatous hamartoma: a rare multifocal variant with features suggesting trauma.

Dermatol Online J. 2009;15(9):6

Authors: Naik V, Arsenovic N, Reed M

Eccrine angiomatous hamartoma (EAH) is a rare, benign cutaneous tumor characterized by proliferation of the eccrine gland elements closely associated with capillary angiomatosis and proliferation of other dermal elements, such as adipose tissue, hair and epidermis. Patients usually present with a solitary nodule on the extremities appearing at birth or during the prepubertal years. However multifocal lesions or late onset of this condition may occur. Eccrine angiomatous hamartoma is usually sporadic, but one familial case of the multifocal variant has been reported. The clinical presentation ranges from a simple angiomatous nodule to erythematous--purpuric plaques. Eccrine angiomatous hamartoma is generally asymptomatic but may occasionally be associated with pain and hyperhidrosis. We report a rare case of the multifocal variant of EAH in a 13-year-old girl, with histological features suggesting trauma. Clinically, this condition must be differentiated from other angiomatoses and a definitive diagnosis is based upon histology. Eccrine angiomatous hamartoma is a benign slowly growing lesion for which aggressive treatment is not indicated. Simple excision is reserved for painful or cosmetically disfiguring examples.

PMID: 19930993 [PubMed - indexed for MEDLINE]
Epithelioid hemangioendothelioma of the liver with metastatic coeliac lymph nodes in an 11-year-old boy.

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

Epithelioid hemangioendothelioma of the liver with metastatic coeliac lymph nodes in an 11-year-old boy.

Pediatr Radiol. 2010 Jan 29;

Authors: Da Ines D, Petitcolin V, Joubert-Zakeyh J, Demeocq F, Garcier JM

Epithelioid hemangioendothelioma of the liver is a very rare vascular tumour in children with intermediate malignant potential. We present a case in which the typical imaging appearances of coalescent peripheral hepatic masses with capsular retraction contributed to the diagnosis. A positron emission tomography-CT (PET-CT) procedure was performed in staging the disease with a strong suspicion of coeliac nodal involvement confirmed after laparotomy and histological analysis. Our case is unique because of the rarity of the disease, the young age of the child, and proven nodal metastases at initial diagnosis. The use of PET-CT allows better staging at initial diagnosis and thus better management with improved follow-up in these patients.

PMID: 20112013 [PubMed - as supplied by publisher]
Vascular endothelial growth factor expression in hepatic epithelioid hemangioendothelioma: Implications for treatment and surgical management.

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

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Vascular endothelial growth factor expression in hepatic epithelioid hemangioendothelioma: Implications for treatment and surgical management.

Liver Transpl. 2010 Jan 26;16(2):191-197

Authors: Emamaullee JA, Edgar R, Toso C, Thiesen A, Bain V, Bigam D, Kneteman N, Shapiro AM

Epithelioid hemangioendothelioma (EHE) is a low-grade, malignant vascular tumor that most commonly presents within the liver. Patients with hepatic EHE are often candidates for liver transplantation as the disease is usually multifocal at diagnosis. Although these patients achieve excellent early outcomes post-transplant, there are very few data regarding tumor markers that can further direct chemotherapy in hepatic EHE to prevent recurrent disease. The purpose of this study was to analyze the expression of the angiogenic factor vascular endothelial growth factor (VEGF) and its receptors in hepatic EHE. Six patients with hepatic EHE were assessed for liver transplantation at our center. Pathology specimens of primary and recurrent EHE were analyzed by hematoxylin and eosin staining and by immunofluorescence for VEGF, fetal liver kinase 1 (Flk-1), and fms-related tyrosine kinase 1 (Flt-1) expression. Five patients underwent liver transplantation, and 1 patient underwent liver resection. Biopsy-proven recurrent EHE occurred in 3 patients. VEGF expression was present in 100% of the EHE specimens examined, whereas Flt-1 expression was present in only 1 sample, and Flk-1 was not observed in any of the specimens. In 1 patient with recurrent hepatic EHE post-liver transplantation, a progressive increase in the VEGF fluorescence intensity and distribution was observed. In conclusion, in this series, VEGF expression was observed in all hepatic EHE specimens analyzed. These data suggest that anti-VEGF chemotherapeutic agents will be of use in patients with hepatic EHE, particularly as a means of reducing the tumor volume prior to resection, as a means of treating unresectable or metastatic disease, or as an adjuvant therapy in the setting of liver transplantation. Liver Transpl 16:191-197, 2010. (c) 2010 AASLD.

PMID: 20104492 [PubMed - as supplied by publisher]
Aberrant VEGF Expression Associated With Neoplasm-Induced Extramedullary Hematopoiesis in an Epithelioid Hemangioendothelioma: A Case Report.

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

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Aberrant VEGF Expression Associated With Neoplasm-Induced Extramedullary Hematopoiesis in an Epithelioid Hemangioendothelioma: A Case Report.

Int J Surg Pathol. 2010 Jan 13;

Authors: Pinczewski J, Papadimitriou JC

The authors present the first report of extramedullary hematopoiesis occurring in association with an epithelioid hemangioendothelioma. A 64-year-old man sought medical attention for upper chest pain. CT evaluation identified a 2.8-cm mass involving the right hemithorax in the area of the right upper lobe and superior vena cava. A biopsy revealed the presence of an epithelioid malignant vascular neoplasm, which on subsequent resection was found to be an epithelioid hemangioendothelioma. The neoplasm was closely associated with an area of extramedullary hematopoiesis. VEGF immunohistochemical staining of the neoplasm revealed a ring of intensely positive staining cells around the periphery of the area of extramedullary hematopoiesis. This finding provides evidence for the role of aberrant VEGF expression in neoplasm-induced extramedullary hematopoiesis. This report discusses possible mechanisms by which extramedullary hematopoiesis may occur in vascular neoplasms.

