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PubMed - Rare Brain Tumor (75 unread)

Sleep disturbances in craniopharyngioma: a challenging diagnosis

Fetched: September 16th, 2021, 5:01am GMT by Ramona Cordani

J Neurol. 2021 Sep 14. doi: 10.1007/s00415-021-10794-1. Online ahead of print.

ABSTRACT

Craniopharyngiomas are rare solid or mixed solid and cystic tumors that arise from Rathke's pouch remnants along the pituitary-hypothalamic axis, from the sella turcica to the brain third ventricle. Both the tumor and its treatment can lead to significant neurological and endocrinological complications. Due to the essential role of the hypothalamus in the complex neurophysiologic process of sleep, tumors involving the hypothalamic area may be responsible for disturbances in sleep-wake regulation with alterations in the circadian rhythm, sleep fragmentation, and increased daytime sleepiness. We report two cases of patients with craniopharyngioma, who came to our attention due to the occurrence of episodes characterized by psychomotor slowing and afinalistic limb movements, temporal and spatial disorientation, psychomotor agitation, and oneiric stupor like episodes. A comprehensive clinical data collection and a targeted diagnostic work-up led to a diagnosis of severe sleep disorder characterized by hypersomnia, altered sleep-wake rhythm, and sleep-related breathing disorder. In addition, the polysomnography revealed peculiar alterations in the sleep structure. The diagnostic work-up lead to an accurate differential diagnosis between epileptic seizures and episodes expressions of sleep disturbances. These clinical features can be challenging to diagnose and can lead to misdiagnosis and inappropriate treatment. Diagnosis of sleep disorders is crucial, considering the impact of sleep on general health, cognition, and neuropsychological functioning. These findings support the need to incorporate a comprehensive sleep evaluation in childhood brain tumor involving the suprasellar/hypothalamic region.

PMID:34522989 | DOI:10.1007/s00415-021-10794-1

Posterior Fossa Teratomas in Adults : A Systematic Review

Fetched: September 16th, 2021, 5:01am GMT by Dong-Won Shin

J Korean Neurosurg Soc. 2021 Sep 15. doi: 10.3340/jkns.2020.0343. Online ahead of print.

ABSTRACT

OBJECTIVE: The occurrence of posterior fossa teratomas in adulthood is extremely rare. In this study, we aimed to report our experience with two cases of posterior fossa mature teratoma in adults who underwent surgical resection. We also performed a systematic review of published papers available to date.

METHODS: We retrospectively reviewed the electronic medical records of patients who had onset of posterior fossa teratomas in adulthood at our institute between 1995 and 2020. We evaluated the clinical, radiographic, and pathological features of mature teratomas at the posterior fossa in adulthood. Furthermore, we searched the PubMed, EMBASE, and Web of Science database and reviewed published articles.

RESULTS: We found 507 articles on database review; of them, 102 were duplicates and 389 were excluded based on the inclusion criteria. Finally, 16 cases of posterior fossa from the web search and related articles. Subsequently, we added two cases that underwent surgery at our institute. We analyzed a total of 18 cases of mature teratomas. Headache was the most common (55.6%) symptom. The teratomas showed heterogeneous signals on magnetic resonance imaging. Thirteen patients (72.2%) had lesion at midline, five patients (27.8%) had calcification. Surgical resection was performed in all patients. No studies reported recurrence after resection.

CONCLUSION: The occurrence of posterior fossa teratomas in adulthood is difficult to diagnose at the initial stage. Radiographic diagnosis alone can lead to misdiagnosis. Pathological confirmation is essential. Surgical resection is a curative option for posterior fossa teratomas in adulthood.

PMID:34521185 | DOI:10.3340/jkns.2020.0343

Pituitary Apoplexy Complicated by Cerebral Infarction: A Case Report

Fetched: September 15th, 2021, 5:01am GMT by Biraj Pokhrel

JNMA J Nepal Med Assoc. 2021 Jul 30;59(239):723-726. doi: 10.31729/jnma.6120.

ABSTRACT

Cerebral infarction is a rare complication of pituitary apoplexy, which can result in significant morbidity if not treated on time. Pituitary apoplexy mostly occurs in pre-existing adenoma, which can remain undiagnosed until symptoms arise. Here, we present a case of a 26-year-old man with undiagnosed acromegaly who presented with left retro-orbital pain, diminished vision of the left eye, and right hemiparesis. Neuroimaging revealed large hemorrhagic sellar mass and ischemic infarction in the left middle cerebral artery territory. Emergency transcranial tumor excision was done, which resulted in significant neurological recovery.

PMID:34508512 | DOI:10.31729/jnma.6120

Rare Encounter of Renal Hydatid Cyst: A Case Report

Fetched: September 15th, 2021, 5:01am GMT by Prabina Basnet

JNMA J Nepal Med Assoc. 2021 Jul 30;59(239):716-718. doi: 10.31729/jnma.6994.

ABSTRACT

Cystic Echinococcosis or Hydatid disease is caused by the infection with the larval stage of long tapeworm, Echinococcus granulosus. This condition often remains asymptomatic for years before the cyst grows large enough to cause symptoms in affected organs. The most common organs involved are liver and lungs although the heart, brain, bone, central nervous system, and kidney may also be involved. This case is about a young woman who presented with left flank pain and urinary tract infection who was later diagnosed as having left renal hydatid cyst. The cyst was approximately 7.8×6.6×8cm with internal multiple septations at the lower pole cortex of the left kidney. Laparoscopic pericystectomy was performed and with no postoperative complications, she was discharged on albendazole and other supportive medication. With timely management using combination therapy, this condition is curable and the patient can live a healthy life with normal kidney function.

PMID:34508491 | DOI:10.31729/jnma.6994

Subconjunctival Mass as Rare Presentation of Even Rarer Intraocular Medulloepithelioma: A Case Report

Fetched: September 15th, 2021, 5:01am GMT by Santosh Chaudhary

JNMA J Nepal Med Assoc. 2021 Jan 31;59(233):77-80. doi: 10.31729/jnma.5269.

ABSTRACT

Medulloepithelioma is a rare childhood embryonal tumor arising from the non-pigmented ciliary epithelium of the pars plicata. We report a case of an 11-year-old male who presented with painless loss of vision of the right eye for the last three years and progressively increasing mass on the superior aspect of the globe for the last three months. On ocular examination, a firm, non-tender mass of 4cm x 3cm was noted in the superior aspect of the globe. CT-Scan of the orbit was suggestive of a foreign body with a haemorrhage or infection. The patient underwent enucleation with minimal manipulation. Histopathological examination of the enucleated globe revealed medulloepithelioma. The intraocular medulloepithelioma presentation is often late and masquerading, which may lead to extraocular extension and metastasis and ultimately unfavorable prognosis.

PMID:34508461 | PMC:PMC7893384 | DOI:10.31729/jnma.5269

Permalink for 'Detection of NTRK fusions in glioblastoma: fluorescent in situ hybridisation is more useful than pan-TRK immunohistochemistry as a screening tool prior to RNA sequencing'

Detection of NTRK fusions in glioblastoma: fluorescent in situ hybridisation is more useful than pan-TRK immunohistochemistry as a screening tool prior to RNA sequencing

Fetched: September 15th, 2021, 5:01am GMT by Amélie Bourhis

Pathology. 2021 Sep 10:S0031-3025(21)00429-3. doi: 10.1016/j.pathol.2021.05.100. Online ahead of print.

ABSTRACT

Glioblastomas are frequent malignant brain tumours with a very poor prognosis and a need for new and efficient therapeutic strategies. With the approval of anti-TRK targeted therapies to treat patients with advanced NTRK-rearranged cancers, independent of the type of cancer, potential new treatment opportunities are available for the 0.5-5% of patients with NTRK-rearranged glioblastomas. Identification of these rare NTRK-rearranged glioblastomas requires efficient diagnostic tools and strategies which are evaluated in this study. We compared the results of NTRK1, NTRK2 and NTRK3 fluorescent in situ hybridisation (FISH) assays to those of pan-TRK immunohistochemistry (IHC) using two EPR17341 and A7H6R clones in a set of 196 patients with glioblastomas. Cases with at least 15% of positive nuclei using FISH analyses were further analysed using RNA sequencing. Above the 15% threshold, seven positive glioblastomas (3.57%) were identified by FISH assays (4 NTRK1, 3 NTRK2, no NTRK3). NTRK rearrangements were confirmed by RNA sequencing analyses in four cases [1 LMNA-NTRK1, 1 PRKAR2A-NTRK2, 1 SPECC1L-NTRK2 and 1 NACC2-NTRK2 fusions, i.e., 4/196 (2%) of NTRK-rearranged tumours in our series] but no rearrangement was detected in three samples with less than 30% of positive tumour nuclei as determined by NTRK1 FISH. Pan-TRK immunostaining showed major discrepancies when using either the EPR17341 or the A7H6R clones for the following criteria: main intensity, H-Score based scoring and homogeneity/heterogeneity of staining (Kappa values <0.2). This led to defining adequate criteria to identify NTRK-rearranged gliomas exhibiting strong and diffuse immunostaining contrasting to the variable and heterogeneous staining in non-NTRK-rearranged gliomas (p<0.0001). As assessing NTRK rearrangements has become crucial for glioma therapy, FISH seems to be a valuable tool to maximise access to TRK testing in patients with glioblastomas. In contrast to other cancers, pan-TRK IHC appears of limited interest in this field because there is no 'on/off' IHC positivity criterion to distinguish between NTRK-rearranged and non-NTRK-rearranged gliomas. RNA sequencing analyses are necessary in FISH positive cases with less than 30% positive nuclei, to avoid false positivity when scoring is close to the detection threshold.

PMID:34518039 | DOI:10.1016/j.pathol.2021.05.100

Permalink for 'Gamma Knife Radiosurgery-Based Combination Treatment Strategies Improve Survival in Patients With Central Nervous System Metastases From Epithelial Ovarian Cancer: A Retrospective Analysis of Two Academic Institutions in Korea and Taiwan'

Gamma Knife Radiosurgery-Based Combination Treatment Strategies Improve Survival in Patients With Central Nervous System Metastases From Epithelial Ovarian Cancer: A Retrospective Analysis of Two Academic Institutions in Korea and Taiwan

Fetched: September 14th, 2021, 5:01am GMT by Yen-Ling Lai

Front Oncol. 2021 Aug 27;11:719936. doi: 10.3389/fonc.2021.719936. eCollection 2021.

ABSTRACT

Central nervous system (CNS) metastases from epithelial ovarian cancer (EOC) are rare. We investigated the clinico-pathological prognostic factors of patients with CNS metastases from EOC and compared the outcomes of various treatment modalities. We retrospectively reviewed the records of patients with CNS metastases from EOC between 2000 and 2020. Information on the clinical and pathological characteristics, treatment, and outcomes of these patients was retrieved from Samsung Medical Center and National Taiwan University Hospital. A total of 94 patients with CNS metastases were identified among 6,300 cases of EOC, resulting in an incidence of 1.49%. Serous histological type [hazard ratio (HR): 0.49 (95% confidence interval [CI] 0.25-0.95), p=0.03], progressive disease [HR: 2.29 (95% CI 1.16-4.54), p=0.01], CNS involvement in first disease relapse [HR: 0.36 (95% CI 0.18-0.70), p=0.002], and gamma knife radiosurgery (GKS)-based combination treatment for EOC patients with CNS lesions [HR: 0.59 (95% CI 0.44-0.79), p<0.001] significantly impacted survival after diagnosis of CNS metastases. In a subgroup analysis, superior survival was observed in patients with CNS involvement not in first tumor recurrence who underwent GKS-based combination therapeutic regimens. The survival benefit of GKS-based treatment was not significant in patients with CNS involvement in first disease relapse, but a trend for longer survival was still observed. In conclusion, GKS-based combination treatment can be considered for the treatment of EOC patients with CNS metastases. The patients with CNS involvement not in first disease relapse could significantly benefit from GKS-based combination strategies.

PMID:34513698 | PMC:PMC8429898 | DOI:10.3389/fonc.2021.719936

Medulloblastoma in an Adult Female Patient: A Rare Presentation

Fetched: September 14th, 2021, 5:01am GMT by Shobha Mandal

Cureus. 2021 Jul 29;13(7):e16713. doi: 10.7759/cureus.16713. eCollection 2021 Jul.

ABSTRACT

Medulloblastoma (MB) is an aggressive malignant tumor of the posterior fossa of the CNS that mainly affects children younger than 15 years of age. It is uncommon in the adult population compared to children. Any adult patient presenting with cerebellar mass must be evaluated with brain tissue biopsy to rule out MB. Our patient is a 27-year-old female who presented with sudden onset of frontal headache and was diagnosed with MB.

PMID:34513353 | PMC:PMC8405177 | DOI:10.7759/cureus.16713

Brain abscess of odontogenic origin in patients with malignant tumors: A report of two cases

Fetched: September 14th, 2021, 5:01am GMT by Teishiki Shibata

Surg Neurol Int. 2021 Aug 16;12:417. doi: 10.25259/SNI_541_2021. eCollection 2021.

ABSTRACT

BACKGROUND: Oral infection and dental manipulations can lead to the development of brain abscesses, a rare but potentially life-threatening condition. Herein, we report patients undergoing cancer treatment who developed brain abscesses of odontogenic origin at our hospital.

CASE DESCRIPTION: Two patients developed brain abscesses during cancer treatment. Both underwent neurosurgical aspiration, and the causative microorganism was identified as Streptococcus intermedius of the Streptococcus anginosus group, which is a part of the normal bacterial flora in the oral cavity. There was clinical and radiographic evidence of dental infection in one of the patients diagnosed with a brain abscess of odontogenic origin. No infectious foci were found in the other patient during hospitalization for the abscess. However, the patient had undergone extraction of an infected tooth approximately 3 months before admission for the abscess, suggesting origination from an oral infection or dental manipulation. The patients' cancers rapidly worsened because cancer treatment in both patients was interrupted for several months to treat the brain abscess.

CONCLUSION: Oral infections can cause severe infections, such as brain abscesses, particularly during the treatment of malignant tumors. Improving the oral environment or treating oral infections before initiating treatment for malignant tumors is highly recommended. In addition, the possibility of odontogenic origin should always be considered as a potential etiology of brain abscesses.

PMID:34513181 | PMC:PMC8422424 | DOI:10.25259/SNI_541_2021

Permalink for 'Indolent nonendemic central nervous system histoplasmosis presenting as an isolated intramedullary enhancing spinal cord lesion'

Indolent nonendemic central nervous system histoplasmosis presenting as an isolated intramedullary enhancing spinal cord lesion

Fetched: September 14th, 2021, 5:01am GMT by Matthew J Recker

Surg Neurol Int. 2021 Aug 9;12:392. doi: 10.25259/SNI_345_2021. eCollection 2021.

ABSTRACT

BACKGROUND: Histoplasma capsulatum infection is largely seen in endemic regions; it results in symptomatic disease in <5% of those infected and is most often a self-limiting respiratory disease. Disseminated histoplasmosis is considered rare in the immunocompetent host. Central nervous system (CNS) dissemination can result in meningitis, encephalitis, and focal lesions in the brain and spinal cord, stroke, and hydrocephalus. An intramedullary spinal cord lesion as the only manifestation of CNS histoplasmosis has been rarely described.

