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rhabdoid meningioma

Jeong-Kwon Kim, Tae-Young Jung, Shin Jung, Kyung-Hwa Lee, Seul-Kee Kim, Eun Jung Lee
Papillary and rhabdoid meningiomas are pathologically World Health Organization (WHO) grade III. Any correlation between clinical prognosis and pathologic component is not clear. We analyzed the prognoses of patients with meningiomas with a rhabdoid or papillary component compared to those of patients with anaplastic meningiomas. From 1994 to June 2013, 14 anaplastic meningiomas, 6 meningiomas with a rhabdoid component, and 5 meningiomas with papillary component were pathologically diagnosed. We analyzed magnetic resonance imaging (MRI) findings, extent of removal, adjuvant treatment, progression-free survival (PFS), overall survival (OS), and pathologic features of 14 anaplastic meningiomas (group A), 5 meningiomas with a predominant (≥50%) papillary or rhabdoid component (group B1), and 6 meningiomas without a predominant (<50%) rhabdoid or papillary component (group B2)...
July 2016: Journal of Korean Neurosurgical Society
Felix Behling, Alonso Barrantes-Freer, Marco Skardelly, Maike Nieser, Arne Christians, Florian Stockhammer, Veit Rohde, Marcos Tatagiba, Christian Hartmann, Christine Stadelmann, Jens Schittenhelm
BACKGROUND: Treatment options for oncological diseases have been enhanced by the advent of targeted therapies. The point mutation of the BRAF gene at codon 600 (BRAF V600E) is found in several tumor entities and can be approached with selective inhibitory antibodies. The BRAF inhibitor vemurafenib has demonstrated clinical efficacy in patients with BRAF V600E-mutant melanoma brain metastases and in other cancer diseases. Therefore the BRAF V600E mutation is a highly interesting oncological target in brain tumors...
2016: Diagnostic Pathology
Ihsan Yuce, Suat Eren, Akin Levent, Mecit Kantarci, Ali Kurt, Onder Hilmi Okay
A 20-year-old male patient was admitted to our clinic with a 1-year history of headache. The patient's systemic-neurological examination and laboratory findings were normal. Computed tomography and magnetic resonance imaging were performed. Imaging findings showed calcified intraventricular mass and subependymal and gyral nodular lesions. There was a slight increase in ventricular volume. Surgical treatment was performed. Pathological specimens revealed the diagnosis of rhabdoid meningioma. Leptomeningeal dissemination refers to diffuse seeding of the leptomeninges by tumor metastases...
2016: Turkish Neurosurgery
Aanchal Kakkar, Saphalta Baghmar, Ajay Garg, Vaishali Suri, Vinod Raina, Chitra Sarkar, Mehar Chand Sharma
Rhabdoid meningioma is a rare meningioma variant, classified as WHO grade III. Although this tumor is known for its aggressive behavior and poor prognosis, extracranial metastasis is rare. We report the rare case of a 31-year-old patient with rhabdoid meningioma which recurred several times despite gross total resection, radiation therapy, and gamma knife radiosurgery, and the last recurrence was associated with metastases to lungs, lymph node and bone. The patient showed no response to paclitaxel-carboplatin, or vincristine-cyclophosphamide-adriamycin chemotherapy, and succumbed to the disease...
July 2016: Brain Tumor Pathology
Jaishri O Blakeley, Scott R Plotkin
Neurofibromatosis type 1 (NF1), neurofibromatosis type 2 (NF2), and schwannomatosis (SWN) are tumor-suppressor syndromes. Each syndrome is an orphan disease; however, the tumors that arise within them represent the most common tumors of the nervous system worldwide. Systematic investigation of the pathways impacted by the loss of function of neurofibromin (encoded byNF1) and merlin (encoded byNF2) have led to therapeutic advances for patients with NF1 and NF2. In the syndrome of SWN, the genetic landscape is more complex, with 2 known causative genes (SMARCB1andLZTR1) accounting for up to 50% of familial SWN patients...
May 2016: Neuro-oncology
Mónica B Mezmezian, Mónica B Carassai, Valeria Dopazo, María L Deforel, María Á Puzzo
INTRODUCTION: Immunostaining of progesterone receptors (PRs) has been described as a prognostic factor related to recurrences in meningiomas. However, its expression in other primary intracranial tumors has been poorly studied. In this paper, we compare the pattern of expression of the receptor in meningiomas with that of nonmeningothelial intracranial tumors to evaluate its value in the diagnosis of the former. MATERIALS AND METHODS: A total of 42 nonmeningothelial intracranial tumors (21 glioblastomas, 4 anaplastic oligodendrogliomas, 4 oligodendrogliomas, 1 pilomyxoid astrocytoma, 3 ependymomas, 8 schwannomas, 1 chordoid chordoma) and 32 meningiomas (1 rhabdoid, 1 papillary, 5 atypical, 7 with histologic features of more aggressive behavior, 1 microcyst, 8 meningothelial, 7 transitional, 2 fibroblastic) were studied for PR by immunohistochemistry...