PMID: 20075024 [PubMed - as supplied by publisher]
Composite haemangioendothelioma with lymph-node metastasis: an unusual presentation at an uncommon site.

Fetched: January 10th, 2010, 2:30pm CST

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Composite haemangioendothelioma with lymph-node metastasis: an unusual presentation at an uncommon site.

Clin Exp Dermatol. 2009 Dec;34(8):e802-6

Authors: Aydingöz IE, Demirkesen C, Serdar ZA, Mansur AT, Yaşar S, Aslan C

A 48-year-old woman presented with red papules on the thigh. Histopathological examination indicated pyogenic granuloma, and the patient was treated with total excision in 2003 and electrocauterization in 2005. Three months later, upon recurrence of the lesions, a diagnosis of composite haemangioendothelioma (CHE) was made. The patient was treated by total excision and lymph-node dissection, which revealed inguinal lymph-node metastasis. Despite the surgery, a further local recurrence occurred, subsequently treated by wide excision en bloc, with adjuvant radiotherapy and chemotherapy. Although CHE is defined as a vascular tumour with low-grade malignancy, the local recurrences and lymph-node metastases resulted in treatment difficulties in this case. Unlike earlier cases, the tumour in our patient presented as localized numerous small papulonodules, and lymph-node metastasis was detected within a relatively short time. Dermatologists and pathologists should be aware of this rare condition and include it in the differential diagnosis of vascular lesions.

PMID: 20055844 [PubMed - in process]
Pediatric liver transplantation for primary malignant liver tumors with a focus on hepatic epithelioid hemangioendothelioma: The UNOS experience.

Fetched: January 10th, 2010, 2:30pm CST

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Pediatric liver transplantation for primary malignant liver tumors with a focus on hepatic epithelioid hemangioendothelioma: The UNOS experience.

Pediatr Transplant. 2009 Dec 30;

Authors: Guiteau JJ, Cotton RT, Karpen SJ, O'Mahony CA, Goss JA

Guiteau JJ, Cotton RT, Karpen SJ, O'Mahony CA, Goss JA. Pediatric liver transplantation for primary malignant liver tumors with a focus on hepatic epithelioid hemangioendothelioma: The UNOS experience. Pediatr Transplantation 2009: 00: 000-000. (c) 2009 John Wiley & Sons A/S. Abstract: Treatment for HEH does not follow a standardized algorithm. From clinical experience, it is assumed that pediatric patients with HEH will fare as well as other common pediatric liver tumors post-OLT. The UNOS dataset was examined for patients with pediatric OLT between 1987 and 2007. Patients were grouped into non-tumors, HB, HCC, HEH, and rare liver tumors. COD analysis was calculated using Fisher's exact test. Patient, allograft, and recurrence-free survival were compared using Kaplan-Meier curves and log-rank tests. A total of 366 patients with pediatric OLT were identified with primary liver tumors (HB - 237, HCC - 58, HEH - 35, other - 36). HEH patient survival (five yr: 60.6%) was poorer than non-tumor OLTpatient survival (five yr: 84.4%). Survival was worse when compared to HB (five yr: 72%) and rare liver tumors (five yr: 78.9%), but better than HCC (five yr: 53.5%). Allograft survival in HEH (five yr: 50.1%) lies between HB (five yr: 63.6%) and HCC (five yr: 42.8%). COD analysis demonstrates recurrence as a major cause in HB and HCC, but not for HEH or other liver tumors. The data suggest that patient survival may not be as high as previously believed and further investigation is warranted.

PMID: 20051026 [PubMed - as supplied by publisher]
Getting a handle on managing rare tumors.

Fetched: January 10th, 2010, 2:30pm CST

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Getting a handle on managing rare tumors.

Arch Surg. 2009 Nov;144(11):1039

Authors: Hebert JC

PMID: 19928296 [PubMed - indexed for MEDLINE]
Intraoral epithelioid hemangioendothelioma: a case report and review of the literature.

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

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Intraoral epithelioid hemangioendothelioma: a case report and review of the literature.

Med Oral Patol Oral Cir Bucal. 2009 Dec 29;

Authors: Gordón-Núñez MA, Silva LM, Lopes MF, Neto SF, Maia AP, Galvao HC

The epithelioid hemangioendothelioma (EH) is an uncommon angiocentric neoplasm of borderline or intermediate malignant potential, between the hemangioma and conventional angiosarcoma. It is characterized by the proliferation of endothelial cells with epithelioid or histiocitóide morphology with vacuolated cytoplasm and occasional eosinophilic spindle cells. Shows potential for local recurrence as well as the ability to metastasize. Rarely affects the oral cavity, it have been described from 1975 until 2008 only 27 oral cases. Morphologically the EHs may be confused with other lesions, from a hemangioma to a squamous cell carcinoma, and thus immunohistochemical analysis is required. This paper reports the clinical and immunohistochemical characteristics of a case of EH in the gingiva of the tooth 35 of a 17 years-old-white-female. We present a review of the clinicopathological and immunohistochemical characteristics of the intraoral epithelioid hemangioendothelioma cases previously reported.

PMID: 20038915 [PubMed - as supplied by publisher]
[A case of primary hepatic epithelioid hemangioendothelioma with spontaneous rupture.]

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

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[A case of primary hepatic epithelioid hemangioendothelioma with spontaneous rupture.]