CASE DESCRIPTION: We present an atypical case of a 44-year-old man from a nonendemic region, on adalimumab therapy for ulcerative colitis who developed an isolated intramedullary spinal cord lesion in the setting of disseminated histoplasmosis. His course was initially indolent with vague systemic symptoms that led to consideration of several other diagnoses including sarcoidosis and lymphoma. Biopsies of several positron emission tomography positive lymph nodes revealed granulomatous inflammation, but no firm diagnosis was achieved. He was ultimately diagnosed with histoplasmosis after an acute respiratory infection in the setting of anti-tumor necrosis factor therapy. With appropriate antifungal therapy, the spinal cord lesion regressed. The previous systemic biopsies were re-reviewed, and rare fungal elements consistent with H. capsulatum were identified. A presumptive diagnosis of CNS histoplasmosis was made in the absence of direct laboratory confirmation in the setting of rapid and complete resolution on antifungal therapy.

CONCLUSION: Disseminated histoplasmosis should be considered in granulomatous disease, even if the patient resides in a nonendemic region. Furthermore, clinicians should be mindful that CNS histoplasmosis may present in an atypical fashion.

PMID:34513158 | PMC:PMC8422457 | DOI:10.25259/SNI_345_2021

Parasagittal cystic meningioma mimicking hemangioblastoma: A case report

Fetched: September 14th, 2021, 5:01am GMT by Abiodun Idowu Okunlola

Surg Neurol Int. 2021 Jul 27;12:368. doi: 10.25259/SNI_507_2021. eCollection 2021.

ABSTRACT

BACKGROUND: Cystic meningioma is a rare variety with similar histological profiles like the solid tumors. It has been documented in both supratentorial and infratentorial compartments presenting radiologically as a large cyst with mural nodule mimicking hemangioblastoma.

CASE DESCRIPTION: We managed a middle-aged woman who presented with recurrent seizures and brain MRI revealed left frontal cystic parasagittal tumor with mural nodule. She had left frontal awake craniotomy and gross total tumor excision. Histology confirmed meningothelial meningioma.

CONCLUSION: Cystic meningioma is rare but should be high in differentials of cystic intracranial tumor with dural based nodules. Awake craniotomy is possible for the excision of parasagittal tumor most especially when it is frontal in location.

PMID:34513135 | PMC:PMC8422409 | DOI:10.25259/SNI_507_2021

Pineal Anaplastic Ependymoma - A Rare Entity

Fetched: September 12th, 2021, 5:01am GMT by Pooja E Moorthy

Neurol India. 2021 Jul-Aug;69(4):1045-1047. doi: 10.4103/0028-3886.325365.

ABSTRACT

Anaplastic ependymoma in the pineal region is rare. Here, we present a rare case of anaplastic ependymoma of the pineal region on a 42-year-old woman who came to our hospital with headache associated with blurring of vision since one month. MRI brain showed a contrast enhancing mass lesion measuring 30 × 30 × 35 mm in the pineal region with obstructive hydrocephalus. Initially, the ventriculoperitoneal (VP) shunt was done, followed by total tumor excision by the infratentorial supracerebellar approach. Histopathological and immunohistochemistry examinations of the tumor showed the features of anaplastic ependymoma (WHO Grade III). Patient made uneventful recovery and underwent radiotherapy. Only 12 cases of pineal ependymoma have been reported so far, of which only three have been anaplastic ependymoma.

PMID:34507442 | DOI:10.4103/0028-3886.325365

Identification of novel SSX1 fusions in synovial sarcoma

Fetched: September 12th, 2021, 5:01am GMT by Akihiko Yoshida

Mod Pathol. 2021 Sep 9. doi: 10.1038/s41379-021-00910-x. Online ahead of print.

ABSTRACT

Synovial sarcoma is characterized by variable epithelial differentiation and specific SS18-SSX gene fusions. The diagnosis is primarily based on phenotype, but fusion gene detection is increasingly being considered indispensable, with SS18 break-apart fluorescence in situ hybridization (FISH) being favored in many laboratories. However, SS18 FISH assay produces negative or atypical results in a minority of cases, leaving uncertainties in diagnosis and management. Here, we analyzed this challenging subset of SS18 FISH-negative/atypical synovial sarcoma using RNA sequencing and monoclonal antibodies that recognize SS18-SSX and the SSX C-terminus. Among 99 synovial sarcoma cases that were previously subjected to SS18 break-apart FISH, eight cases were reported as negative and three cases were indeterminate, owing to atypical signal patterns. Three of these 11 tumors (two monophasic and one biphasic) harbored novel EWSR1-SSX1 fusions, were negative for SS18-SSX staining, and were positive for SSX C-terminus staining. One monophasic tumor harbored a novel MN1-SSX1 fusion, and showed negative SS18-SSX expression and positive SSX C-terminus staining. Another monophasic tumor carried an SS18L1-SSX1 fusion, and was weakly positive for SS18-SSX, while SMARCB1 expression was reduced. The presence of these novel and/or rare fusions was confirmed using RT-PCR and Sanger sequencing. EWSR1-SSX1 was further validated by EWSR1 FISH assay. The remaining six tumors (five monophasic and one biphasic) showed strong SS18-SSX expression, and RNA sequencing successfully performed in three cases identified canonical SS18-SSX2 fusions. Based on a DNA methylation-based unsupervised clustering, the tumors with EWSR1-SSX1 and SS18L1-SSX1 clustered with synovial sarcoma, while the MN1-SSX1-positive tumor was not co-clustered despite classic histology and immunoprofile. In summary, we discovered novel and rare SSX1 fusions to non-SS18 genes in synovial sarcoma. The expanded genetic landscape carries significant diagnostic implications and advances our understanding of the oncogenic mechanism.

PMID:34504309 | DOI:10.1038/s41379-021-00910-x

Targeted Therapies in Rare Brain Tumours

Fetched: September 10th, 2021, 5:01am GMT by Francesco Bruno

Int J Mol Sci. 2021 Jul 26;22(15):7949. doi: 10.3390/ijms22157949.

ABSTRACT

Rare central nervous system (CNS) tumours represent a unique challenge. Given the difficulty of conducting dedicated clinical trials, there is a lack of therapies for these tumours supported by high quality evidence, and knowledge regarding the impact of standard treatments (i.e., surgery, radiotherapy or chemotherapy) is commonly based on retrospective studies. Recently, new molecular techniques have led to the discovery of actionable molecular alterations. The aim of this article is to review recent progress in the molecular understanding of and therapeutic options for rare brain tumours, both in children and adults. We will discuss options such as targeting the mechanistic target of rapamycin (mTOR) pathway in subependymal giant cells astrocytomas (SEGAs) of tuberous sclerosis and BRAF V600E mutation in rare glial (pleomorphic xanthoastrocytomas) or glioneuronal (gangliogliomas) tumours, which are a model of how specific molecular treatments can also favourably impact neurological symptoms (such as seizures) and quality of life. Moreover, we will discuss initial experiences in targeting new molecular alterations in gliomas, such as isocitrate dehydrogenase (IDH) mutations and neurotrophic tyrosine receptor kinase (NTRK) fusions, and in medulloblastomas such as the sonic hedgehog (SHH) pathway.

PMID:34360713 | PMC:PMC8348084 | DOI:10.3390/ijms22157949

Two Cases of Hypovascular Pituitary Metastases and Literature Review

Fetched: September 10th, 2021, 5:01am GMT by Ning Zhao

Zhongguo Yi Xue Ke Xue Yuan Xue Bao. 2021 Aug;43(4):663-668. doi: 10.3881/j.issn.1000-503X.12939.

ABSTRACT

Pituitary metastasis,a rare kind of intracranial malignant tumor,is characterized by metastasis from all parts of the body to the pituitary.The common tumors metastatic to pituitary are abundant with blood supply,located in the posterior pituitary lobe and/or the pituitary stalk.The lesion shows infiltrative growth,and mainly demonstrates low signal on T1 weighted imaging and high signal on T2 weighted imaging.It is usually enhanced significantly after iodinated contrast administration.The metastatic tumor with poor blood supply is similar to pituitary macroadenoma on magnetic resonance image,which makes it difficult to be differentiated.We reported two cases of pathologically diagnosed pituitary metastasis with poor blood supply and reviewed related papers,aiming to provide the imaging differentiation points of hypovascular pituitary metastasis.

PMID:34494542 | DOI:10.3881/j.issn.1000-503X.12939

Paraneoplastic limbic encephalitis complicating pulmonary adenocarcinoma: a case report

Fetched: September 10th, 2021, 5:01am GMT by Houda Snene

Pan Afr Med J. 2021 Jun 2;39:95. doi: 10.11604/pamj.2021.39.95.26568. eCollection 2021.

ABSTRACT

Limbic encephalitis (LE) is a rare disease often of paraneoplastic origin. It is frequently associated with bronchopulmonary cancer. Diagnosis is based on brain magnetic resonance imaging (MRI). We here report the case of a 54-year-old female patient with a history of active smoking, presenting with chronic dry cough. Chest X-ray showed suspicious right lung opacity. Bronchial fibroscopy and bronchial biopsies were not contributory. Thoraco-abdomino-pelvic computed tomography (CT)-scan and brain CT-scan showed a mass in the right upper lobe classified as T4N2M1a. CT-guided lung biopsy confirmed the diagnosis of bronchopulmonary adenocarcinoma. The patient had reported a recent history of memory disturbances associated with depressed mood, anxiety and paroxysmal confusion. Metabolic screening and tests for detecting infection were normal and brain MRI suggested limbic encephalitis. The evolution was characterized by rapid disease progression; the patient died in about ten days.

PMID:34466197 | PMC:PMC8379404 | DOI:10.11604/pamj.2021.39.95.26568

Molecular profiling of pediatric meningiomas shows tumor characteristics distinct from adult meningiomas

Fetched: September 9th, 2021, 5:01am GMT by Elmar Kirches

Acta Neuropathol. 2021 Sep 8. doi: 10.1007/s00401-021-02351-x. Online ahead of print.

ABSTRACT

In contrast to adults, meningiomas are uncommon tumors in childhood and adolescence. Whether adult and pediatric meningiomas differ on a molecular level is unclear. Here we report detailed genomic analyses of 37 pediatric meningiomas by sequencing and DNA methylation profiling. Histologically, the series was dominated by meningioma subtypes with aggressive behavior, with 70% of patients suffering from WHO grade II or III meningiomas. The most frequent cytogenetic aberrations were loss of chromosomes 22 (23/37 [62%]), 1 (9/37 [24%]), 18 (7/37 [19%]), and 14 (5/37 [14%]). Tumors with NF2 alterations exhibited overall increased chromosomal instability. Unsupervised clustering of DNA methylation profiles revealed separation into three groups: designated group 1 composed of clear cell and papillary meningiomas, whereas group 2A comprised predominantly atypical meningiomas and group 2B enriched for rare high-grade subtypes (rhabdoid, chordoid). Meningiomas from NF2 patients clustered exclusively within groups 1 and 2A. When compared with a dataset of 105 adult meningiomas, the pediatric meningiomas largely grouped separately. Targeted panel DNA sequencing of 34 tumors revealed frequent NF2 alterations, while other typical alterations found in adult non-NF2 tumors were absent. These data demonstrate that pediatric meningiomas are characterized by molecular features distinct from adult tumors.

PMID:34495383 | DOI:10.1007/s00401-021-02351-x

Diffuse leptomeningeal glioneuronal tumour (DLGNT) in children: the emerging role of genomic analysis

Fetched: September 9th, 2021, 5:01am GMT by Neevika Manoharan

Acta Neuropathol Commun. 2021 Sep 7;9(1):147. doi: 10.1186/s40478-021-01248-w.

ABSTRACT

Diffuse leptomeningeal glioneuronal tumours (DLGNT) represent rare enigmatic CNS tumours of childhood. Most patients with this disease share common radiological and histopathological features but the clinical course of this disease is variable. A radiological hallmark of this disease is widespread leptomeningeal enhancement that may involve the entire neuroaxis with predilection for the posterior fossa and spine. The classic pathologic features include low- to moderate-density cellular lesions with OLIG2 expression and evidence of 'oligodendroglioma-like' appearance. The MAPK/ERK signaling pathway has recently been reported as a potential driver of tumourigenesis in up to 80% of DLGNT with KIAA1549:BRAF fusions being the most common event seen. Until now, limited analysis of the biological drivers of tumourigenesis has been undertaken via targeted profiling, chromosomal analysis and immunohistochemistry. Our study represents the first examples of comprehensive genomic sequencing in DLGNT and shows that it is not only feasible but crucial to our understanding of this rare disease. Moreover, we demonstrate that DLGNT may be more genomically complex than single-event MAPK/ERK signaling pathway tumours.

PMID:34493325 | PMC:PMC8422739 | DOI:10.1186/s40478-021-01248-w

Mediastinal ectopic thyroid tissue, an imitator of an enlarged lymph node with metastatic pulmonary neoplasia

Fetched: September 8th, 2021, 5:01am GMT by Alan Tsai

Diagn Cytopathol. 2021 Sep 7. doi: 10.1002/dc.24866. Online ahead of print.

ABSTRACT

The prevalence of ectopic thyroid tissue, based on autopsy studies, is between 7% and 10%, but there are rare cases reported in the thoracic region. Here, we encountered a case of thoracic ectopic thyroid tissue presenting as a presumed enlarged mediastinal lymph node. A 50-year-old female with a history of lung adenocarcinoma, status post resection, presented with complaints of headache, dizziness, and nausea. Magnetic resonance imaging found two brain lesions consistent with metastasis. Computed tomography scan showed enlarged mediastinal lymph nodes and thyroid nodules. Fine-needle aspiration (FNA) of one thyroid nodule was positive for papillary thyroid carcinoma. FNA of the mediastinal lymph nodes were negative for metastatic carcinoma but revealed thyroid tissue in the 2.9 × 1.6 cm presumed 2 L lymph node. The morphological features and immunohistochemical stains confirmed thyroid tissue, and there were no cytological features of thyroid carcinoma. In patients with a history of a pulmonary tumor (such as adenocarcinoma, low-grade neuroendocrine tumor), ectopic thyroid tissue, although a rare event, could represent a pitfall in the cytologic evaluation of mediastinal lymph nodes aspirates obtained from staging procedures. Careful morphologic examination with a panel of immunohistochemical studies are useful in making the correct diagnosis, leading to appropriate patient management.

PMID:34492168 | DOI:10.1002/dc.24866

Neuroinflammation Associated With Tumor Necrosis Factor-alpha Inhibitor Exposure

Fetched: September 8th, 2021, 5:01am GMT by Amy W Yu

Neurol Clin Pract. 2021 Aug;11(4):e488-e496. doi: 10.1212/CPJ.0000000000001014.

ABSTRACT

OBJECTIVE: To identify the frequency and clinical spectrum of neuroinflammation associated with exposure to tumor necrosis factor-α inhibitors (TNFi).

METHODS: We performed a single-system retrospective cohort study. Adults in the Yale New Haven Health System with documented use of any US Food and Drug Administration-approved TNFi between 2007 and 2017 were identified via automated review of the electronic medical record. Those who also had brain MRIs were identified and categorized as either TNFi exposed or unexposed. Individuals with MRI findings concerning for neuroinflammation were identified, and detailed chart reviews were performed.