January 13, 2016: Applied Immunohistochemistry & Molecular Morphology: AIMM
Li Wang, Fugang Han, Ming Chen, Yue Chen
An 18-year-old man with progressive headache and vomiting for 2 weeks had significantly elevated levels of WBC count, which kept on rising over time during in-hospital evaluation. Exhaustive examinations did not reveal infection or any other explanations of increased WBC count. Instead, brain MRI and FDG PET/CT identified a malignant lesion in the brain without abnormality elsewhere. The pathological examination revealed a rhabdoid meningioma. The level of the WBC counts returned to normal promptly after surgical resection of the tumor, which confirmed the diagnosis of paraneoplastic leukemoid reaction...
March 2016: Clinical Nuclear Medicine
Valeria Barresi, Giovanni Branca, Alessandro Raso, Samantha Mascelli, Maria Caffo, Giovanni Tuccari
Rhabdoid tumors are a spectrum of neoplasias composed of cells which show rhabdoid morphology but are devoid of skeletal muscle differentiation. These tumors are characterized by inactivation of the INI1/SMARCB1 gene and they have been described in virtually every anatomical site, including the central nervous system (CNS) and sinonasal tract. Rhabdoid tumor of the CNS was named atypical teratoid rhabdoid tumor (ATRT) and it mainly affects children under the age of 3 years with supra- or infra-tentorial location...
November 16, 2015: Neuropathology: Official Journal of the Japanese Society of Neuropathology
Tadanori Tomita, Gordan Grahovac
OBJECTIVE: Cerebellopontine angle (CPA) and cerebellomedullary fissure (CMF) tumors are rare in children and information is scarce in the literature. This retrospective study reports their histological distribution and tumor origin, and describes surgical resections and post-operative outcome based upon the authors' consecutive personal series. METHODS: Clinical data of infants and children 16 years old or younger of age treated from 2001 to 2012 by a single surgeon was retrospectively reviewed...
October 2015: Child's Nervous System: ChNS: Official Journal of the International Society for Pediatric Neurosurgery
Jie Huang, Zhaoxia Zhou, Xiaxiang Jin
Female patients, 50 years old, have a recurrent unilateral aggravating headache for 5 years. Without runny nose, sneezing, nasal hemorrhage, smell or vision loss. Prefessional examination: there is a visible hoar neoplasm in the right middle nasal meatus With smooth surface and rich in vascular. The nasopharyngeal MRI shows that there is a occupancy lesion in the right nasal cavity and sinuses, well-demarcated, about 21. 5 mm x 25.5 mm x 37.0 mm. Angiofibroma is the most likely diagnosis. Postoperative pathological section shows that tumor are hypercellular, which contains big nucleus...
May 2015: Journal of Clinical Otorhinolaryngology, Head, and Neck Surgery
Jo Van Dorpe, Anne Hoorens, Nadine Van Roy, Franceska Dedeurwaerdere, David Creytens
No abstract text is available yet for this article.
March 2016: Histopathology
S Zouaoui, A Darlix, V Rigau, H Mathieu-Daudé, F Bauchet, F Bessaoud, P Fabbro-Peray, B Trétarre, D Figarella-Branger, L Taillandier, H Loiseau, L Bauchet
PURPOSE: This work describes the clinical epidemiology and pathology for patients undergoing surgery for newly diagnosed meningiomas in France between 2006 and 2010. METHODS: The methodology is based on a multidisciplinary national network previously established by the French Brain Tumor DataBase (FBTDB) (in French: Recensement national histologique des tumeurs primitives du système nerveux central [RnhTPSNC]), and the active participation of the scientific societies involved in neuro-oncology in France...
June 11, 2015: Neuro-Chirurgie
Kavita Mardi, R C Thakur, Biswajit Biswas
We reported a case of meningioma with rhabdoid morphology but lacking histological features of malignancy in arising from the spinal cord in a 28-year-old male. The tumor showed light microscopic, immunohistochemical evidence of meningothelial differentiation together with diffuse areas exhibiting rhabdoid morphology. The rhabdoid areas were characterized by cells with large cytoplasmic eosinophilic inclusions and eccentric nuclei. Unlike most cases reported in the literature, this case lacked significant mitotic activity and other atypical features...
April 2015: Asian Journal of Neurosurgery
Ch Karunakar Reddy, A Divakar Rao, Chandra K Ballal, Shrijeet Chakraborti
Rhabdoid meningioma is an uncommon anaplastic variant of meningioma. We describe the clinicoradiological and histomorphological features of two such cases of meningioma, with a brief review of literature. One case in a 9-year-old girl, presented initially as an atypical meningioma in the right fronto-parietal region, which on recurrence 18 months later, evolved into a rhabdoid meningioma. The second case in a 33-year-old male was located in the right parieto-occipital region.