Korean J Hepatol. 2009 Dec;15(4):510-6

Authors: Kim GH, Kim YS, Kim HO, Kim KH, Hung YK, Jung DH, Kim JH, Kwon OS, Choi DJ, Kim JH

Primary hepatic epithelioid hemangioendothelioma is a rare neoplasm of endothelial origin. The clinical manifestations are nonspecific, ranging from complete absence of symptoms to hepatic failure and death. Spontaneous rupture of a hepatic epithelioid hemangioendothelioma is an extremely rare presentation. We present a case of primary hepatic epithelioid hemangioendothelioma in a 65-year-old male patient with alcoholic liver cirrhosis. He was hospitalized due to epigastric pain and multiple liver masses on abdominal ultrasound. Dynamic liver CT imaging revealed multiple peripheral nodular enhanced mass lesions with delayed centripetal enhancement, and the adjacent collection of high-attenuation fluid along the liver capsule. Abdominal tapping revealed blood in the peritoneal cavity. Primary hepatic epithelioid hemangioendothelioma with spontaneous rupture was finally diagnosed based on a histopathologic examination revealing positive immunohistochemical staining for CD34.

PMID: 20037270 [PubMed - in process]
High-intensity interval training may reduce in-stent restenosis following percutaneous coronary intervention with stent implantation A randomized controlled trial evaluating the relationship to endothelial function and inflammation.

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

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High-intensity interval training may reduce in-stent restenosis following percutaneous coronary intervention with stent implantation A randomized controlled trial evaluating the relationship to endothelial function and inflammation.

Am Heart J. 2009 Nov;158(5):734-41

Authors: Munk PS, Staal EM, Butt N, Isaksen K, Larsen AI

BACKGROUND: High-intensity interval training has been shown to be superior to moderate continuous exercise training in improving exercise capacity and endothelial function in patients with coronary artery disease. The objective of this study was to evaluate this training model on in-stent restenosis following percutaneous coronary intervention for stable or unstable angina. METHODS AND RESULTS: We prospectively randomized 40 patients after percutaneous coronary intervention with implantation of a bare metal stent (n = 30) or drug eluting stent (n = 32) to a 6-month supervised high-intensity interval exercise training program (n = 20) or to a control group (n = 20). At six months, restenosis, measured as in-segment late luminal loss of the stented coronary area, was smaller in the training group 0.10 (0.52) mm compared to the control group 0.39 (0.38) mm (P = .01). Reduction of late luminal loss in the training group was consistent with both stent types. Peak oxygen uptake increased in the training and control group by 16.8% and 7.8%, respectively (P < .01). Flow-mediated dilation improved 5.2% (7.6) in the training group and decreased -0.1% (8.1) in the control group (P = .01). Levels of high-sensitivity C-reactive protein decreased by -0.4 (1.1) mg/L in the training group and increased by 0.1 (1.2) mg/L in the control group (P = .03 for trend). CONCLUSIONS: Regular high-intensity interval exercise training was associated with a significant reduction in late luminal loss in the stented coronary segment. This effect was associated with increased aerobic capacity, improved endothelium function, and attenuated inflammation.

PMID: 19853690 [PubMed - indexed for MEDLINE]
[Infantile vascular tumors.]

Fetched: December 22nd, 2009, 8:01am CST

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[Infantile vascular tumors.]

An Pediatr (Barc). 2009 Dec 18;

Authors: López Almaraz R, López Gutiérrez JC, Belendez Bieler C, Herrero Hernández A, Mateos González ME, Ramírez Villar G

The use in the past of an imprecise terminology to designate vascular tumors has contributed to its incorrect diagnosis, and as a consequence, to inadequate treatment. In childhood, different types of vascular tumors may be present. Hemangiomas of infancy are by far the most frequent, and other less common types are congenital hemangiomas (rapidly involuting or RICH and non-involuting or NICH), kaposiform hemangioendothelioma, angioblastoma or tufted angioma and pyogenic granuloma. The correct knowledge and diagnosis, always in a multidisciplinary setting, is required to reduce incorrect diagnosis, unnecessary complementary examinations and invasive tests, and for the patient to receive the most effective and precise treatment in each case. This article reviews the historical evolution, nomenclature and classification of vascular lesions, the different clinical and pathological characteristics of each vascular tumor, the complementary examinations required correct diagnosis, the differential diagnosis, as well as highlighting the treatment options currently available for different vascular tumors and related clinical conditions.

PMID: 20022827 [PubMed - as supplied by publisher]
Unique vascular tumor primary arising in the liver and exhibiting histopathological features consistent with so-called polymorphous hemangioendothelioma.

Fetched: December 22nd, 2009, 8:01am CST

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Unique vascular tumor primary arising in the liver and exhibiting histopathological features consistent with so-called polymorphous hemangioendothelioma.

Pathol Int. 2009 Dec 1;59(12):890-894

Authors: Cobianchi L, Lucioni M, Rosso R, Zonta S, Fiandrino G, Alessiani M, Doni M, Ferrari A, Magrini U, Paulli M, Dionigi P

Reported herein is an unusual vascular tumor primary arising in the liver and exhibiting unique histopathological features. A 47-year-old woman underwent left hepatectomy because of a large hepatic mass. On histology the tumor had a composite pattern, consisting of angiomatous, retiform and solid areas, formed by oval to cuboidal to spindle cells, that expressed only endothelial markers (CD31 and factor VIII-related antigen). These findings led to the diagnosis of a low-grade vascular neoplasm with morphological features consistent with so-called polymorphous hemangioendothelioma. The tumor was completely resected. At 24 month follow up the patient was alive, without evidence of disease. Polymorphous hemangioendothelioma is a rare vascular neoplasm, with borderline malignant potential, which usually occurs in lymph nodes and, rarely, at extranodal sites. Its classification as an entity has been questioned recently. The unusual morphological features of the present case, which do not fit neatly with any other recognized hemangioendothelioma subtype, indicate that the family of vascular tumors is broader than currently accepted. In addition the present case widens the spectrum of primary vascular tumors arising in the liver.

PMID: 20021616 [PubMed - as supplied by publisher]
Antiangiogenic properties of a nutrient mixture in a model of hemangioma.

Fetched: December 22nd, 2009, 8:01am CST

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Antiangiogenic properties of a nutrient mixture in a model of hemangioma.