RESULTS: A total of 4,391 patients received TNFi, and 547 also had brain MRI. After exclusion criteria were applied, 375 MRIs occurred after TNFi exposure, and 132 MRIs occurred before TNFi. MRIs were normal for 20.8% of exposed patients. The most common abnormal finding was nonspecific, punctate T2 hyperintensities. Seventeen cases (4.5%) among the exposed cohort had findings consistent with neuroinflammation, of which 58.8% required TNFi discontinuation and additional immunotherapy, whereas an additional 23.5% discontinued TNFi alone. After 3 years, 70.6% had stable MRI findings, whereas 11.8% demonstrated progression. The 10-year period prevalence of neuroinflammation in all subjects exposed to TNFi was 0.4%.

CONCLUSIONS: Neuroinflammatory phenomena following TNFi are a common concern for those treating patients with autoimmune disease. This is a large-scale study identifying the epidemiology surrounding this phenomenon. TNFi-associated inflammation was a rare outcome in our cohort. Most treated patients had either normal or nonspecific MRI findings. Further risk stratification parameters need to be identified.

PMID:34484946 | PMC:PMC8382406 | DOI:10.1212/CPJ.0000000000001014

Permalink for 'Case report of an elderly woman with atypical imaging for primary central nervous system lymphoma who needed a brain biopsy for diagnosis'

Case report of an elderly woman with atypical imaging for primary central nervous system lymphoma who needed a brain biopsy for diagnosis

Fetched: September 8th, 2021, 5:01am GMT by Kosuke Matsuzono

J Int Med Res. 2021 Aug;49(8):3000605211035197. doi: 10.1177/03000605211035197.

ABSTRACT

Primary central nervous system lymphoma (PCNSL) is a rare form of non-Hodgkin lymphoma, but its diagnosis is challenging in some cases. A brain biopsy is the gold standard for diagnosing PCNSL, but its invasiveness can be problematic. Thus, noninvasive imaging examinations have been developed for the pre-surgical diagnosis of PCNSL, including gadolinium-enhanced magnetic resonance imaging (MRI), ¹²³I-N-isopropyl-p-iodoamphetamine single-photon emission computed tomography (¹²³I-IMP SPECT), and positron emission tomography with ¹⁸F-fluorodeoxyglucose (¹⁸F-FDG PET). Here, we report the case of a 71-year-old woman with negative imaging findings for PCNSL, but who was diagnosed with PCNSL by a brain biopsy and histological analysis. Her imaging results were negative for gadolinium-enhanced cranial MRI, with low uptake in ¹²³I-IMP SPECT and hypometabolism in ¹⁸F-FDG PET. However, a stereotactic brain biopsy from an abnormal lesion revealed that many round cells had infiltrated into the brain. Moreover, many infiltrating cells were positive for cluster of differentiation (CD)20 and CD79a, and proliferation marker protein Ki-67-positive cells accounted for nearly 80% of all cells. Based on these results, our final pathological diagnosis was PCNSL. The present case highlights the possibility of a PCNSL diagnosis even when all imaging-related examinations display negative results.

PMID:34461767 | PMC:PMC8414941 | DOI:10.1177/03000605211035197

METASTATIC CHORIOCARCINOMA PRESENTING AS RENAL COLIC AND SKIN LESION - CASE REPORT

Fetched: September 8th, 2021, 5:01am GMT by Michał Pietrus

Wiad Lek. 2021;74(7):1763-1766.

ABSTRACT

OBJECTIVE: Choriocarcinoma is a rare malignant disease that is usually associated with a gestational event. Kidney metastasis might be misdiagnosed as renal cell carcinoma or kidney abscess. To the best of our knowledge, only 13 cases of cutaneous metastasis of choriocarcinoma have been reported in the literature so far. We report a case of choriocarcinoma that manifested with multiple metastases to the lung, skin, kidney and brain. Case report: We reported a case of a 37-year-old woman with a history of hydatiform mole, with symptoms of renal colic and abnormal findings on the skin. Chest X-ray revealed visible focal change 80 mm in diameter, located in the left lung area. The CT exposed in both kidneys multiple hypodense foci, 32 mm in size, suggesting multifocal abscesses with disruptions and perforation to paranephric area. Due to the presence of and temporary loss of vision in the right eye head CT was performed revealing metastatic changes in the brain. The diferential diagnosis between renal cancer, lung carcinoma and choriocarcinoma was achieved only after surgical removal skin lesion. This was the first time in our experience with choriocarcinoma. Immunohistochemically, the analysis was positive for beta hCG, cytokeratin AE1/AE, CK 8/18, CD10, EMA, alfa 1-inhibin and negative for protein 63, CD30 and CD117. Serum hCG level was 394590,0 mIU/mL.

CONCLUSION: Conclusions: Choriocarcinoma should be taken into consideration when associated symptoms and significantly elevated blood levels of β-hCG were identified.

PMID:34459784

Permalink for 'Microscopic biopsy after unsuccessful endoscopic biopsy for primary central nervous system lymphoma of the corpus callosum: A case report'

Microscopic biopsy after unsuccessful endoscopic biopsy for primary central nervous system lymphoma of the corpus callosum: A case report

Fetched: September 4th, 2021, 5:00am GMT by Tokunori Kanazawa

Ann Med Surg (Lond). 2021 Aug 24;69:102746. doi: 10.1016/j.amsu.2021.102746. eCollection 2021 Sep.

ABSTRACT

INTRODUCTION AND IMPORTANCE: Primary central nervous system lymphoma (PCNSL) is a rare tumor with a poor prognosis. Early brain biopsy is essential to avoid a diagnostic delay. To date, reports of successful diagnosis for PCNSL of the corpus callosum by endoscopic biopsy are rare.

CASE PRESENTATION: Herein, we report the case of an elderly woman with PCNSL of the corpus callosum who initially presented with rapidly progressive dementia. The condition was finally diagnosed by microscopic biopsy after unsuccessful endoscopic biopsy. Moreover, the postoperative course was uneventful. She is currently receiving systemic chemotherapy.

CLINICAL DISCUSSION: Early diagnosis and subsequent systemic chemotherapy with or without whole brain radiotherapy are critical for PCNSL. Endoscopic biopsy may be a diagnostic option for suspected PCNSL, although stereotactic needle biopsy is most commonly used.

CONCLUSION: Utilizing neuronavigation and 5-aminolevulinic acid (ALA) fluorescence guidance could be helpful in identifying lesions insufficiently exposed by endoscopic visualization. However, cerebrospinal fluid (CSF) loss due to the endoscopic approach through the ventricle might be a cause of neuronavigation misregistration.

PMID:34471530 | PMC:PMC8387894 | DOI:10.1016/j.amsu.2021.102746

Neurocutaneous melanocytosis-associated malignant melanoma presenting with peritoneal seeding

Fetched: September 1st, 2021, 5:01am GMT by Ugur Sener

Pediatr Dermatol. 2021 Aug 31. doi: 10.1111/pde.14789. Online ahead of print.

ABSTRACT

Neurocutaneous melanocytosis (NCM) is characterized by melanocyte deposition in the leptomeninges and brain parenchyma, primarily occurring in children with large or giant congenital melanocytic nevi (LCMN) or multiple congenital melanocytic nevi. Patients with NCM may develop hydrocephalus and increased intracranial pressure, which can be managed with ventriculoperitoneal (VP) shunting. We present the case of a 16-month-old girl who developed peritoneal carcinomatosis and malignant ascites following VP shunting for hydrocephalus secondary to NCM to increase awareness of this rare, but serious, complication of cerebrospinal fluid diversion.

PMID:34463389 | DOI:10.1111/pde.14789

Permalink for 'Selective extension of cerebral vascular calcification in an autopsy case of Fahr's syndrome associated with asymptomatic hypoparathyroidism'

Selective extension of cerebral vascular calcification in an autopsy case of Fahr's syndrome associated with asymptomatic hypoparathyroidism

Fetched: September 1st, 2021, 5:01am GMT by Tamaki Iwase

Neuropathology. 2021 Aug 30. doi: 10.1111/neup.12760. Online ahead of print.

ABSTRACT

We report an autopsy case of Fahr's syndrome in an 85-year-old woman associated with asymptomatic hypoparathyroidism. The patient was diagnosed as having brain calcification at 65 years of age. She developed mild dementia at 75, parkinsonism at 76, and severe dementia at 82. Computed tomography revealed extensive, symmetric intracranial calcification, involving both sides of the basal ganglia and cerebellar dentate nuclei, and severe cerebral atrophy that developed afterwards. A neuropathological examination revealed intracranial calcification, particularly in the wall of the arterioles and capillaries having numerous calcium deposits. Severe vascular calcification and severe neuronal loss without α-synuclein accumulation were found in the substantia nigra. There were high-level neuropathological changes indicative of Alzheimer's disease. Although the colocalization of calcium and amyloid-β deposits in the same arterial wall was rare, both of them were located in a similar layer of the arterial wall. The vascular calcification in the basal ganglia spread continuously through the corona radiata into the selective cerebral areas along the medullary arteries, but did not involve the corpus callosum or insular region. Stone formation was observed at the corona radiata adjacent to the superolateral angles of the lateral ventricles. We hypothesized that there would be a stereotypical extension pattern of vascular calcification related to the arrangement of penetrating arteries in Fahr's syndrome.

PMID:34462978 | DOI:10.1111/neup.12760

Central nervous system metastases in thymic epithelial tumors: a brief report of real-world insight from RYTHMIC

Fetched: August 30th, 2021, 5:01am GMT by J C Benitez

J Thorac Oncol. 2021 Aug 26:S1556-0864(21)02400-X. doi: 10.1016/j.jtho.2021.08.008. Online ahead of print.

ABSTRACT

Thymic epithelial tumors (TETs) are rare malignancies ranging from indolent thymoma A to aggressive thymic carcinomas (TC). Brain metastases are extremely infrequent for TETs and have only been described in case reports or small single-center series. RYTHMIC (Réseau tumeurs THYMiques et Cancer) is a French nationwide network mandated to systematically review every TET case, and prospectively includes all consecutive patients discussed by national or regional tumor boards. We analyzed patients with TETs and CNS metastasis during their cancer history from this large French registry. Over an 8-year period, 2909 patients were included in the database, including 248 TC (8.5%). Fourteen patients had central nervous system (CNS) metastases, 5 (36%) at diagnosis and 9 (64%) at relapse. Among them, 12 (86%) patients had a diagnosis of TC and 2 (14%) had thymoma A and B3. Surgical biopsies were performed and the histological subtype for non-TC tumors was centrally confirmed. Median overall survival (OS) was 22 months (95%CI 9.8-34.2), with longer, albeit non-significant, OS when CNS metastases were present at diagnosis vs relapse (not reached vs 17 months; p=0.29); median progression-free survival was 13 vs 8 months (p=0.06) respectively. A higher risk of death (HR=5.34, 95%CI [1.3-21.9]; p=0.02) and relapse (HR=1.89, 95%CI [0.9-3.7]; p=0.06) was observed for patients suffering from TC with brain metastases compared to those without CNS extension. CNS disease was extremely rare in our TET cohort (0.48%), reported at both diagnosis and progression, present primarily in TC, with prevalence rising to 4.9%.

PMID:34455064 | DOI:10.1016/j.jtho.2021.08.008

Permalink for 'A first report of a rare TP53 variant associated with Li-Fraumeni syndrome manifesting as invasive breast cancer and malignant solitary fibrous tumor'

A first report of a rare TP53 variant associated with Li-Fraumeni syndrome manifesting as invasive breast cancer and malignant solitary fibrous tumor

Fetched: August 29th, 2021, 5:01am GMT by Juraj Prejac

World J Surg Oncol. 2021 Aug 27;19(1):254. doi: 10.1186/s12957-021-02370-8.

ABSTRACT

BACKGROUND: Li-Fraumeni is a rare autosomal dominant cancer predisposition syndrome. The basis is a germline mutation of TP53 gene which encodes tumor suppressor protein resulting in early onset of tumors, most often breast cancer, soft tissue sarcomas, brain tumors, adrenocortical carcinomas, and leukemia.

CASE REPORT: We present a case of a young woman with a positive family history for cancer diagnosed with malignant solitary fibrous tumor and luminal B-like invasive breast cancer. Breast cancer and sarcomas account for the majority of tumors associated with Li-Fraumeni syndrome, yet solitary fibrous tumor is a rare clinical entity with no established guidelines for treatment. Even though both primary tumors were successfully resected, the sarcoma relapsed in the form of lung metastases. The NGS analysis revealed single nucleotide variant (c.1101-1G>A) in TP53 gene, affecting the acceptor splice site at intron 10. Until now, only one case of this genetic variant has been documented with conflicting interpretations of pathogenicity.

CONCLUSIONS: The knowledge of TP53 mutation status is essential since the management of these patients requires different approach to avoid excessive toxicity due to the risk of developing secondary malignancy. Using the clinical criteria to screen for affected individuals facilitates appropriate early genetic counseling of patients and their families. Following the American College of Medical Genetics criteria, we believe that the reported single nucleotide variant (c.1101-1G>A) in TP53 gene should be considered pathogenic.

PMID:34452612 | PMC:PMC8399826 | DOI:10.1186/s12957-021-02370-8

Detection of Human Papillomavirus Integration in Brain Metastases from Oropharyngeal Tumors by Targeted Sequencing

Fetched: August 29th, 2021, 5:01am GMT by Brian McEllin

Viruses. 2021 Aug 3;13(8):1536. doi: 10.3390/v13081536.

ABSTRACT

Human papillomavirus (HPV) positive and negative head and neck squamous cell carcinoma (HNSCC) are known to have differential phenotypes, including the incidence and location of metastases. HPV positive (HPV+) HNSCC are more likely to metastasize to distant sites, such as the lung, brain, and skin. Among these locations, metastasis to the brain is a rare event, and little is known about specific risk factors for this phenotype. In this report, we describe two patients who developed brain metastases from HNSCC. Both patient tumors had p16^INK4a overexpression, suggesting these tumors were HPV+. This was confirmed after PCR, in situ hybridization, and mass spectrometry detected the presence of HPV type 16 (HPV16) DNA, RNA and protein. To further characterize the presence of HPV16, we used a target enrichment strategy on tumor DNA and RNA to isolate the viral sequences from the brain metastases. Analysis by targeted next generation sequencing revealed that both tumors had the HPV genome integrated into the host genome at known hotspots, 8q24.21 and 14q24.1. Applying a similar target enrichment strategy to a larger cohort of HPV+ HNSCC brain metastases could help to identify biomarkers that can predict metastasis and/or identify novel therapeutic options.

PMID:34452401 | PMC:PMC8402651 | DOI:10.3390/v13081536

Epigenetic Deregulation of the Histone Methyltransferase KMT5B Contributes to Malignant Transformation in Glioblastoma

Fetched: August 29th, 2021, 5:01am GMT by Virginia López

Front Cell Dev Biol. 2021 Aug 10;9:671838. doi: 10.3389/fcell.2021.671838. eCollection 2021.