February 2015: Journal of Clinical and Diagnostic Research: JCDR
David L Stockman, Jonathan L Curry, Carlos A Torres-Cabala, Ian R Watson, Alan E Siroy, Roland L Bassett, Lihua Zou, Keyur P Patel, Rajyalakshmi Luthra, Michael A Davies, Jennifer A Wargo, Mark A Routbort, Russell R Broaddus, Victor G Prieto, Alexander J Lazar, Michael T Tetzlaff
BACKGROUND: SMARCB1 (INI1/BAF47/SNF5) encodes a part of a multiprotein complex that regulates gene expression through chromatin remodeling. SMARCB1 expression is lost or downregulated in multiple human tumors, including epithelioid sarcoma, meningioma and rhabdoid tumors of the brain, soft tissue and kidney. METHODS: A 46-gene or 50-gene next-generation sequencing AmpliSeq Cancer Panel (Life Technologies; San Francisco, CA, USA) was applied to ∼1400 primary or metastatic melanoma tissues...
May 2015: Journal of Cutaneous Pathology
William Makis, Karey McCann, Alexander J B McEwan
An 18-year-old girl presented with a 3-year history of a recurrent skull base mass confirmed to be a rhabdoid papillary meningioma. The tumor was octreotide avid and metastatic to the lungs, thoracic lymph nodes, and bones, and she was referred for PRRT (peptide receptor radionuclide therapy) with 177Lu DOTATATE. After 3 induction treatment cycles of 177Lu DOTATATE, she experienced significant improvements in her symptoms; however, just before the fourth treatment, she developed cervical spinal cord compression and passed away shortly thereafter...
March 2015: Clinical Nuclear Medicine
Rajesh Parameshwaran Nair, Vinod, Yashdeep Sarma, Bhavna Nayal, Sumeet Kaur Dil, Pradeep Kumar Tripathi
INTRODUCTION: Tumors involving the parotid are predominantly primary with metastatic lesions forming a miniscule population. Meningioma metastasizing to the parotid is extremely rare and hence can often be mistaken for the more common primary salivary gland neoplasms. PRESENTATION OF CASE: A 59-year-old male presented with a swelling in the left parotid region. Fine needle aspiration cytology was suggestive of myoepithelial predominant pleomorphic adenoma. A superficial parotidectomy performed revealed a tumor composed of rhabdoid cells with abundant finely granular eosinophilic cytoplasm raising a possibility of myoepithelioma...
2015: International Journal of Surgery Case Reports
Charlotte Dai Kubicky, Arjun Sahgal, Eric L Chang, Simon S Lo
There are close to 70,000 new cases of primary central nervous system tumors diagnosed annually in the United States. Meningiomas, gliomas, nerve sheath tumors and pituitary tumors account for 85% of them. There is abundant literature on these commonly occurring tumors but data from the literature on infrequently encountered tumors such as atypical teratoid/rhabdoid tumor, choroid plexus carcinoma, ganglioglioma, hemangiopericytoma, and pleomorphic xanthoastrocytoma are limited. This review provides an overview of the clinicopathologic and therapeutic aspects of these rare primary central nervous system tumors...
July 30, 2014: Rare Tumors
Oz Mordechai, Sergey Postovsky, Eugene Vlodavsky, Ayelet Eran, Shlomi Constantini, Eynat Dotan, Emmanuela Cagnano, Myriam Weyl-Ben-Arush
Rhabdoid meningioma is an aggressive phenotype of meningioma, associated with a poor prognosis. We present a very rare case of high-grade meningioma with rhabdoid features that eventually expressed in a coma state. Comprehensive genomic profiling using a Next Generation Sequencing (NGS) assay revealed three genomic alterations: activating BRAF mutation (V600E), loss of CDKN2A/2B, and APC I1307K. After treatment with BRAF inhibitor (dabrafenib), the child's clinical condition improved progressively. After seven months, an MEK inhibitor was added (trametinib)...
April 2015: Pediatric Hematology and Oncology
Wenting Jia, Yukihiko Sonoda, Ryuta Saito, Toshiki Endo, Mika Watanabe, Teiji Tominaga
INTRODUCTION: Rhabdoid papillary meningioma is a rare meningioma variant categorized as WHO grade III. We report an 11-year-old girl with an intracerebral rhabdoid papillary meningioma. MATERIALS AND METHODS: An 11-year-old girl presented to our hospital with a short history of severe headache and vomiting. MRI revealed a left frontal intracerebral cystic lesion with rim enhancement. The tumor, including the cyst wall, was totally removed. Since the histological diagnosis was rhabdoid papillary meningioma, she was treated with postoperative radiotherapy...
December 2014: Child's Nervous System: ChNS: Official Journal of the International Society for Pediatric Neurosurgery
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