Exp Oncol. 2009 Dec;31(4):FirstPage-LastPage

Authors: Roomi MW, Kalinovsky T, Niedzwiecki A, Rath M

The pathogenesis of hemangiomas is still largely unknown and the current therapy, such as systemic corticosteroid, vincristine, and interferon-alpha, is toxic and remains unsatisfactory. A nutrient mixture (NM) containing lysine, proline, ascorbic acid and green tea extract has shown significant anti-angiogenic and anti-tumor effect against a number of cancer cell lines. Aim: Using a mouse hemangioendothelioma model, we investigated the efficacy of NM. We also tested the effect of NM in vitro, evaluating cell viability, MMP secretion, invasion, morphology and apoptosis. Methods: Athymic nude mice, 5-6 weeks old, were inoculated with 3 x106 EOMA cells subcutaneously and randomly divided into two groups; group A was fed a regular diet and group B - a regular diet supplemented with 0.5% NM. Four weeks later, the mice were sacrificed and their tumors were excised, weighed and processed for histology. We also tested the effect of NM in vitro. Results: NM inhibited the growth of tumors by 50%. In vitro, NM exhibited dose response cytotoxicity with 10%, 30% and 55% at 10, 100 and 1000 mug/ml. Invasion through Matrigel was inhibited at 50, 100 and 500 mug/ml by 25%, 30% and 100% respectively. NM induced dose-dependent apoptosis of EOMA cells. Conclusions: These results suggest that NM may have therapeutic potential in treating infantile hemangioendotheliomas and, perhaps, other cutaneous vascular tumors.

PMID: 20010532 [PubMed - in process]
[Retiform haemangioendothelioma: a case report]

Fetched: December 22nd, 2009, 8:01am CST

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[Retiform haemangioendothelioma: a case report]

Ann Pathol. 2009 Dec;29(6):491-4

Authors: de Wind A, Meert V, Chahidi N, Theunis A, Somerhausen Nde S

Retiform haemangioendothelioma is a locally aggressive, very rarely metastasizing vascular lesion. Histologically, it is characterized by distinctive arborizing blood vessels resembling "rete testis" and lined by endothelial cells with characteristic hobnail morphology. We present an additional case, in the leg of a 64-year-old patient. We discuss the classification of hemangioendotheliomas. The term hemangioendothelioma should be restricted to vascular tumours of "intermediate malignancy" but has been used to designate tumours with variable histological features and clinical behaviour. Spindle cell hemangio(endothelio)ma is currently regarded as a benign reactive lesion. Kaposiform hemangioendothelioma is potentially lethal due to consumption coagulopathy but no metastasizing case has been reported. Epithelioid hemangioendothelioma is associated with a significant metastatic risk and has been included in the category of malignant vascular tumors. The vascular lesions fulfilling the strict definition of hemangioendothelioma include retiform hemangioendothelioma, papillary intralymphatic angioendothelioma "Dabska's tumor", composite hemangioendothelioma and perhaps the controversial polymorphic hemangioendothelioma.

PMID: 20005438 [PubMed - in process]
A single center experience of combined liver kidney transplantation.

Fetched: December 22nd, 2009, 8:01am CST

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A single center experience of combined liver kidney transplantation.

Clin Transplant. 2009 Dec;23 Suppl 21:102-14

Authors: Mehrabi A, Fonouni H, Ayoub E, Rahbari NN, Müller SA, Morath Ch, Seckinger J, Sadeghi M, Golriz M, Esmaeilzadeh M, Hillebrand N, Weitz J, Zeier M, Büchler MW, Schmidt J, Schmied BM

With advancements in the operative techniques, patient survival following liver transplantation (LTx) has increased substantially. This has led to the acceleration of pre-existing kidney disease because of immunosuppressive nephrotoxicity making additional kidney transplantation (KTx) inevitable. On the other hand, in a growing number of patients on the waiting list to receive liver, long waiting time has resulted in adverse effect of decompensated liver on the kidney function. During the last two decades, the transplant community has considered combined liver kidney transplantation (CLKTx) to overcome this problem. The aim of our study is to present an overview of our experience as well as a review of the literature in CLKTx and to discuss the controversy in this regard. All performed CLKTx (n = 22) at our institution as well as all available reported case series focusing on CLKTx are extracted. The references of the manuscripts were cross-checked to implement further articles into the review. The analyzed parameters include demographic data, indication for LTx and KTx, duration on the waiting list, Model for End-Stage Liver Disease (MELD) score, Child-Turcotte-Pugh (CTP) score, immunosuppressive regimen, post-transplant complications, graft and patient survival, and cause of death. From 1988 to 2009, a total of 22 CLKTx were performed at our institution. The median age of the patients at the time of CLKTx was 44.8 (range: 4.5-58.3 yr). The indications for LTx were liver cirrhosis, hyperoxaluria type 1, polycystic liver disease, primary or secondary sclerosing cholangitis, malignant hepatic epithelioid hemangioendothelioma, cystinosis, and congenital biliary fibrosis. The KTx indications were end-stage renal disease of various causes, hyperoxaluria type 1, polycystic kidney disease, and cystinosis. The mean follow-up duration for CLKTx patients were 4.6 +/- 3.5 yr (range: 0.5-12 yr). Overall, the most important encountered complications were sepsis (n = 8), liver failure leading to retransplantation (n = 4), liver rejection (n = 3), and kidney rejection (n = 1). The overall patient survival rate was 80%. Review of the literature showed that from 1984 to 2008, 3536 CLKTx cases were reported. The main indications for CLKTx were oxalosis of both organs, liver cirrhosis and chronic renal failure, polycystic liver and kidney disease, and liver cirrhosis along with hepatorenal syndrome (HRS). The most common encountered complications following CLKTx were infection, bleeding, biliary complications, retransplantation of the liver, acute hepatic artery thrombosis, and retransplantation of the kidney. From the available data regarding the need for post-operative dialysis (n = 673), a total of 175 recipients (26%) required hemodialysis. During the follow-up period, 154 episodes of liver rejection (4.3%) and 113 episodes of kidney rejection (3.2%) occurred. The cumulative 1, 2, 3, and 5 yr survival of both organs were 78.2%, 74.4%, 62.4%, and 60.9%, respectively. Additionally, the cumulative 1, 2, 3, and 5 yr patient survival were 84.9%, 52.8%, 45.4%, and 42.6%, respectively. The total number of reported deaths was 181 of 2808 cases (6.4%), from them the cause of death in 99 (55%) cases was sepsis. It can be concluded that there is still no definitive evidence of better graft and patient survival in CLKTx recipients when compared with LTx alone because of the complexity of the exact definition of irreversible kidney function in LTx candidates. Additionally, CLKTx is better to be performed earlier than isolated LTx and KTx leading to the avoidance of deterioration of clinical status, high rate of graft loss, and mortality. Shorter graft ischemia time and more effective immunosuppressive regimens can reduce the incidence of graft malfunctioning in CLKTx patients. Providing a model to reliably determine the need for CLKTx seems necessary. Such a model can be shaped based upon new and precise markers of renal function, and modification of MELD system.