ABSTRACT

Glioblastoma multiforme (GBM) is the most common and aggressive type of brain tumor in adulthood. Epigenetic mechanisms are known to play a key role in GBM although the involvement of histone methyltransferase KMT5B and its mark H4K20me2 has remained largely unexplored. The present study shows that DNA hypermethylation and loss of DNA hydroxymethylation is associated with KMT5B downregulation and genome-wide reduction of H4K20me2 levels in a set of human GBM samples and cell lines as compared with non-tumoral specimens. Ectopic overexpression of KMT5B induced tumor suppressor-like features in vitro and in a mouse tumor xenograft model, as well as changes in the expression of several glioblastoma-related genes. H4K20me2 enrichment was found immediately upstream of the promoter regions of a subset of deregulated genes, thus suggesting a possible role for KMT5B in GBM through the epigenetic modulation of key target cancer genes.

PMID:34447744 | PMC:PMC8383299 | DOI:10.3389/fcell.2021.671838

Permalink for 'Ganglioside Composition Distinguishes Anaplastic Ganglioglioma Tumor Tissue from Peritumoral Brain Tissue: Complementary Mass Spectrometry and Thin-Layer Chromatography Evidence'

Ganglioside Composition Distinguishes Anaplastic Ganglioglioma Tumor Tissue from Peritumoral Brain Tissue: Complementary Mass Spectrometry and Thin-Layer Chromatography Evidence

Fetched: August 29th, 2021, 5:01am GMT by Dragana Fabris

Int J Mol Sci. 2021 Aug 17;22(16):8844. doi: 10.3390/ijms22168844.

ABSTRACT

Gangliosides serve as antitumor therapy targets and aberrations in their composition strongly correlate with tumor growth and invasiveness. Anaplastic ganglioglioma is a rare, poorly characterized, malignant neuronal-glial tumor type. We present the first comparative characterization of ganglioside composition in anaplastic ganglioglioma vs. peritumoral and healthy brain tissues by combining mass spectrometry and thin-layer chromatography. Anaplastic ganglioglioma ganglioside composition was highly distinguishable from both peritumoral and healthy tissue despite having five to six times lower total content. Ten out of twelve MS-identified ganglioside classes, defined by unique glycan residues, were represented by a large number and considerable abundance of individual species with different fatty acid residues (C16-C24) in ceramide portions. The major structurally identified class was tumor-associated GD3 (>50%) with 11 species; GD3 (d18:1/24:0) being the most abundant. The dominant sphingoid base residue in ganglioside ceramides was sphingosine (d18:1), followed by eicosasphingosine (d20:1). The peritumoral tissue ganglioside composition was estimated as normal. Specific ganglioside composition and large variability of ganglioside ceramide structures determined in anaplastic ganglioglioma demonstrate realistic ganglioside expression patterns and correspond to the profile of high-grade malignancy brain tumors.

PMID:34445547 | PMC:PMC8396361 | DOI:10.3390/ijms22168844

Engineering Amyloid Aggregation as a New Way to Eliminate Cancer Stem Cells by the Disruption of Iron Homeostasis

Fetched: August 27th, 2021, 5:01am GMT by Hongshuang Qin

Nano Lett. 2021 Sep 8;21(17):7379-7387. doi: 10.1021/acs.nanolett.1c02734. Epub 2021 Aug 26.

ABSTRACT

Cancer stem cells (CSCs) play crucial roles in tumor initiation. Amyloid β (Aβ), which is associated with Alzheimer's disease (AD), has been identified to induce cytotoxicity in tumor cells besides brain cells. Herein, we find that oligomeric Aβ1-42 and Aβ1-40 (OAβ1-42 and OAβ1-40) can repress the viability of breast CSCs. Intriguingly, OAβ1-42 and OAβ1-40 preferentially induce the growth arrest of breast CSCs by contrast with the bulk cancer cells. Further studies indicate that OAβ1-42 and OAβ1-40 disturb iron homeostasis, which results in iron accumulation in lysosomes. The iron in lysosomes then induces ROS production by Fenton reaction, leading to breast CSC death. In vivo experiments show that the tumorigenesis of breast CSCs pretreated with OAβ1-42 is inhibited. These results reveal that OAβ1-42 and OAβ1-40 are multifaceted players with the ability to eliminate CSCs. Our work may provide a new clue to better understand the biological functions of amyloid oligomers.

PMID:34436904 | DOI:10.1021/acs.nanolett.1c02734

Adult Medulloblastoma Demographic, Tumor and Treatment Impact since 2006: A Canadian University Experience

Fetched: August 27th, 2021, 5:01am GMT by Maria Camila Quinones

Curr Oncol. 2021 Aug 14;28(4):3104-3114. doi: 10.3390/curroncol28040271.

ABSTRACT

Medulloblastoma is an aggressive primary brain tumor that is extremely rare in adults; therefore, prospective studies are limited. We reviewed the information of all MB patients treated at the CHUM between 2006 and 2017. We divided our cohort by age and further divided adult patients (53%) in two groups, those diagnosed between 2006-2012 and 2013-2017. In our adult population, median follow up was 26 months and SHH-activated MB comprised 39% of tumors. Adult 5yOS was 80% and first-line therapy led to a 5yPFS of 77%. The absence of radiosensitizing chemotherapy (100% vs. 50%; p = 0.033) negatively influenced 5yPFS. 96% of adult patients received radiotherapy and 48% of them received concomitant radiosensitizing chemotherapy. Complete surgical resection was performed on 85% of adults, but the extent of resection did not have a discernable impact on survival and did not change with time. Adjuvant chemotherapy did not clearly affect prognosis (5yOS 80% vs. 67%, p = 0.155; 5yPFS 78% vs. 67%, p = 0.114). From 2006-2012, the most common chemotherapy regimen (69%) was Cisplatinum, Lomustine and Vincristine, which was replaced in 2013 by Cisplatinum, Etoposide and Cyclophosphamide (77%) with a trend for worse survival. Nine patients recurred and seven of these (78%) were treated with palliative chemotherapy. In conclusion, we did not identify prognostic demographic or tumor factors in our adult MB population. The presence of radiosensitizing chemotherapy was associated with a more favorable PFS. Cisplatinum, Lomustine and Vincristine regimen might be a better adjuvant chemotherapy regimen.

PMID:34436037 | PMC:PMC8395420 | DOI:10.3390/curroncol28040271

Stereotactic Radiotherapy for Recurrent Post-Transplant Primary Central Nervous System Lymphoma

Fetched: August 27th, 2021, 5:01am GMT by Cecil M Benitez

Cureus. 2021 Jul 21;13(7):e16537. doi: 10.7759/cureus.16537. eCollection 2021 Jul.

ABSTRACT

Post-transplant primary central nervous system lymphoma (PCNSL) is a rare complication of solid organ transplantation. The optimal therapy for post-transplant PCNSL is not well established and generally includes reduction of immunosuppression and chemotherapy. Progression after front-line chemotherapy is common, and whole-brain radiotherapy (WBRT) is a standard salvage treatment as there is a concern that localized treatment fields would not prevent out-of-field recurrences. However, WBRT is associated with neurotoxicity and morbidity in these patients with inherently poor prognoses. Here, we report a patient with local recurrence of post-transplant PCNSL who was treated with fractionated stereotactic radiotherapy (SRT). He had no clinical toxicity from treatment and maintained pre-treatment neurocognition and performance status. Local control was achieved for 20 months following SRT, at which point he developed an in-field recurrence. He restarted lymphoma therapy but died one month later from fungal pneumonia. For central nervous system (CNS) lymphoma, further data are needed to optimize tumor control and toxicity outcomes and identify patients in whom localized radiotherapy fields may be beneficial, avoiding the potential toxicity of WBRT.

PMID:34430145 | PMC:PMC8378593 | DOI:10.7759/cureus.16537

Cerebellar hemangioblastoma: Case report with review of the literature

Fetched: August 27th, 2021, 5:01am GMT by Mohamed Lahkim

Radiol Case Rep. 2021 Aug 14;16(10):3109-3112. doi: 10.1016/j.radcr.2021.07.027. eCollection 2021 Oct.

ABSTRACT

Originally recognized by Cushing and Bailey, hemangioblastoma is a developmental vascular neoplasm that is predominantly found in the posterior fossa. It is a highly vascularized tumor, with well-differentiated histologic features. Although rare, it remains the most common primary tumor of cerebellum in adults, along with metastases. MRI is the gold standard, allowing a precise characterization of the lesion's features, and its relationship with the surrounding structures. We report the case of a patient with a cerebellar symptomatology, in whom brain MRI raises the diagnosis of hemangioblastoma, which was then confirmed by histopathologic examination.

PMID:34429813 | PMC:PMC8367806 | DOI:10.1016/j.radcr.2021.07.027

Permalink for 'Intensive surveillance endoscopy for multiple gastrointestinal tumors in a patient with constitutional mismatch repair deficiency: case report'

Intensive surveillance endoscopy for multiple gastrointestinal tumors in a patient with constitutional mismatch repair deficiency: case report

Fetched: August 27th, 2021, 5:01am GMT by Takayuki Ando

BMC Gastroenterol. 2021 Aug 23;21(1):326. doi: 10.1186/s12876-021-01902-6.

ABSTRACT

BACKGROUND: Constitutional mismatch repair deficiency (CMMRD) is an extremely rare autosomal recessive hereditary disease characterized by the absence of mismatch repair gene activity from birth, which results in brain tumors, colonic polyposis, gastrointestinal cancers, and lymphomas later in life. An aggressive approach, including colectomy or proctocolectomy, is recommended for the treatment of colorectal cancer. Additionally, partial colectomy with subsequent endoscopic surveillance may be an alternative strategy due to poor patient's condition, although there is no evidence of surveillance endoscopy after partial colectomy for CMMRD.

CASE PRESENTATION: A 13-year-old male patient with a history of T-lymphoblastic lymphoma underwent total gastrointestinal endoscopy, which revealed rectal cancer, colorectal polyposis, and duodenal adenoma. Differential diagnosis included constitutional mismatch repair deficiency according to its scoring system and microsatellite instability, and subsequent germline mutation testing for mismatch repair genes confirmed the diagnosis of constitutional mismatch repair deficiency based on a homozygous mutation in mutS homolog 6 (MSH6). The patient and his family refused colectomy due to the high risk of malignancies other than colorectal cancer, which could require radical surgery. Therefore, the patient underwent low anterior resection of the rectosigmoid colon for rectal cancer and intensive surveillance endoscopy for the remaining colon polyposis. During the 3-year period after initial surgery, 130 polyps were removed and the number of polyps gradually decreased during 6-months interval surveillance endoscopies, although only one polyp was diagnosed as invasive adenocarcinoma (pT1).

CONCLUSIONS: Our experience of short surveillance endoscopy illustrates that this strategy might be one of options according to patient's condition.

PMID:34425783 | PMC:PMC8381554 | DOI:10.1186/s12876-021-01902-6

ESE Clinical Practice Guideline on functioning and nonfunctioning pituitary adenomas in pregnancy

Fetched: August 27th, 2021, 5:01am GMT by A Luger

Eur J Endocrinol. 2021 Aug 23;185(3):G1-G33. doi: 10.1530/EJE-21-0462.

ABSTRACT

Pregnancies are rare in women with pituitary adenomas, which may relate to hormone excess from secretory subtypes such as prolactinomas or corticotroph adenomas. Decreased fertility may also result from pituitary hormone deficiencies due to compression of the gland by large tumours and/or surgical or radiation treatment of the lesion. Counselling premenopausal women with pituitary adenomas about their chance of conceiving spontaneously or with assisted reproductive technology, and the optimal pre-conception treatment, should start at the time of initial diagnosis. The normal physiological changes during pregnancy need to be considered when interpreting endocrine tests in women with pituitary adenomas. Dose adjustments in hormone substitution therapies may be needed across the trimesters. When medical therapy is used for pituitary hormone excess, consideration should be given to the known efficacy and safety data specific to pregnant women for each therapeutic option. In healthy women, pituitary gland size increases during pregnancy. Since some pituitary adenomas also enlarge during pregnancy, there is a risk of visual impairment, especially in women with macroadenomas or tumours near the optic chiasm. Pituitary apoplexy represents a rare acute complication of adenomas requiring surveillance, with surgical intervention needed in some cases. This guideline describes the choice and timing of diagnostic tests and treatments from the pre-conception stage until after delivery, taking into account adenoma size, location and endocrine activity. In most cases, pregnant women with pituitary adenomas should be managed by a multidisciplinary team in a centre specialised in the treatment of such tumours.

PMID:34425558 | DOI:10.1530/EJE-21-0462

No evidence of human genome integration of SARS-CoV-2 found by long-read DNA sequencing

Fetched: August 27th, 2021, 5:01am GMT by Nathan Smits

Cell Rep. 2021 Aug 17;36(7):109530. doi: 10.1016/j.celrep.2021.109530. Epub 2021 Jul 28.

ABSTRACT

A recent study proposed that severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) hijacks the LINE-1 (L1) retrotransposition machinery to integrate into the DNA of infected cells. If confirmed, this finding could have significant clinical implications. Here, we apply deep (>50×) long-read Oxford Nanopore Technologies (ONT) sequencing to HEK293T cells infected with SARS-CoV-2 and do not find the virus integrated into the genome. By examining ONT data from separate HEK293T cultivars, we completely resolve 78 L1 insertions arising in vitro in the absence of L1 overexpression systems. ONT sequencing applied to hepatitis B virus (HBV)-positive liver cancer tissues located a single HBV insertion. These experiments demonstrate reliable resolution of retrotransposon and exogenous virus insertions by ONT sequencing. That we find no evidence of SARS-CoV-2 integration suggests that such events are, at most, extremely rare in vivo and therefore are unlikely to drive oncogenesis or explain post-recovery detection of the virus.

PMID:34380018 | PMC:PMC8316065 | DOI:10.1016/j.celrep.2021.109530

Unsupervised machine learning reveals key immune cell subsets in COVID-19, rhinovirus infection, and cancer therapy

Fetched: August 27th, 2021, 5:01am GMT by Sierra M Barone

Elife. 2021 Aug 5;10:e64653. doi: 10.7554/eLife.64653.

ABSTRACT

For an emerging disease like COVID-19, systems immunology tools may quickly identify and quantitatively characterize cells associated with disease progression or clinical response. With repeated sampling, immune monitoring creates a real-time portrait of the cells reacting to a novel virus before disease-specific knowledge and tools are established. However, single cell analysis tools can struggle to reveal rare cells that are under 0.1% of the population. Here, the machine learning workflow Tracking Responders EXpanding (T-REX) was created to identify changes in both rare and common cells across human immune monitoring settings. T-REX identified cells with highly similar phenotypes that localized to hotspots of significant change during rhinovirus and SARS-CoV-2 infections. Specialized MHCII tetramer reagents that mark rhinovirus-specific CD4+ cells were left out during analysis and then used to test whether T-REX identified biologically significant cells. T-REX identified rhinovirus-specific CD4+ T cells based on phenotypically homogeneous cells expanding by ≥95% following infection. T-REX successfully identified hotspots of virus-specific T cells by comparing infection (day 7) to either pre-infection (day 0) or post-infection (day 28) samples. Plotting the direction and degree of change for each individual donor provided a useful summary view and revealed patterns of immune system behavior across immune monitoring settings. For example, the magnitude and direction of change in some COVID-19 patients was comparable to blast crisis acute myeloid leukemia patients undergoing a complete response to chemotherapy. Other COVID-19 patients instead displayed an immune trajectory like that seen in rhinovirus infection or checkpoint inhibitor therapy for melanoma. The T-REX algorithm thus rapidly identifies and characterizes mechanistically significant cells and places emerging diseases into a systems immunology context for comparison to well-studied immune changes.

PMID:34350827 | PMC:PMC8370768 | DOI:10.7554/eLife.64653

De-differentiated metastatic adenosquamous carcinoma arising from germ cell tumor in the brain and spine

Fetched: August 25th, 2021, 5:01am GMT by Brian Fiani

Acta Neurochir (Wien). 2021 Aug 23. doi: 10.1007/s00701-021-04973-2. Online ahead of print.

ABSTRACT

Intracranial germ cell tumors have an estimated incidence of 0.4-3.4% in the Western Hemisphere. Patients can present with a variety of differing clinical signs and symptoms including headache, nausea/vomiting, hydrocephalus, obtundation, pyramidal tract signs, ataxia, and hypothalamic/pituitary dysfunction. Rarely germ cell tumors can transform into alternative malignancy. In these cases, treatment options may be difficult. Metastasis to the brain is not uncommon in germ cell tumors and is frequently reported within the pineal region; however, they are less common intraventricularly, within the posterior fossa and have never been reported after malignant transformation. Herein, we present the first reported case of a metastatic adenosquamous carcinoma transformed from a yolk sac tumor with diffuse cerebral metastasis in atypical locations of the brain including intraventricular and posterior fossa. A 53-year-old right-handed Caucasian female was transferred from an outside hospital for a chief complaint of altered mental status with CT head showing right side intraventricular mass and cerebellar hemorrhage. MRI of the brain found multifocal contrast-enhancing lesions of the right lateral ventricle, right cerebellum, right frontal lobe, diffuse lumbar dural enhancement, and an intramedullary lesion at the cervico-medullary junction of the brainstem. The right lateral ventricular lesion and right cerebellar lesions were resected. Pathology findings support a diagnosis of adenosquamous carcinoma, and the morphologic and immunophenotypic features suggest development as a somatic malignancy in a germ cell neoplasm with features of a yolk sac tumor. Germ cell tumors are typically included within the differential of pineal region masses; however, other locations such as intraventricular and posterior fossa are rarely seen. Even rarer are cases with malignant transformation to an alternative lesion for which treatment options are exceptionally scarce. Neurosurgeons and oncologists alike should be aware of this rare possible lesion to add to a broad differential diagnosis.

PMID:34424392 | DOI:10.1007/s00701-021-04973-2

Disseminated nocardiosis caused by Nocardia farcinica in a patient with colon cancer: A case report and literature review

Fetched: August 25th, 2021, 5:01am GMT by Eung Kyum Lee

Medicine (Baltimore). 2021 Jul 23;100(29):e26682. doi: 10.1097/MD.0000000000026682.

ABSTRACT

RATIONALE: Nocardiosis is an uncommon and potentially life-threatening infection that usually affects immunocompromised hosts. No clinical guidelines have been established for managing this rare disease, and the optimal treatment modality remains unclear. Nocardia farcinica, a relatively infrequent pathogen of nocardiosis, causes a clinically aggressive infection. In addition to our patient data, our search of the literature for patients who presented with empyema caused by N. farcinica will provide fundamental information for optimal treatment modalities.

PATIENT CONCERNS: A 64-year-old man was diagnosed with empyema, 4 days following surgery for sigmoid colon cancer. Brain lesions were evaluated only after N. farcinica was isolated and identified as the causative pathogen through repeated culture tests.

DIAGNOSES: N. farcinica was isolated from the pleural effusion and confirmed as the pathogen through 16S rRNA sequencing.

INTERVENTIONS: The patient was successfully treated with tube thoracotomy, neurosurgical evacuation, and a combination of trimethoprim/sulfamethoxazole plus imipenem. Long-term antibiotic therapy was required to prevent recurrence.

OUTCOMES: Pyothorax showed a good clinical response to antimicrobial therapy and drainage of pleural effusion, whereas brain abscess did not respond to medical therapy and required surgery. The patient eventually recovered and continued chemotherapy as treatment for sigmoid colon cancer.

LESSONS: Although extremely rare, this report demonstrates the importance of considering Nocardia infection as the differential diagnosis in immunocompromised patients who present with empyema. In particular, because of the N. farcinica infection's tendency to spread and the resistance of the organism to antibiotics, aggressive evaluation of metastatic lesions and standardized support from microbiological laboratories are important. Surgery may be required in some patients with brain abscesses to improve the chance of survival.

PMID:34398037 | PMC:PMC8294930 | DOI:10.1097/MD.0000000000026682

Small-cell lung cancer transformation from EGFR-mutant adenocarcinoma after EGFR-TKIs resistance: A case report

Fetched: August 25th, 2021, 5:01am GMT by Yiqian Jiang

Medicine (Baltimore). 2021 Aug 13;100(32):e26911. doi: 10.1097/MD.0000000000026911.

ABSTRACT

RATIONALE: With the recent advancements in molecular biology research, epidermal growth factor receptor-tyrosine kinase inhibitors (EGFR-TKIs) have emerged as excellent therapies for patients with EGFR-mutant cancers. However, these patients inevitably develop cross-acquired resistance to EGFR-TKIs. Transformation to small-cell lung cancer (SCLC) is considered a rare resistance mechanism against EGFR-TKI therapy. Here, we report a case of TKI resistance due to SCLC transformation and demonstrate its mechanisms and clinical features.

PATIENT CONCERNS: A 54-year-old Chinese man with a history of smoking for 40 years complained of an intermittent cough in March 2019.

DIAGNOSIS: Transbronchial lung biopsy was performed on the basal segment of the left lower lobe, which confirmed lung adenocarcinoma. In January 2020, repeat biopsy was performed, and the results of immunohistochemistry (IHC) staining showed TTF-1 (+), CK7 (+), napsin A (+), syn (+), and CD56 (+), with a Ki-67 (+) index 80% of small cell carcinomas. Infiltrating adenocarcinomas and small cell carcinomas were observed.

INTERVENTIONS: Icotinib (125 mg thrice daily) was administered as a first-line treatment from June 2019. We subsequently administered a chemotherapy regimen consisting of etoposide (180 mg, days 1-3) plus cisplatin (45 mg, days 1-3) every 3 weeks for 1 cycle after recurrence. As the patient could not tolerate further chemotherapy, he continued taking icotinib orally and received whole-brain radiotherapy 10 times to a total dose of 30 Gy after brain metastases.

OUTCOMES: The patient relapsed after successful treatment with icotinib for 9 months. A partial response was achieved after 4 cycles of chemotherapy, and despite the brief success of chemotherapy, our patient exhibited brain metastasis and metastases of the eleventh thoracic spine and the second lumbar vertebra with pathological fracture. The patient eventually died of aggressive cancer progression.

LESSONS: Our case highlights the possibility of SCLC transformation from EGFR-mutant adenocarcinoma and the importance of repeat biopsy for drug resistance. Serum neuron-specific enolase levels may also be useful for detecting early SCLC transformation.

PMID:34397927 | PMC:PMC8360407 | DOI:10.1097/MD.0000000000026911

PATZ1 fusions define a novel molecularly distinct neuroepithelial tumor entity with a broad histological spectrum

Fetched: August 22nd, 2021, 5:01am GMT by Karam T Alhalabi

Acta Neuropathol. 2021 Aug 21. doi: 10.1007/s00401-021-02354-8. Online ahead of print.

ABSTRACT

Large-scale molecular profiling studies in recent years have shown that central nervous system (CNS) tumors display a much greater heterogeneity in terms of molecularly distinct entities, cellular origins and genetic drivers than anticipated from histological assessment. DNA methylation profiling has emerged as a useful tool for robust tumor classification, providing new insights into these heterogeneous molecular classes. This is particularly true for rare CNS tumors with a broad morphological spectrum, which are not possible to assign as separate entities based on histological similarity alone. Here, we describe a molecularly distinct subset of predominantly pediatric CNS neoplasms (n = 60) that harbor PATZ1 fusions. The original histological diagnoses of these tumors covered a wide spectrum of tumor types and malignancy grades. While the single most common diagnosis was glioblastoma (GBM), clinical data of the PATZ1-fused tumors showed a better prognosis than typical GBM, despite frequent relapses. RNA sequencing revealed recurrent MN1:PATZ1 or EWSR1:PATZ1 fusions related to (often extensive) copy number variations on chromosome 22, where PATZ1 and the two fusion partners are located. These fusions have individually been reported in a number of glial/glioneuronal tumors, as well as extracranial sarcomas. We show here that they are more common than previously acknowledged, and together define a biologically distinct CNS tumor type with high expression of neural development markers such as PAX2, GATA2 and IGF2. Drug screening performed on the MN1:PATZ1 fusion-bearing KS-1 brain tumor cell line revealed preliminary candidates for further study. In summary, PATZ1 fusions define a molecular class of histologically polyphenotypic neuroepithelial tumors, which show an intermediate prognosis under current treatment regimens.

PMID:34417833 | DOI:10.1007/s00401-021-02354-8

Rare intracranial EWSR1-rearranged myxoid mesenchymal tumour in a teenager

Fetched: August 22nd, 2021, 5:01am GMT by Jason W Adams

BMJ Case Rep. 2021 Aug 10;14(8):e245282. doi: 10.1136/bcr-2021-245282.

NO ABSTRACT

PMID:34376425 | PMC:PMC8356179 | DOI:10.1136/bcr-2021-245282

Germ Cell Seminoma

Fetched: August 22nd, 2021, 5:01am GMT by Qandeel Sadiq

2021 Aug 11. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2021 Jan–.

ABSTRACT

Germ cell tumors (GCT), derived from primordial germ cells, are a diverse group of neoplasms that arise in the gonads (testicles and ovaries) primarily and may also arise in the anterior mediastinum, pineal gland, and brain. They are clinically classified into two major categories, with significant clinical and prognostic implications: Seminomas and non-seminomas. Seminomas generally have a good prognosis, while non-seminomatous tumors are much more likely to present with metastatic disease as well as mixed germ cell tumors comprising of two or more than two different germ cell tumor components. Although rare, GCT may also arise from extragonadal sites. Term “extragonadal germ cell tumor” (EGGCT) is implied in the absence of any primary gonadal neoplasm and represent 1% to 3% of all GCT. Seminoma constitutes 60% of all primary extragonadal GCT and is seen most notably in midline locations of the body like anterior mediastinum, central nervous system, and retroperitoneum. Seminoma in the retroperitoneum is generally metastatic disease from primary gonadal seminoma unless proven otherwise. In the central nervous system, the pineal gland and suprasellar regions are involved more frequently. Germinoma, an intracranial counterpart of seminoma (extragonadal seminoma), comprises approximately two-third of all intracranial GCT.

Germinoma can be pure or mixed (coexisting with other GCT, classified as non-germinomatous GCT). Non-germinomatous GCT makes up the remaining one third, including embryonal carcinoma, yolk sac tumor, choriocarcinomas, teratomas, and mixed tumors (containing more than one histologic element). Less commonly involved sites include paraventricular regions of basal ganglia and thalamus. Very rarely, synchronous involvement of the pineal and the suprasellar region is observed, called bifocal germinoma. Germinoma is more common in young adolescents and is usually diagnosed in the second decade of life. The prognosis is good, with a 5-year survival rate greater than 90% since the tumor is highly radiosensitive.

PMID:32644667 | Bookshelf:NBK559241

Somatotroph Tumors and the Epigenetic Status of the GNAS Locus

Fetched: August 20th, 2021, 5:01am GMT by Pauline Romanet

Int J Mol Sci. 2021 Jul 15;22(14):7570. doi: 10.3390/ijms22147570.

ABSTRACT

Forty percent of somatotroph tumors harbor recurrent activating GNAS mutations, historically called the gsp oncogene. In gsp-negative somatotroph tumors, GNAS expression itself is highly variable; those with GNAS overexpression most resemble phenotypically those carrying the gsp oncogene. GNAS is monoallelically expressed in the normal pituitary due to methylation-based imprinting. We hypothesize that changes in GNAS imprinting of gsp-negative tumors affect GNAS expression levels and tumorigenesis. We characterized the GNAS locus in two independent somatotroph tumor cohorts: one of 23 tumors previously published (PMID: 31883967) and classified by pan-genomic analysis, and a second with 82 tumors. Multi-omics analysis of the first cohort identified a significant difference between gsp-negative and gsp-positive tumors in the methylation index at the known differentially methylated region (DMR) of the GNAS A/B transcript promoter, which was confirmed in the larger series of 82 tumors. GNAS allelic expression was analyzed using a polymorphic Fok1 cleavage site in 32 heterozygous gsp-negative tumors. GNAS expression was significantly reduced in the 14 tumors with relaxed GNAS imprinting and biallelic expression, compared to 18 tumors with monoallelic expression. Tumors with relaxed GNAS imprinting showed significantly lower SSTR2 and AIP expression levels. Altered A/B DMR methylation was found exclusively in gsp-negative somatotroph tumors. 43% of gsp-negative tumors showed GNAS imprinting relaxation, which correlated with lower GNAS, SSTR2 and AIP expression, indicating lower sensitivity to somatostatin analogues and potentially aggressive behavior.

PMID:34299200 | PMC:PMC8306130 | DOI:10.3390/ijms22147570

Mature cystic teratoma of the right cerebellopontine angle: a rare case report

Fetched: August 20th, 2021, 5:01am GMT by Reshma Sattar

Br J Neurosurg. 2021 Aug 19:1-4. doi: 10.1080/02688697.2021.1967287. Online ahead of print.

ABSTRACT

BACKGROUND AND IMPORTANCE: Intracranial mature cystic teratomas are benign neoplasms that commonly occur at the midline. Mature cystic teratomas at the cerebellopontine (CP) angle are very rare. They are unique germ cell tumours curable by safe total surgical resection and have good prognosis. This case report documents the clinical, radiological, histological features and operative findings of mature cystic teratoma at CP angle.

CLINICAL PRESENTATION: We present a rare case of a mature cystic teratoma at the CP angle in a 24-year-old woman who presented with brainstem compression and cranial nerve deficits. Brain MRI showed atypical findings like hyperintense areas in both T1 and T2 weighted images, calcification and diffusion restriction in part of the lesion. She underwent near total resection of the tumour via right retrosigmoid approach. Intraoperatively, the lesion was intra-arachnoidal unlike schwannomas and the cyst contained sebum-like material, fibrous areas with calcification which are unusual features of common CP angle tumours. Histopathological examination showed well differentiated mature tissues from all three germinal layers and confirmed the diagnosis of a mature cystic teratoma arising from the right CP angle. Patient had good outcome with neurologic recovery.

CONCLUSIONS: Mature cystic teratoma is a rare clinical entity and should be considered in patients with CP angle tumours when there are atypical findings in brain MRI imaging. Cysts with sebum-like material, fibrous areas with calcification and poor tumour-arachnoid plane intraoperatively strongly suggest the possibility of mature cystic teratoma.

PMID:34410217 | DOI:10.1080/02688697.2021.1967287

Subgroup and subtype-specific outcomes in adult medulloblastoma

Fetched: August 20th, 2021, 5:01am GMT by Hallie Coltin

Acta Neuropathol. 2021 Aug 18. doi: 10.1007/s00401-021-02358-4. Online ahead of print.