PMID: 19930323 [PubMed - in process]
Epithelioid angiomatous nodule of head and neck.

Fetched: December 22nd, 2009, 8:01am CST

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Epithelioid angiomatous nodule of head and neck.

Pathol Res Pract. 2009;205(11):753-7

Authors: Sun ZJ, Cai Y, Zhao YF, Hu X, Zhang W, Chen XM, Lai FM

Epithelioid vascular proliferations comprise a wide morphologic spectrum, ranging from reactive hyperplasia via benign tumor to malignant conditions. Epithelioid angiomatous nodule (EAN) is a recently recognized benign vascular proliferation with distinct clinical and pathological features. We review the literature and present six additional EANs in the head and neck examined by immunohistochemistry. This series comprised an equal number of males (n=3) and females (n=3) aged 13-45 years (mean 25.8 years). The sites of origin included the tongue (n=2), skin of the lip (n=2), periorbital and temporal skin (n=1), and nose (n=1). A painless solitary nodule (n=4) was the most common presentation. The lesions were distributed in the epidermis and subcutis, with a characteristic unilobular solid proliferation of polygonal epithelioid endothelial cells with intracytoplasmic primitive lumina. The distinction of EAN from other lesions of the heterogeneous group with epithelioid vascular proliferation can usually be made on the basis of the clinical and morphological features. This series indicates that the distribution of EAN in the head and neck region is not uncommon, which is in contrast to what was initially thought. EAN may be a variant of epithelioid hemangioma.

PMID: 19596525 [PubMed - indexed for MEDLINE]
CD10 is expressed in most epithelioid hemangioendotheliomas: a potential diagnostic pitfall.

Fetched: December 9th, 2009, 7:36am CST

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CD10 is expressed in most epithelioid hemangioendotheliomas: a potential diagnostic pitfall.

Arch Pathol Lab Med. 2009 Dec;133(12):1965-8

Authors: Weinreb I, Cunningham KS, Perez-Ordoñez B, Hwang DM

CONTEXT: -Epithelioid hemangioendothelioma (EHE) is a vascular neoplasm that occasionally is difficult to distinguish from primary/metastatic carcinomas, particularly when EHEs express keratins. We recently encountered an EHE with strong CD10 positivity mimicking renal cell carcinoma. OBJECTIVE: -To examine sensitivity and specificity of CD10 in EHE. DESIGN: -Nine EHEs were stained with keratins, factor VIII, CD31, CD34, and CD10. Mimics of EHE were also retrieved and stained with CD10. RESULTS: -The EHE patients included 5 men and 4 women. Patients ranged in age from 24 to 74 years. Tumors were located in liver (3), skin (2), lung/pleura (2), and sternomastoid and mediastinum (1 each). Two had skin metastases. All EHEs were positive for vascular markers. A total of 7 of 9 primary tumors expressed cytoplasmic and intracytoplasmic luminal CD10. The 2 skin metastases were positive, whereas 2 primary skin EHEs were negative. Of the mimics, CD10 showed staining in 7 of 23 cases: 3 of 3 renal cell carcinomas, 1 of 7 other carcinomas, 2 of 3 epithelioid angiosarcomas, 1 of 3 melanomas, 0 of 3 mesotheliomas, and 0 of 4 epithelioid hemangiomas. CONCLUSIONS: -CD10 has a sensitivity of 78% (confidence interval, 63.6%-92.4%) and specificity of 70% (confidence interval, 54%-85.9%) for EHE. There is a growing list of tumors that show expression of CD10. Pathologists should be aware of this diagnostic pitfall.

PMID: 19961253 [PubMed - in process]
[Surgical treatment for bone nonunion after massive allograft transplantation]

Fetched: December 9th, 2009, 7:36am CST

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[Surgical treatment for bone nonunion after massive allograft transplantation]