ABSTRACT

Medulloblastoma, a common pediatric malignant central nervous system tumour, represent a small proportion of brain tumours in adults. Previously it has been shown that in adults, Sonic Hedgehog (SHH)-activated tumours predominate, with Wingless-type (WNT) and Group 4 being less common, but molecular risk stratification remains a challenge. We performed an integrated analysis consisting of genome-wide methylation profiling, copy number profiling, somatic nucleotide variants and correlation of clinical variables across a cohort of 191 adult medulloblastoma cases identified through the Medulloblastoma Advanced Genomics International Consortium. We identified 30 WNT, 112 SHH, 6 Group 3, and 41 Group 4 tumours. Patients with SHH tumours were significantly older at diagnosis compared to other subgroups (p < 0.0001). Five-year progression-free survival (PFS) for WNT, SHH, Group 3, and Group 4 tumours was 64.4 (48.0-86.5), 61.9% (51.6-74.2), 80.0% (95% CI 51.6-100.0), and 44.9% (95% CI 28.6-70.7), respectively (p = 0.06). None of the clinical variables (age, sex, metastatic status, extent of resection, chemotherapy, radiotherapy) were associated with subgroup-specific PFS. Survival among patients with SHH tumours was significantly worse for cases with chromosome 3p loss (HR 2.9, 95% CI 1.1-7.6; p = 0.02), chromosome 10q loss (HR 4.6, 95% CI 2.3-9.4; p < 0.0001), chromosome 17p loss (HR 2.3, 95% CI 1.1-4.8; p = 0.02), and PTCH1 mutations (HR 2.6, 95% CI 1.1-6.2; p = 0.04). The prognostic significance of 3p loss and 10q loss persisted in multivariable regression models. For Group 4 tumours, chromosome 8 loss was strongly associated with improved survival, which was validated in a non-overlapping cohort (combined cohort HR 0.2, 95% CI 0.1-0.7; p = 0.007). Unlike in pediatric medulloblastoma, whole chromosome 11 loss in Group 4 and chromosome 14q loss in SHH was not associated with improved survival, where MYCN, GLI2 and MYC amplification were rare. In sum, we report unique subgroup-specific cytogenetic features of adult medulloblastoma, which are distinct from those in younger patients, and correlate with survival disparities. Our findings suggest that clinical trials that incorporate new strategies tailored to high-risk adult medulloblastoma patients are urgently needed.

PMID:34409497 | DOI:10.1007/s00401-021-02358-4

Central nervous system metastases: a rare presentation of nasopharyngeal carcinoma

Fetched: August 20th, 2021, 5:01am GMT by Sehrish Abrar

BMJ Case Rep. 2021 Aug 17;14(8):e243892. doi: 10.1136/bcr-2021-243892.

ABSTRACT

We report a case of a male patient. He presented with nasal obstruction and epistaxis. The MRI of the brain showed a mass in the nasopharynx and enlarged cervical lymph nodes. Besides this, there was an extra-axial, dural-based lesion in brain and subcentimetre nodules in both lungs. He received induction chemotherapy followed by chemoradiation therapy to the primary site and dural-based metastatic deposit. He re-presented with bilateral lower limb weakness. The MRI of the spine showed metastatic deposits within the thoracic cord parenchyma and meningeal deposits at the lumbar region. Palliative radiation was used to treat spinal cord and meningeal metastases. He died a few months later because of systemic disease progression. Considering the rarity of this presentation in nasopharyngeal carcinoma and lack of clear guidelines for standard treatment, we report this case to share our experience of management.

PMID:34404660 | PMC:PMC8372796 | DOI:10.1136/bcr-2021-243892

Long-term extracranial metastatic relapse of an intraventricular solitary fibrous tumor: a case report

Fetched: August 19th, 2021, 5:01am GMT by Tarek Assi

Anticancer Drugs. 2021 Aug 16. doi: 10.1097/CAD.0000000000001182. Online ahead of print.

ABSTRACT

Solitary fibrous tumors (SFTs), an uncommon subtype of soft tissue sarcomas mesenchymal, are frequently diagnosed in the intra-thoracic region. Distant metastasis is detected in 10-40% of SFT patients with a predilection site to the lungs; nevertheless, these can occur rarely in the liver, brain, and bones. This entity was also reported in the abdomen, trunk, head, and neck, extremities but also, in the central nervous system (CNS). In the latter form of SFTs, the meninges were mostly affected while only a few cases were reported in the intraventricular and intraparenchymal region with distinct clinical and behavioral characteristics. In this article, we report the rare case of a relapsing intraventricular SFT with secondary extra-cranial metastases to the bones and soft tissues after 11 years of initial diagnosis, with focus on the available data in the literature on CNS SFTs.

PMID:34407040 | DOI:10.1097/CAD.0000000000001182

Adult giant cerebellar cavernous malformations: case report and review of the literature

Fetched: August 19th, 2021, 5:01am GMT by Rodrigo Inácio Pongeluppi

Br J Neurosurg. 2021 Aug 18:1-6. doi: 10.1080/02688697.2021.1961685. Online ahead of print.

ABSTRACT

Cavernous malformations are vascular malformations that can occur anywhere in the central nervous system (CNS). Giant cavernous malformations (GCM) are extremely rare in adults, especially in the posterior fossa. Herein, we described a 48-year-old male who presented with vertigo and postural instability for three months. Neuroimaging revealed a 131.15 cm³ heterogeneous midline upper cerebellar lesion. After a suboccipital craniotomy, a gross total resection (GTR) was accomplished. Histopathologic examination revealed a huge cavernous malformation. Only 27 GCM adult cases were reported in the English-based literature. Only two patients had cerebellar lesions and, to the best of our knowledge, this is the first case of cerebellar vermis GCM. We concluded that cerebellar GCM (CGCM) in adults are exceedingly rare and indolent lesions. These lesions can radiologically and clinically mimic neoplastic lesions that have to be considered in the differential diagnosis. GTR is the mainstay of treatment and, whenever possible, should be attempted.

PMID:34406083 | DOI:10.1080/02688697.2021.1961685

Primary diffuse large B-cell lymphoma of the cranial vault with Trousseau syndrome: a case report

Fetched: August 19th, 2021, 5:01am GMT by Tatsuya Uchida

J Med Case Rep. 2021 Aug 18;15(1):431. doi: 10.1186/s13256-021-02979-z.

ABSTRACT

BACKGROUND: It is extremely rare for primary non-Hodgkin's lymphomas to occur singly in the cranial vault. One case diagnosed as primary diffuse large B-cell lymphoma is reported, initially misdiagnosed as metastatic skull tumor, complicated with Trousseau syndrome.

CASE DESCRIPTION: The patient was a 60-year-old Japanese woman with no particular previous medical history. In a head computed tomography examination for vertigo, bone destructive skull tumor covering the right frontal, parietal, and temporal bones was incidentally discovered. As positron emission tomography indicated an abnormal accumulation in the large intestine and multiple cerebral infarctions suspicious of Trousseau syndrome were observed on magnetic resonance images, a metastatic skull tumor due to colorectal cancer was first considered. However, various tumor markers were negative, and colonoscopic biopsy indicated no colorectal abnormality. After pathological examination of the resected tumor, it was diagnosed as diffuse large B-cell lymphoma. The tumor affected muscles and skin but did not develop in the brain or the dura mater. As further general examination revealed no other abnormalities, we considered that it was primary diffuse large B-cell lymphoma in the cranial vault associated with Trousseau syndrome. Treatment with rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisolone and high-dose methotrexate reduced the residual lesion; coagulation abnormalities, which are frequently associated with Trousseau syndrome, also improved.

CONCLUSIONS: Skull tumors can result from a variety of malignancies, and their diagnosis may be complicated with Trousseau syndrome. However, even in cases of a single lesion in the cranial vault without invasion of the central nervous system, diffuse large B-cell lymphoma should be considered as a differential diagnosis.

PMID:34404459 | PMC:PMC8371799 | DOI:10.1186/s13256-021-02979-z

Multiple brain abscesses presented with monoparesis in a patient with lung abscess mimicking lung cancer

Fetched: August 18th, 2021, 5:01am GMT by Yoon-Hee Choo

Radiol Case Rep. 2021 Aug 7;16(10):3007-3011. doi: 10.1016/j.radcr.2021.07.025. eCollection 2021 Oct.

ABSTRACT

Multiple brain abscesses associated with the lung mass require differential diagnosis from cancerous diseases. Here, we report a rare case of multiple brain abscesses originating from a lung abscess. A 65-year-old man presented with a 2-day history of motor weakness during antibiotic treatment for pneumonia. Brain magnetic resonance imaging (MRI) revealed multiple enhanced lesions of various sizes in the whole brain. Diffusion-weighted MRI showed high signal intensity in several lesions, and magnetic resonance (MR) spectroscopy showed reduced N-acetyl-aspartate (NAA) and high lactate-lipid complex levels. Positron emission tomography/computed tomography revealed a hypermetabolic mass-like lesion (size: 5 × 3 cm) in the right lower lobe. The patient was diagnosed with organizing pneumonia by bronchoscopy and was successfully treated with empirical antibiotics for multiple brain abscesses and lung abscesses. If new neurological deficits occur during the treatment of inflammatory diseases such as pneumonia, the possibility of brain abscesses and cancerous conditions should be considered. Appropriate diagnosis and antibiotic treatment should be performed to ensure favorable outcomes.

PMID:34401043 | PMC:PMC8358127 | DOI:10.1016/j.radcr.2021.07.025

Cystic meningioma: A case report with a literature review

Fetched: August 18th, 2021, 5:01am GMT by Mohamed Lahkim

Radiol Case Rep. 2021 Aug 4;16(10):2958-2961. doi: 10.1016/j.radcr.2021.07.016. eCollection 2021 Oct.

ABSTRACT

Cystic meningiomas are very rare tumors of the central nervous system. We report the case of a 62-year-old female how presented headaches resistant to usual analgesics with behavior disorders. Cerebral CT scan showed a right frontal extra-axial tumor with firm and cystic component, brain MRI evoked the diagnosis, surgery removed the entire tumor and histological examination confirmed it. A cystic meningioma should not be omitted from the differential diagnosis of brain tumors with a cystic component and which clinical, radiological, histological and therapeutic features are discussed.

PMID:34401033 | PMC:PMC8350377 | DOI:10.1016/j.radcr.2021.07.016

A case report of congenital temporal bone encephalocele presenting as a tumor in a child

Fetched: August 18th, 2021, 5:01am GMT by Doan-Van Ngoc

Radiol Case Rep. 2021 Aug 3;16(10):2945-2948. doi: 10.1016/j.radcr.2021.06.064. eCollection 2021 Oct.

ABSTRACT

An encephalocele may be congenital or acquired and is characterized by the herniation of cranial contents through a skull bone defect. Most congenital encephaloceles occur in the occipital area, and temporal bone encephaloceles in children are rare. Congenital encephaloceles can be diagnosed either prenatally or after birth. We describe the case of a congenital temporal bone encephalocele in a 2-month-old boy that was diagnosed after birth. The patient presented with seizures and a bulging mass in the right neck that was detected by his mother during the second month after birth. The combined results from brain magnetic resonance imaging, computed tomography, and histological analysis confirmed the diagnosis of encephalocele. Although the surgical repair was offered, the family declined.

PMID:34401031 | PMC:PMC8350188 | DOI:10.1016/j.radcr.2021.06.064

Permalink for '18F-FDG PET/CT Findings in a Rare Case of Paraneoplastic Vestibulocerebellar Syndrome Associated With Isolated Antiamphiphysin Antibodies'

18F-FDG PET/CT Findings in a Rare Case of Paraneoplastic Vestibulocerebellar Syndrome Associated With Isolated Antiamphiphysin Antibodies

Fetched: August 16th, 2021, 5:01am GMT by Shivaram Rao Komandla

Clin Nucl Med. 2021 Aug 5. doi: 10.1097/RLU.0000000000003868. Online ahead of print.

ABSTRACT

Paraneoplastic cerebellar degeneration (PCD) is an immune-mediated neurological disease characterized by adaptive immune response against onconeural antigens physiologically expressed in the cerebellum. It is characterized by presence of highly specific onconeural autoantibodies such as anti-Yo, anti-Hu, anti-Ri, and anti-Ma2 in the serum and cerebrospinal fluid as diagnostic biomarkers. Antiamphiphysin autoantibody-related paraneoplastic encephalitis is a less commonly seen autoimmune neurological disorder usually presenting as stiff person syndrome. We present an unusual case of isolated antiamphiphysin antibody-related PCD presenting as vestibulocerebellar syndrome with associated sensorineural hearing loss and sensory neuropathy. FDG PET helped in topographical localization of brain lesion along with early detection of extragonadal germ cell tumor in the retroperitoneum.

PMID:34392286 | DOI:10.1097/RLU.0000000000003868

Intracranial perianeurysmal cysts: case series and review of the literature

Fetched: August 13th, 2021, 5:01am GMT by Ee Shern Liang

J Neurointerv Surg. 2021 Aug 11:neurintsurg-2021-017807. doi: 10.1136/neurintsurg-2021-017807. Online ahead of print.

ABSTRACT

BACKGROUND: Intracranial perianeurysmal cysts are a rare finding associated with cerebral aneurysms. Patients may present with symptoms secondary to mass effect from perianeurysmal cysts requiring drainage. These lesions can masquerade as neoplasms if dedicated vascular imaging is not performed, leading to misdiagnosis.

METHOD: A retrospective search of our database was done for intracranial aneurysms that have been treated between 1998 and 2020. A literature search was then performed on PubMed and Google Scholar with the search terms 'aneurysm', 'intracranial/intracerebral', 'cyst', and 'perianeurysmal cyst'. Patient demographics, aneurysms and cysts characteristics were then summarized as a table and in the discussion.

RESULTS: Three cases where intracranial aneurysm had associated perianeurysmal cysts were found in our database. Combined with the available literature a total of 19 cases of perianeurysmal cysts have thus far been reported since this entity was first described in 2002. A significant number of perianeurysmal cysts (5/19) required intervention. In 5/19 cases the patient presented with a perianeurysmal cyst without a history of subarachnoid hemorrhage. Of the 10 cases where aneurysm follow-up was reported there were 5 cases where there was aneurysm recurrence necessitating re-treatment.

CONCLUSION: Significant variability exists in the patient demographics, aneurysm and cyst characteristics of perianeurysmal cysts. This suggests that there is no single unified etiology and pathogenesis. These lesions are a rare finding and at present do not appear to carry diagnostic or prognostic significance. Management of perianeurysmal cysts is case-dependent and intervention should be considered when treating the related aneurysm, especially in patients with secondary symptoms.

PMID:34380710 | DOI:10.1136/neurintsurg-2021-017807

Permalink for 'Clinical characterization of adult medulloblastoma and the effect of first-line therapies on outcome; The MD Anderson Cancer Center experience'

Clinical characterization of adult medulloblastoma and the effect of first-line therapies on outcome; The MD Anderson Cancer Center experience

Fetched: August 12th, 2021, 5:01am GMT by Nazanin K Majd

Neurooncol Adv. 2021 Jun 22;3(1):vdab079. doi: 10.1093/noajnl/vdab079. eCollection 2021 Jan-Dec.

ABSTRACT

BACKGROUND: Adult medulloblastoma (MB) is rare, and management guidelines are largely based on pediatric clinical trials and retrospective series. Limited data exist with respect to clinical characteristics, prognostic factors, and outcomes based on first-line treatments.

METHODS: Two hundred adults with MB seen at a single institution from January 1978 to April 2017 were identified and followed for a median of 8.4 y (7.1, 10.3).

RESULTS: Patient's median age at diagnosis was 29 y (18, 63). One hundred eleven (55.5%) were standard-risk, 59 (29.5%) were high-risk, and 30 (15.0%) were indeterminate. Most received post-operative radiation (RT) (184 [92.0%]), and 105 (52.5%) received first-line chemotherapy. Median overall survival (OS) was 8.8 y (7.2, 12.2) and median progression-free survival (PFS) was 6.6 y (4.9, 11.2). High-risk patients had inferior OS (Hazard ratio [HR] = 2.5 [1.5, 4.2], P = .0006) and PFS (HR = 2.3 [1.3, 3.9], P = .002) compared to standard-risk patients. Age, sex, and metastatic disease were not associated with survival. After adjusting for risk status, those who received RT plus adjuvant chemotherapy had superior PFS compared to RT plus neoadjuvant chemotherapy [HR = 0.46 (0.22, 0.95), P = .0357]. Within a subgroup for whom detailed clinical data were available, those who received RT plus adjuvant chemotherapy had improved PFS compared to RT only [HR = 0.24 (0.074-0.76), P = .016]. The substitution of cisplatin for carboplatin and the elimination of vincristine did not negatively affect outcomes.

CONCLUSION: This is the largest single-institution retrospective study of adult MB to our knowledge and identifies standard-risk status, first-line RT and adjuvant chemotherapy as factors associated with improved outcomes.

PMID:34377987 | PMC:PMC8350154 | DOI:10.1093/noajnl/vdab079

Permalink for 'Intracranial Solitary Fibrous Tumor/Hemangiopericytoma: A Clinicoradiological Poorly Recognized Entity- An Institutional Experience'

Intracranial Solitary Fibrous Tumor/Hemangiopericytoma: A Clinicoradiological Poorly Recognized Entity- An Institutional Experience

Fetched: August 11th, 2021, 5:01am GMT by Nibedita Sahoo

Turk Neurosurg. 2020 Nov 19. doi: 10.5137/1019-5149.JTN.31204-20.2. Online ahead of print.

ABSTRACT

AIM: Intracranial solitary fibrous tumors (SFTs) and hemangiopericytomas (HPCs) are rare nonmeningothelial mesenchymal tumors sharing fusion of NGF1-A binding protein 2 (NAB2) and signal transducers and activators of transcription (STAT6.).The WHO classification of central nervous system (CNS) tumors (2016) highlights that molecular confirmation of NAB2/STAT6 fusion or immunohistochemical nuclear expression of STAT6 is mandatory for the diagnosis of SFT/HPC.Herein, we present a series of four cases of SFT/HPC of the brain, which mimicked other CNS tumors both clinically and radiologically.

MATERIAL AND METHODS: This is a retrospective study over a period of two and a half years. Out of the 156 operated cases of brain tumors, four patients (2.56%) were diagnosed with SFT/HPC. The clinicoradiological details with the surgical procedure were retrieved from the archived hospital records.

RESULTS: All cases were males, of which three were in their 5th decade while one was a 14-month-old baby. Two cases were primary and the rest were recurrent.The location of tumors was extra-axial left cerebellotentorial,clivaldural-based,left cerebellar, and in the left frontoparietal region,respectively. The clinical impression was meningioma in three cases,while it was primitive neuroectodermal tumor (PNET) /atypical teratoid/rhabdoid tumor (ATRT) in one case.With the detailed histomorphology and immunohistochemistry, the final diagnosis was anaplastic hemangiopericytoma(WHO grade III) for all the cases. During our follow-up, one patient died with the disease, while the rest are doing well.

CONCLUSION: SFT/HPC should be kept in the differential diagnosis of all dura-based hypervascular masses, especially in recurrent cases, due to its aggressiveness and high recurrence rate.

PMID:34374969 | DOI:10.5137/1019-5149.JTN.31204-20.2

Leptomeningeal Carcinomatosis Secondary to a Gastrointestinal Stromal Tumor

Fetched: August 10th, 2021, 5:01am GMT by Rachel Wlock

Cureus. 2021 Jul 6;13(7):e16212. doi: 10.7759/cureus.16212. eCollection 2021 Jul.

ABSTRACT

A 66-year-old male presented to the emergency department (ED) with altered mental status and concern for stroke. Seven months prior to presentation, the patient was diagnosed with a benign gastrointestinal stromal tumor (GIST), for which he did not receive further workup. Initially, there was a concern for a stroke, however, CT of the brain, CT angiography of the head and neck, and MRI of the brain were negative for any acute abnormalities. Lumbar puncture (LP) revealed a low glucose level with increased protein and white blood cells in the cerebrospinal fluid (CSF). The patient did not display the typical signs and symptoms of meningitis, however, inpatient antibiotic treatment was initiated. The patient continued to deteriorate and repeat LP with cytology was ordered, which revealed malignant cells that were similar to the biopsy of the GIST; our patient was diagnosed with leptomeningeal carcinomatosis (LMC) secondary to the GIST. This case report presents a rare complication of a solid GIST and highlights the need for a high clinical index of suspicion for LMC, including those previously deemed to be benign.

PMID:34367813 | PMC:PMC8341026 | DOI:10.7759/cureus.16212

Primary Intramedullary Spinal Melanoma: A Rare Disease of the Spinal Cord

Fetched: August 10th, 2021, 5:01am GMT by Fatima Tuz Zahra

Cureus. 2021 Jul 5;13(7):e16194. doi: 10.7759/cureus.16194. eCollection 2021 Jul.

ABSTRACT

Primary malignant melanoma of the intramedullary region of the spinal cord has rarely been reported in the literature. These tumors can have variable appearance on magnetic resonance imaging (MRI) due to different extents of melanin and hemorrhage. Histopathologic confirmation and a comprehensive workup to rule out extra-spinal melanoma are required to make definitive diagnosis. We present a case of a patient diagnosed with primary intramedullary spinal melanoma in his lower thoracic spinal cord who was effectively treated with surgical resection, adjuvant radiation, and adjuvant immunotherapy. Gross total resection (GTR) is most vital in the management of this spinal tumor. Although several studies have established the efficacy of immunotherapy agents in advanced malignant melanoma, the use of these agents has not been studied in primary central nervous system melanomas. This case provides insight into the diagnostic approach and treatment options for this unique malignancy.

PMID:34367798 | PMC:PMC8336382 | DOI:10.7759/cureus.16194

Permalink for 'Hematologic Emergencies in the Postoperative Neurointensive Care Unit Setting: Illustrative Case Series and Differential Diagnosis'

Hematologic Emergencies in the Postoperative Neurointensive Care Unit Setting: Illustrative Case Series and Differential Diagnosis

Fetched: August 8th, 2021, 5:01am GMT by Rana Hanna Al Shaikh

J Stroke Cerebrovasc Dis. 2021 Aug 3;30(10):106019. doi: 10.1016/j.jstrokecerebrovasdis.2021.106019. Online ahead of print.

ABSTRACT

OBJECTIVES: Investigating the development of acute thrombocytopenia, differential etiologies, and potentially the rare manifestation of disseminated intravascular coagulation after brain tumor resection of primary and secondary malignancies.

MATERIALS AND METHODS: We performed a retrospective review of a case series of post-operative neurosurgical patients which developed thrombocytopenia. We applied National Library of Medicine search engine methodology using the terms disseminated intravascular coagulation and brain tumors.

RESULTS: We report clinical, radiographic, and laboratory data of four Neurointensive care unit patients that developed thrombocytopenia, three with disseminated intravascular coagulation after craniotomy, and one with heparin-induced thrombocytopenia masquerading as low grade disseminated intravascular coagulation. All four patients presented with cranial lesions and underwent neurosurgical resection. Underlying disorders included: high grade glioma, stage IV lung cancer with metastases, and meningioma. One patient survived and was able to recover after several days of hospitalization, while another patient was discharged to hospice. Search results illustrated that disseminated intravascular coagulation in the presence of glioblastoma multiforme is rare (only four patients) and may be due to a release of coagulation factors like tissue plasminogen activator, treated with antifibrinolytic agents. Searching the terms disseminated intravascular coagulation and brain tumors in the National Library of Medicine search engine yielded 116 results; eight were relevant to our study.

CONCLUSIONS: Correlation of thrombocytopenia after neurosurgery for glioblastoma multiforme and disseminated intravascular coagulation is rare. It is extremely challenging to manage these patients with concomitant deep vein thrombosis/pulmonary embolism and intracranial bleeding. Heparin-induced thrombocytopenia is common yet possesses a different hematological coagulation profile and has more pharmacologic options. Neurointensive care unit teams should recognize intraoperative and post-operative disseminated intravascular coagulation cases, and heparin-induced thrombocytopenia in the differential of post-operative thrombocytopenia with specific pharmacologic interventions.

PMID:34359018 | DOI:10.1016/j.jstrokecerebrovasdis.2021.106019

Permalink for 'Next-Generation Sequencing in the Diagnosis of Metastatic Lesions: Reclassification of a Glioblastoma as an Endometrial Cancer Metastasis to the Brain'

Next-Generation Sequencing in the Diagnosis of Metastatic Lesions: Reclassification of a Glioblastoma as an Endometrial Cancer Metastasis to the Brain

Fetched: August 7th, 2021, 5:01am GMT by Shuk On Annie Leung

Oncologist. 2021 Aug 6. doi: 10.1002/onco.13927. Online ahead of print.

ABSTRACT

Endometrial cancer is the most common gynecologic cancer in the U.S., but metastasis to the brain is rare, and diagnosis can be challenging. Traditional tools for determining if a tumor is a primary or metastatic lesion include pan-imaging, histopathologic studies, and immunohistochemistry. Molecular testing with next-generation sequencing has been increasingly used to augment these tests. We present a case of a patient who initially presented with a brain lesion diagnosed as glioblastoma on histology and immunohistochemistry, but whose diagnosis was later changed to metastasis from an endometrial primary based on molecular findings. The two tumors shared a common microsatellite instability signature and 51 DNA variants, including oncogenic driver mutations KRAS p.G13D, PIK3CA p.E545A, and PTEN p.I135V and p.K267Rfs*9. This highlights the power of molecular analysis in making the diagnosis in cases of rare metastases. KEY POINTS: Brain metastasis from endometrial primary is rare, and histopathological features may be augmented with molecular analysis to aid in diagnosis. Comparison of the molecular makeup of the primary endometrial lesion with the metastatic lesion may reveal high-risk molecular features that may be indicative of metastatic potential.

PMID:34355460 | DOI:10.1002/onco.13927

Histone H3.3 G34-mutant Diffuse Gliomas in Adults

Fetched: August 6th, 2021, 5:01am GMT by Leiming Wang

Am J Surg Pathol. 2021 Aug 5. doi: 10.1097/PAS.0000000000001781. Online ahead of print.

ABSTRACT

The characteristics of H3.3 G34-mutant gliomas in adults have yet to be specifically described. Thirty adults with H3.3 G34-mutant diffuse gliomas were retrospectively reviewed for clinical and pathologic information. Molecular profiling using next-generation sequencing was performed in 29 of the 30 H3.3 G34-mutant patients with 1 patient lacking available tumor samples, as well as 82 IDH/H3 wild-type adult diffuse glioma patients. The age at diagnosis of H3.3 G34-mutant diffuse gliomas was significantly younger than IDH/H3 wild-type gliomas (24 vs. 57 y, P<0.001). Overall, 19 of the 30 patients were diagnosed of glioblastoma with the primitive neuronal component, and 8 were glioblastoma. The molecular profiling analysis revealed higher frequencies of Olig-2 loss of expression, TP53 mutation, ATRX mutation, PDGFRA mutation, and MGMT promoter methylation (P<0.05) in H3.3 G34-mutant gliomas than IDH/H3 wild-type gliomas. No TERT promoter mutation and only 1 case of EGFR amplification were detected in the H3.3 G34-mutant cohort, the frequencies of which were significantly higher in the IDH/H3 wild-type cohort. A dismal prognosis was observed in H3.3 G34-mutant patients comparing to IDH/H3 wild-type cohort (overall survival: 14 vs. 22 mo; P=0.026). Univariate and multivariate analyses showed that the extent of resection and TP53 mutation were independently affecting prognosis. The distinct pathologic and molecular features of H3.3 G34-mutant diffuse gliomas in adult patients demonstrated the clinical importance of detecting H3.3 G34R/V mutations. The dismal prognosis of this rare high-grade glioma disease we reported here would further promote the investigation of dedicated therapeutic strategies.

PMID:34352809 | DOI:10.1097/PAS.0000000000001781

Rare cases of medulloblastoma with hypermutation

Fetched: August 6th, 2021, 5:01am GMT by Aditi Bagchi

Cancer Rep (Hoboken). 2021 Aug 5:e1521. doi: 10.1002/cnr2.1521. Online ahead of print.

ABSTRACT

BACKGROUND: Medulloblastoma is the most common malignant brain tumor of childhood and is considered a tumor with low mutational burden (~1 Mut/Mb). Therefore, though the medulloblastoma genomes have been extensively characterized in literature, reports on potential hypermutations and underlying mutagenic processes in medulloblastomas are limited.

AIM: In this report, we studied the landscape of mutational burden in primary and recurrent medulloblastoma. Furthermore, we wanted to understand the differences in underlying mutagenic mechanisms in medulloblastoma with low and high mutational burdens.

METHODS: Fifty-three primary and recurrent medulloblastoma genomic sequence were downloaded from the European Genome Archive as BAM files. Thirty-three cases were obtained from formalin-fixed paraffin-embedded tissues from pathology diagnostic archives of Spectrum Health and Cooperative Human Tissue Network. Somatic mutations were called using Mutect2, following best practices guidelines for Genome Analysis Toolkit V4. Mutational signatures were analyzed using deconstructSigs.

RESULTS: We identified nine medulloblastoma cases with high mutational burden (>5 Mut/Mb). Of them, five cases met the criteria of hypermutation (>10Mut/Mb), two of the five tumors had canonical mutations in the POLE proof-reading domain, where a large proportion of mutations in these tumor genomes contributed to signature 10. The hypermutated cases also demonstrated mutational signatures 14, 15, and 21, indicating the role of mis match repair deficiency in their mutagenesis. Of the four known molecular subgroups in medulloblastoma-SHH, WNT, Group 3, and Group 4-both the POLE-mutated cases belonged to the SHH subgroup. This report identifies rare cases of hypermutation in medulloblastoma driven by defects in DNA repair mechanisms.

CONCLUSION: Hypermutation in medulloblastoma can impact therapeutic decisions, especially at recurrence in otherwise fatal high risk SHH-medulloblastomas. A defect in DNA repair leading to SHH -medulloblastoma is yet another important mechanism that should be further investigated in the genesis of these tumors. Therefore, this report provides important scientific and clinical rationale for future research looking for incidence of hypermutation in large cohorts of medulloblastoma patients.

PMID:34351088 | DOI:10.1002/cnr2.1521

An Intracranial Extradural Dermoid Cyst Presenting with Two Dermal Sinuses and an Abscess in a Child

Fetched: August 6th, 2021, 5:01am GMT by Aysha Albastaki

Case Rep Neurol Med. 2021 Jul 26;2021:9917673. doi: 10.1155/2021/9917673. eCollection 2021.