Zhonghua Wai Ke Za Zhi. 2009 Jun 1;47(11):837-41

Authors: Hao L, Wang T, Xu HR, Niu XH

OBJECTIVE: To evaluate the surgical treatment and outcome of autogenous bone grafting and internal fixation in management of bone nonunion after massive allograft transplantation. METHODS: From January 1994 to December 2006, 41 of 176 patients underwent bone nonunion after massive allograft transplantation. Twenty-two of 41 patients received autogenous bone grafting. Complete clinical and follow-up data was available for 15 cases. The average age at secondary autogenous bone grafting was 24 years old (ranging from 15 to 34). The primary diseases included osteosarcoma (5 cases), giant cell tumor (4 cases), parosteal osteosarcoma (2 cases), hemangioendothelioma (2 cases) and primitive neuroectodermal tumor (2 cases). Tumor was located at distal femur in 7 patients, middle of humerus in 3, middle of femur in 2, proximal tibia in 2 and proximal humerus in 1. Eight of 15 patients with simple bone nonunion received autogenous bone grafting. Another 7 patients with bone nonunion and fracture of primary internal fixation underwent autogenous bone grafting and re-internal fixation. RESULTS: At a mean follow-up of 46.8 months (ranging from 18 to 148 months), bone union was observed in 13 of 15 patients (86.7%) with the mean healing time 13.3 months (ranging from 5 to 20). Bone union could be observed in all 8 patients with simple bone nonunion and 5 of 7 patients with bone nonunion and internal fixation fracture, similar healing time 14 and 12 months respectively. There was no infection or any other complications. Two patients underwent re-nonunion received prosthesis replacement at last. The mean MSTS score of 13 patients was 25.1, with 8 simple bone nonunion patients and 5 combined with internal fixation fracture patients 25.4 and 24.6 respectively, also basically no difference. CONCLUSIONS: Autogenous bone grafting and internal fixation in management of nonunion after massive allograft transplantation have the advantage of easy operation, less complications, high rate of bone healing and good function result with obvious superiority to prosthesis replacement. For management of nonunion after massive allograft transplantation, autogenous bone grafting and internal fixation is mostly recommended.

PMID: 19961014 [PubMed - in process]
[Atypical vascular tumors of the gastrointestinal tract: four uncommon cases]

Fetched: December 9th, 2009, 7:36am CST

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[Atypical vascular tumors of the gastrointestinal tract: four uncommon cases]

Cir Pediatr. 2009 Jul;22(3):125-7

Authors: Burgos L, Gutiérrez JC, Barrena S, De la Torre C, Suárez O, Luis AL

AIM: A small but significant percentage of vascular tumors may develop at extracutaneous location. They are difficult to detect on the physical exam and usually they require immediate intervention. Pediatric surgeons must have acknowledge of its prognostic and therapeutic implications. PATIENTS AND RESULTS: We report 4 of these patients. Patient 1 was a healthy newborn who presented in the second week of life, recurrent severe gastrointestinal bleeding, thrombocytopenia and anemia. Diagnosis of multifocal linfangioendoteliomatosis with thrombocytopenia was established. Patient 2 had prenatal diagnosis of ascites and presented at birth sepsis, anemia, thrombocytopenia and hypoproteinemia. Upon laparotomy hemorrhagic ascites and thickening of rectum-sigmoid wall and mesentery were found. Pathologic diagnosis was Kaposiform hemangioendothelioma and the clinical course was consistent with Kassabach-Merrit phenomenon. Patient 3 had at birth, multifocal hepatic GLUT1- hemangiomatosis with severe cardiac insufficiency and coagulopathy. She died while waiting for a liver transplantation. Patient 4 is a girl who presented in the newborn period with vomiting and hematochezia. She required several transfusions and endoscopic biopsies showed a vascular tumor that infiltrated duodenum, jejunum and mesentery. Imaging studies and histologic findings on biopsy led to the diagnostic of juvenile hemangioma GLUT-1+. CONCLUSIONS: Vascular tumors of the digestive tract may be difficult to diagnosis and their classification is still incomplete. Pediatric surgeons must be acquainted with these varieties of tumors because they are always involved in diagnosis and therapeutic decision making.

PMID: 19957858 [PubMed - in process]
[Clinical analysis of 17 cases of pneumatic compression therapy in infants with Kasabach-Merritt phenomenon]

Fetched: December 9th, 2009, 7:36am CST

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[Clinical analysis of 17 cases of pneumatic compression therapy in infants with Kasabach-Merritt phenomenon]

Zhonghua Yi Xue Za Zhi. 2009 Jul 14;89(26):1830-3

Authors: Liu XJ, Tai MZ, Tian MX, Qin ZP

OBJECTIVE: To summarize the efficacy and safety of employing pneumatic compression therapy in infants with Kasabach-Merritt phenomenon (KMP). METHODS: Seventeen patients with KMP (11 males, 6 females) were treated with pneumatic compression therapy from October 1997 to May 2008. And their clinical characteristics, course of treatment and clinical and laboratory data were retrospectively analyzed. Among 17 patients, 8 cases were located in trunk, 5 in lower extremities and 4 in upper extremities. The diameters of lesions exceeded 8 cm in all patients. The platelet count was all < 100 x 10(9)/L while hemoglobin < 110 g/L and fibrinogen < 2.0 g/L. The self-designed device for pneumatic compression henmangioma therapy was employed (Patent No: ZL97232266. 3). Biopsy and exairesis were performed from the local lesions with KMP in order to determine the pathological features. RESULTS: Two patients were cured after pneumatic compression therapy for 4 and 6 months respectively, and their lesions disappeared, blood parameters became normal and remained relapse-free after a 5/11-year follow-up. Eleven patients were effective after pneumatic compression therapy for 4 - 6 months, and improvement was demonstrated after 6 - 24 months follow-up without any treatment. Two patients showed improvement after pneumatic compression therapy for 6 months after a follow-up for 5 months or 2 years without progression. After a 6-month pneumatic compression therapy, 2 ineffective patients underwent surgical resection. There were 14 cases of kaposiform hemangioendothelioma (KHE) and 3 cases of tufted hemangioma (TA). CONCLUSION: Pneumatic compression therapy has definite curative effects for KMP lesions in extremities and trunk and its side effects are fewer.

PMID: 19953927 [PubMed - in process]
[Erratum to: "Hepatic Epithelioid hemangioendothelioma: A rare live tumor" [J Radiol 2009;90:845-8].]

Fetched: December 9th, 2009, 7:36am CST

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[Erratum to: "Hepatic Epithelioid hemangioendothelioma: A rare live tumor" [J Radiol 2009;90:845-8].]

J Radiol. 2009 Nov;90(11 Pt 1):1764

Authors: Demuynck F, Morvan J, Brochart C, Blanpain S, Brasseur A, Boukadoum N, Herlin C, Fuks D, Chatelain D, Yzet T, Deramond H

PMID: 19953069 [PubMed - in process]
DO INTRACRANIAL NEOPLASMS DIFFER IN OLLIER DISEASE AND MAFFUCCI SYNDROME? AN IN-DEPTH ANALYSIS OF THE LITERATURE.

Fetched: November 27th, 2009, 11:19am CST

DO INTRACRANIAL NEOPLASMS DIFFER IN OLLIER DISEASE AND MAFFUCCI SYNDROME? AN IN-DEPTH ANALYSIS OF THE LITERATURE.

Neurosurgery. 2009 Dec;65(6):1106-1115

Authors: Ranger A, Szymczak A

OBJECTIVE: Ollier disease (OD) and Maffucci syndrome are closely related, very rare syndromes, that are both associated with multiple sites of chondrodysplasia. They differ primarily with the additional association of Maffucci syndrome with vascular abnormalities, including hemangiomas. Both are associated with chondrosarcomas and other nonsarcomatous neoplasms (NSN), but Maffucci syndrome is thought to have greater malignancy potential. We examined whether OD and Maffucci syndrome differ in the number, histology, in the location of intracranial malignancies, and in the demographics of such patients, as reported in the medical literature. METHODS: Relevant cases were identified by electronic searches on PubMed, SciSearch, Scientific Commons, Springer Link, and Google. Translate DotNet and Babelfish were used to translate non-English text. Unpaired Student's t tests were used to compare OD and Maffucci syndrome, and chondrosarcoma and NSN patients for mean age; Pearson chi analysis was used for comparisons of gender distribution, geographical distribution (by continent), site of lesion, and for OD versus Maffucci syndrome, tumor type (chondrosarcoma versus NSN). RESULTS: Forty-six patients with 47 intracranial malignancies were identified: 24 with OD, including 6 with a chondrosarcoma and 18 with an NSN; and 22 with Maffucci syndrome, including 13 with a chondrosarcoma, 8 with an NSN, and 1 with both. The 2 syndromes were statistically different in the distribution of chondrosarcomas versus NSNs (P = 0.002). All chondrosarcomas originated at the base of the cranium. All 18 NSNs in OD were of glial cell origin, but only 5 of 9 NSNs in Maffucci syndrome were glial; other tumors included pituitary adenoma, olfactory neuroblastoma, malignant chordoma, and spindle cell hemangioendothelioma. Patients with OD were more than 10 years younger than their Maffucci syndrome counterparts (24.7 versus 34.9 years; P = 0.002), as were patients with OD and chondrosarcoma versus those with Maffucci syndrome and chondrosarcoma (24.7 versus 36.2 years; P = 0.035). The 2 syndromes did not differ in overall sex distribution. OD and Maffucci syndrome differed in geographical distribution, with 10 Maffucci syndrome but no OD patients with malignancy either in Asia (7 patients) or South America (3 patients). Among NSNs, OD and Maffucci syndrome did not differ as to site of lesion within the brain. CONCLUSION: OD and Maffucci syndrome differ with respect to the distribution of intracranial malignancies by histology, and geographical and age distribution of cases, with OD patients younger by approximately a decade, and Maffucci syndrome patients more likely to live in Asia or South America.

PMID: 19934970 [PubMed - as supplied by publisher]
Hemangiomas, angiosarcomas, and vascular malformations represent the signaling abnormalities of pathogenic angiogenesis.

Fetched: November 27th, 2009, 11:19am CST

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Hemangiomas, angiosarcomas, and vascular malformations represent the signaling abnormalities of pathogenic angiogenesis.

Curr Mol Med. 2009 Nov;9(8):929-34

Authors: Arbiser JL, Bonner MY, Berrios RL

Angiogenesis is a major factor in the development of benign, inflammatory, and malignant processes of the skin. Endothelial cells are the effector cells of angiogenesis, and understanding their response to growth factors and inhibitors is critical to understanding the pathogenesis and treatment of skin disease. Hemangiomas, benign tumors of endothelial cells, represent the most common tumor of childhood. In our previous studies, we have found that tumor vasculature in human solid tumors expresses similarities in signaling to that of hemangiomas, making the knowledge of signaling in hemangiomas widely applicable. These similarities include expression of reactive oxygen, NFkB and akt in tumor vasculature. Furthermore, we have studied malignant vascular tumors, including hemangioendothelioma and angiosarcoma and have shown distinct signaling abnormalities in these tumors. The incidence of these tumors is expected to rise due to environmental insults, such as radiation and lumpectomy for breast cancer, dietary and industrial carcinogens (hepatic angiosarcoma), and chronic ultraviolet exposure and potential Agent Orange exposure. I hypothesize that hemangiomas, angiosarcomas, and vascular malformations represent the extremes of signaling abnormalities seen in pathogenic angiogenesis.

PMID: 19925405 [PubMed - in process]
Pulmonary epithelioid hemangioendothelioma: a case report.

Fetched: November 27th, 2009, 11:19am CST

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Pulmonary epithelioid hemangioendothelioma: a case report.

Cases J. 2009;2:8235

Authors: Ouadnouni Y, Bouchikh M, Achir A, Zouaidia F, Smahi M, Msougar Y, Lakranbi M, Afqir S, Mahassini N, Benosman A

INTRODUCTION: The pulmonary epithelioid hemangioendothelioma is a rare vascular intermediate malignancy tumour. CASE PRESENTATION: A 45-year-old man, he shows an isolated chronic cough with a preserved general state of health. The thoracoabdominal Computed tomography showed three well limited opacities of the right lung, among them one shows some calcifications; which we entirely resected by enucleation after a pneumotomy. The histologic examination with immunomarking led to an epithelioid hemangioendothelioma. CONCLUSIONS: The pulmonary epithelioid hemangioendothelioma is a tumour of unpredictable prognosis, bad when linked to the plurifocal and symptomatic forms.