ABSTRACT

Dermoid cysts account for only a small fraction of intracranial masses, with extradural dermoid cysts being considered a much rarer entity than those located intradurally. Intracranial dermoid cysts vary in clinical presentations: some maybe asymptomatic whilst others harbor features of raised intracranial pressure, neurological deficits, or even aseptic meningitis. Dermoid cysts may also present with cutaneous lesions. Herein, we report a rare case of a 1-year-old female presenting with a midline, scalp abscess. Brain MRI revealed an intracranial, extradural tumor, with features suggestive of a dermoid cyst, unusually located in the crista galli, and complicated by the formation of two cutaneous sinus tracts. After identification and characterization by MRI, bitemporal craniotomy was performed with complete excision of the mass and sinus tracts. Histological analysis confirmed dermoid cyst as the final diagnosis. Postoperatively, the patient recovered fully and had no evidence of recurrence in subsequent visits. The case mentioned above highlights the rarity of such a presentation for an intracranial extradural dermoid cyst and the vitality of early imaging for midline cutaneous lesions for identification of intracranial extensions and avoidance of detrimental consequences.

PMID:34350042 | PMC:PMC8328740 | DOI:10.1155/2021/9917673

Clinical variability of neurofibromatosis 1: A modifying role of cooccurring PTPN11 variants and atypical brain MRI findings

Fetched: August 5th, 2021, 5:01am GMT by Alessandra D'Amico

Clin Genet. 2021 Aug 4. doi: 10.1111/cge.14040. Online ahead of print.

ABSTRACT

Neurofibromatosis 1 (NF1) is a disorder characterized by variable expressivity caused by loss-of-function variants in NF1, encoding neurofibromin, a protein negatively controlling RAS signaling. We evaluated whether concurrent variation in proteins functionally linked to neurofibromin contribute to the variable expressivity of NF1. Parallel sequencing of a RASopathy gene panel in 138 individuals with molecularly confirmed clinical diagnosis of NF1 identified missense variants in PTPN11, encoding SHP2, a positive regulator of RAS signaling, in four subjects from three unrelated families. Three subjects were heterozygous for a gain-of-function variant and showed a severe expression of NF1 (developmental delay, multiple cerebral neoplasms and peculiar cortical MRI findings), and features resembling Noonan syndrome (a RASopathy caused by activating variants in PTPN11). Conversely, the fourth subject, who showed an attenuated presentation, carried a previously unreported PTPN11 variant that had a hypomorphic behavior in vitro. Our findings document that functionally relevant PTPN11 variants occur in a small but significant proportion of subjects with NF1 modulating disease presentation, suggesting a model in which the clinical expression of pathogenic NF1 variants is modified by concomitant dysregulation of protein(s) functionally linked to neurofibromin. We also suggest targeting of SHP2 function as an approach to treat evolutive complications of NF1.

PMID:34346503 | DOI:10.1111/cge.14040

Meningioma as the host for metastatic breast cancer: A rare occurrence with important therapeutic impact

Fetched: August 5th, 2021, 5:01am GMT by Mohammad Hosseinzadeh

Surg Neurol Int. 2021 Jun 28;12:314. doi: 10.25259/SNI_148_2021. eCollection 2021.

ABSTRACT

BACKGROUND: Tumor-to-tumor metastasis is a rare condition. There are few reports of metastatic tumors within intracranial tumors, including meningiomas. Since some metastatic tumors have osteoblastic imaging pattern, it is not always easy to differentiate them from meningioma on preoperative studies.

CASE DESCRIPTION: A 60-year-old female referred to our center complaining about a progressive headache, nausea, and vomiting for the past month. She had a history of breast cancer treated with radical mastectomy (5 years ago) and adjuvant chemotherapy (until 1 year ago). Workups revealed a dural-based mass in the left temporobasal and midline subfrontal regions. Histopathological study showed breast cancer metastasis nests within the primary meningioma.

CONCLUSION: As the diagnosis of metastatic nests inside a benign tumor, drastically alters postoperative adjuvant treatments, a high index of suspicion is needed evaluating tumors from patients with a history of systemic neoplasms.

PMID:34345455 | PMC:PMC8326081 | DOI:10.25259/SNI_148_2021

A rare, pediatric, fourth-ventricular, anaplastic astrocytoma

Fetched: August 5th, 2021, 5:01am GMT by Dang Vinh Hiep

Radiol Case Rep. 2021 Jul 15;16(9):2676-2679. doi: 10.1016/j.radcr.2021.06.050. eCollection 2021 Sep.

ABSTRACT

Anaplastic astrocytoma, a diffusely infiltrating, malignant, astrocytic, primary brain tumor, is most commonly observed between 30 and 50 years of age. Anaplastic astrocytomas are now classified as WHO grade III lesions, with imaging characteristics and prognosis between diffuse low-grade astrocytomas (WHO grade II) and glioblastomas (WHO IV). Anaplastic astrocytoma can appear mostly in the cerebrum followed by cerebellum. However, it is rarely observed in the fourth ventricle. In this article, we aimed to describe an uncommon case of a pediatric, fourth-ventricular, anaplastic astrocytoma. A 9-year-old male who underwent MRI brain then adopted gross-total tumor eradication. The final histopathology findings were consistent with an anaplastic astrocytoma.

PMID:34345330 | PMC:PMC8319461 | DOI:10.1016/j.radcr.2021.06.050

Primary Dural Lymphoma Mimicking En Plaque Cerebellopontine Angle Meningioma

Fetched: August 4th, 2021, 5:01am GMT by Mei Xu

Case Rep Pathol. 2021 Jul 23;2021:2845995. doi: 10.1155/2021/2845995. eCollection 2021.

ABSTRACT

Primary dural lymphoma (PDL) usually arises from the calvarial dura without the brain parenchyma or systemic involvement and thus may not be considered as a typical form of primary CNS lymphoma (PCNSL). It is exceedingly rare. When it occurs, it might not be suspected as a primary diagnosis on clinical and radiologic findings. We present a PDL case that occurs at the cerebellopontine (CP) angle mimicking en plaque meningioma. The tumor histopathology showed a lymphoproliferative disorder immunophenotypically consistent with a low-grade marginal zone lymphoma. Bone marrow and systemic involvements were not identified, and a diagnosis of PDL was established. As a residual tumor at the CP angle was inaccessible to surgery, postoperative radiation therapy was performed. No recurrence was found at 15-month follow-up. PDLs are mostly indolent and have a good prognosis. There is no doubt that the most important differential diagnosis is meningioma. Furthermore, the present case emphasizes the necessity of an intraoperative consultation and knowledge of this rare yet essential form of PCNSL so that appropriate studies can be ordered.

PMID:34341695 | PMC:PMC8325585 | DOI:10.1155/2021/2845995

Melanoma of the central nervous system: A report of three cases

Fetched: August 4th, 2021, 5:01am GMT by Pooja K Gajaria

Indian J Pathol Microbiol. 2021 Jul-Sep;64(3):535-540. doi: 10.4103/IJPM.IJPM_642_20.

ABSTRACT

Melanomas within the Central Nervous System (CNS) are most commonly metastatic lesions, with primary melanomas comprising only 0.05-0.07% of all brain tumors. We report three cases of primary CNS melanoma. The patients were young adults. There were two females and one male. On preoperative investigations, two cases were misdiagnosed to be angiomas on Magnetic Resonance Imaging (MRI). The melanotic nature of the lesion was an intraoperative observation. Pathologic examination showed features of malignancy with invasion of tumor cells into the brain parenchyma. In two patients, presence of systemic lesions were ruled out after surgery by whole-body Positron Emission Tomography (PET) scan. These patients were subject to adjuvant radiotherapy, while one patient succumbed immediately post-surgery. Primary CNS melanomas are rare with no defined treatment protocols. Histopathology diagnosis is crucial to rule out pigmented mimics.

PMID:34341267 | DOI:10.4103/IJPM.IJPM_642_20

Permalink for 'Malignant phyllodes tumor with synchronous metastases to axillary lymph nodes, lung at the presentation: a case report and literature review'

Malignant phyllodes tumor with synchronous metastases to axillary lymph nodes, lung at the presentation: a case report and literature review

Fetched: August 3rd, 2021, 5:01am GMT by Quang Hong Le

J Surg Case Rep. 2021 Jul 29;2021(7):rjab302. doi: 10.1093/jscr/rjab302. eCollection 2021 Jul.

ABSTRACT

Phyllodes tumors are rare mesenchymal tumors of breast. Malignant phyllodes tumors can develop metastases to distal organs with spreading hematogenously to most frequent sites as lungs, bone and brain. Regional lymph node enlargement is common but metastasis to lymph node is a very rare phenomenon with prevalence about 1.1-3.8%. In this report, we introduce a case of malignant phyllodes tumor of breast in 57-year-old female patient. Synchronous metastases to axillary lymph nodes and lung were found at the presentation. She was treated with mastectomy combined with axillary lymph node dissection and adjuvant chemotherapy. Axillary lymph node dissection can be considered in case of proven metastatic or suspiciously palpable lymph nodes.

PMID:34336184 | PMC:PMC8321595 | DOI:10.1093/jscr/rjab302

Choriocarcinoma brain metastasis in a patient in the third trimester: a case report

Fetched: August 3rd, 2021, 5:01am GMT by Chunjuan Shen

J Med Case Rep. 2021 Jul 29;15(1):417. doi: 10.1186/s13256-021-02808-3.

ABSTRACT

BACKGROUND: Metastatic choriocarcinoma in the third trimester of pregnancy is extremely rare.

CASE PRESENTATION: A 25-year-old Chinese woman (gravida 3, para 0) who was 28 weeks pregnant was admitted for sudden convulsion, aconuresis, and unconsciousness. The decision was made to perform an emergency cesarean delivery and craniotomy, hematoma clearance, and decompression. Pathological examination confirmed choriocarcinoma with brain metastasis. The patient underwent chemotherapy with the etoposide, cisplatin (EP) and etoposide, methotrexate and dactinomycin alternating with cyclophosphamide and vincristine (EMACO) regimens. A satisfactory result was achieved.

CONCLUSIONS: When encountering intracranial mass or bilateral pulmonary nodules in a pregnant woman, especially one in the third trimester, metastatic choriocarcinoma should be considered.

PMID:34325722 | PMC:PMC8320190 | DOI:10.1186/s13256-021-02808-3

The GIST of It: A Rare Presentation of Neurofibromatosis Type I

Fetched: August 3rd, 2021, 5:01am GMT by Amit R Hudgi

Cureus. 2021 Jun 29;13(6):e16034. doi: 10.7759/cureus.16034. eCollection 2021 Jun.

ABSTRACT

Neurofibromatosis-1 (NF-1) is an autosomal dominant condition characterized by cutaneous pigmentation and tumour formation along nerves in the brain, skin, and other organs. Gastrointestinal stromal tumours (GIST) are rare mesenchymal tumours involving the gastrointestinal tract (GI) associated with NF-1. We present a case of life-threatening GI bleeding from GIST in a patient with NF-1. In NF-1 patients presenting with GI bleeding, GISTs should be part of the differential. Clinicians must have a low threshold for urgent abdominal imaging if endoscopy does not detect the source of GI bleeding.

PMID:34336521 | PMC:PMC8319233 | DOI:10.7759/cureus.16034

Focal Neurologic Deficit After Epidural Catheter Removal Leads to Meningioma Diagnosis

Fetched: August 3rd, 2021, 5:01am GMT by Morgan A Clond

Cureus. 2021 Jun 29;13(6):e16015. doi: 10.7759/cureus.16015. eCollection 2021 Jun.

ABSTRACT

We present an unusual case of a 60-year-old female who developed subtle, new-onset left upper and lower extremity weakness on day five of perioperative thoracic epidural placement. The onset of a focal neurological deficit after epidural placement usually raises suspicion for the presence of an epidural hematoma, abscess, or traumatic cord lesion. However, in this patient, brain imaging revealed a large, previously undiagnosed intracranial mass. Classically, the risk of mass-related intracranial pressure shifts leading to neurological changes is associated with spinal techniques, including diagnostic lumbar puncture, combined spinal-epidural catheter analgesia, and unintended dural puncture during epidural placement. However, based on this case and our summary of case reports in the literature, we determined that symptom onset associated with an intracranial mass may also arise after apparently uncomplicated epidural placement. Symptom onset in our case series ranged from six hours to ten days and was highly variable depending on tumor location, with reported signs and symptoms including headache, vision changes, focal deficits, or alterations of consciousness. Further studies are required to establish definitive causation between the epidural technique and changes in cerebrospinal fluid pressures leading to symptom onset. Though rare, this is a time-sensitive diagnosis that must be considered for any patient with unexplained neurological findings after neuraxial anesthesia.

PMID:34336505 | PMC:PMC8319221 | DOI:10.7759/cureus.16015

Plurihormonal pituitary macroadenoma: a case report

Fetched: August 1st, 2021, 5:01am GMT by Ebtesam Allehaibi

J Med Case Rep. 2021 Jul 29;15(1):407. doi: 10.1186/s13256-021-02948-6.

ABSTRACT

BACKGROUND: Plurihormonal pituitary adenomas are a unique type of pituitary adenomas that secrete two or more pituitary hormones normally associated with separate cell types that have different immunocytochemical and ultrastructural features. Although they represent 10-15% of all pituitary tumors, only a small fraction of plurihormonal pituitary adenomas clinically secrete multiple hormones. The most common hormone combinations secreted by plurihormonal pituitary adenomas are growth hormone, prolactin, and one or more glycoprotein hormones. The most common hormonal symptom is acromegaly (50%). The aim of this case report is to bring awareness about this rare type of pituitary adenomas and to describe the unique presentation of our patient, even though plurihormonal pituitary adenomas are known mostly as a clinically silent tumors.

CASE PRESENTATION: Herein, we describe an unusual case of plurihormonal pituitary adenoma with triple-positive staining for adrenocorticotropic hormone, growth hormone, and prolactin. The patient is a 65-year-old Egyptian woman who presented with mass effect symptoms of the pituitary tumor, which primarily manifested as severe headache and visual field defects. She also presented with some cushingoid features, and further analysis confirmed Cushing's disease; slightly high prolactin and normal growth hormone levels were observed. She underwent transsphenoidal surgery and has been in remission thus far. Only a few cases have been reported in the literature, but none has exhibited silent acromegaly or mass effect symptoms as the initial presentation.

CONCLUSION: This case highlights an unusual plurihormonal pituitary adenoma case with a rare combination of secreted hormones; mass effect symptoms were dominant, as were uncommon visual field defects. Our case further proves that immunohistochemical analyses of all pituitary hormones are needed to ensure correct diagnosis and to alert clinicians to the need for more rigorous follow-up due to the higher morbidity of these patients. Our case report approval number Federal Wide Assurance NIH, USA is FWA00018774 IRB registration number with OHRP/NIH is IRB00010471.

PMID:34321093 | PMC:PMC8320198 | DOI:10.1186/s13256-021-02948-6

Rare Cancer News & Clinical Trials » PubMed - Rare Brain Tumor

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PubMed - Rare Brain Tumor (75 unread)

Fetched: September 16th, 2021, 5:01am GMT by Ramona Cordani

Fetched: September 16th, 2021, 5:01am GMT by Dong-Won Shin

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