PMID: 19918408 [PubMed - in process]
Treatment of hepatic epithelioid hemangioendothelioma: a single-institution experience with 25 cases.

Fetched: November 27th, 2009, 11:19am CST

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Treatment of hepatic epithelioid hemangioendothelioma: a single-institution experience with 25 cases.

Arch Surg. 2009 Nov;144(11):1035-9

Authors: Cardinal J, de Vera ME, Marsh JW, Steel JL, Geller DA, Fontes P, Nalesnik M, Gamblin TC

OBJECTIVE: To examine treatment of hepatic epithelioid hemangioendothelioma (EHE), a rare vascular tumor with a variable course. Treatment modalities at our institution include liver resection, transplantation, and catheter-based therapies. DESIGN, PATIENTS, AND MAIN OUTCOME MEASURES: Retrospective review of 25 patients treated for hepatic EHE (1976-2007). We examined treatment modality, overall survival, complications, and clinicopathologic characteristics. RESULTS: Of the 25 patients treated for hepatic EHE, 17 underwent liver transplantation (LT); 4, transcatheter arterial chemoembolization (TACE); 2, resection; and 2, TACE followed by LT. Twelve patients (48%) were male. The median age at diagnosis was 38 years (range, 9 months to 72 years). Mean overall survival was 167 (95% confidence interval [CI], 123-212) months, with 172 (124-220) months in the LT group and 83 (54-112) months in the TACE group. The 2 patients in the resection group remain alive after 19 and 71 months. The 2 patients treated with TACE followed by LT died after 13 and 113 months. Extrahepatic disease was identified as a predictor of outcome. Patients with extrahepatic disease treated with TACE fared better than those treated with surgical approaches (mean survival, 83.0 [95% CI, 54.2-111.8] vs 38.8 [23.7-53.8] months; P = .12). CONCLUSIONS: Hepatic EHE is a rare tumor that can be treated with surgical or nonsurgical approaches. In our experience, LT is used for patients with advanced local disease, whereas TACE is the primary modality when extrahepatic disease or comorbid conditions prohibiting LT are present. To our knowledge, this is the largest single-institution experience describing the various therapeutic modalities in the treatment of hepatic EHE.

PMID: 19917940 [PubMed - in process]
Cutaneous metastasis of epithelioid haemangioendothelioma.

Fetched: November 27th, 2009, 11:19am CST

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Cutaneous metastasis of epithelioid haemangioendothelioma.

Pathology. 2009;41(6):585-7

Authors: Cooper CL, Danieletto S, Fong E, Harris MF, Konya J, Cooper WA, McCarthy SW, Scolyer RA

PMID: 19900110 [PubMed - in process]
Lenalidomide stops progression of multifocal epithelioid hemangioendothelioma including intracranial disease.

Fetched: November 27th, 2009, 11:19am CST

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Lenalidomide stops progression of multifocal epithelioid hemangioendothelioma including intracranial disease.

J Neurooncol. 2009 Nov 7;

Authors: Sumrall A, Fredericks R, Berthold A, Shumaker G

Epithelioid Hemangioendothelioma (EH) is a rare soft-tissue tumor which may present as an isolated tumor or can spread to affect internal organs. The course of EH varies, based on the tissue of origin. This case report describes a young woman who developed cutaneous EH with concurrent intracranial disease during pregnancy. After resection, the lesions returned. Even after several courses of chemotherapy and radiotherapy, the patient developed multifocal disease including pulmonary, skeletal, and liver disease. She now exhibits stable disease after approximately 6 years of therapy with lenalidomide.

PMID: 19898746 [PubMed - as supplied by publisher]
[A case of epithelioid hemangioendothelioma of the liver: detection by FDG-PET]

Fetched: November 27th, 2009, 11:19am CST

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[A case of epithelioid hemangioendothelioma of the liver: detection by FDG-PET]

Nippon Shokakibyo Gakkai Zasshi. 2009 Nov;106(11):1650-9

Authors: Katsushima S, Komeda T, Endoh B, Mizumoto Y, Watanabe T, Shima N, Adachi E, Kitaoka S, Hashiba M, Tomono T, Esaka N, Ohta Y, Tanaka Y, Morimoto H, Kuroda E, Ohta R, Maekawa T, Minamiguchi S

A 57 year-old woman was admitted for focal accumulation of 18F-fluorodeoxyglucose (FDG) in the liver detected by positron emission tomography (PET). A 25- mm hypovascular tumor was detected by computed tomography. Tumor biopsy revealed many atypical cells with positive staining for factor VIII-related-antigen in sinusoids. Right lobectomy was performed and the tumor was diagnosed as epithelioid hemangioendothelioma (EHE) pathologically. We demonstrated that FDG-PET was useful for the diagnosis of EHE and making deciding on therapeutic strategy.

PMID: 19893296 [PubMed - in process]
[Functional reconstruction of the superficial posterior compartment of the leg after tumour ablation in an infant]

Fetched: November 27th, 2009, 11:19am CST

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[Functional reconstruction of the superficial posterior compartment of the leg after tumour ablation in an infant]

Handchir Mikrochir Plast Chir. 2009 Aug;41(4):244-7

Authors: Goertz O, Lehnhardt M, Hirsch T, Stricker I, Steinau HU, Homann HH

A 33-month-old female child was diagnosed with a papillary intralymphatic angioendothelioma, a rare type of low-grade angiosarcoma, on the dorsal lower leg. The soleus and gastrocnemius muscle including the Achilles tendon and the subcutis and cutis were resected. The defect was functionally reconstructed by a neurovascular musculocutaneous latissimus dorsi free-flap transfer.

PMID: 19688654 [PubMed - indexed for MEDLINE]