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rhabdoid meningioma

Ailing Guo, Vigneyshwar Suresh, Xianzhi Liu, Fuyou Guo
PURPOSE: Giant pediatric intracranial tumor (GPIT) remains to be a challenging disease with high morbidity and mortality. METHODS: The clinical data of 60 patients under 18 years of age operated on with GPIT (≥5 cm in diameter) were retrospectively analyzed. RESULTS: Gross total resection was achieved in 46 cases (77%) and subtotal resection was obtained in 14 cases (23%). Ninety percent (47/52) of the cases with obstructive hydrocephalus were resolved remarkably and only 10% (5/52) of the patients needed a ventriculoperitoneal shunt after tumor resection...
March 2017: Child's Nervous System: ChNS: Official Journal of the International Society for Pediatric Neurosurgery
Ganesh M Shankar, Malak Abedalthagafi, Rachael A Vaubel, Parker H Merrill, Naema Nayyar, Corey M Gill, Ryan Brewster, Wenya Linda Bi, Pankaj K Agarwalla, Aaron R Thorner, David A Reardon, Ossama Al-Mefty, Patrick Y Wen, Brian M Alexander, Paul van Hummelen, Tracy T Batchelor, Keith L Ligon, Azra H Ligon, Matthew Meyerson, Ian F Dunn, Rameen Beroukhim, David N Louis, Arie Perry, Scott L Carter, Caterina Giannini, William T Curry, Daniel P Cahill, Frederick G Barker, Priscilla K Brastianos, Sandro Santagata
Background.: Patients with meningiomas have widely divergent clinical courses. Some entirely recover following surgery alone, while others have relentless tumor recurrences. This clinical conundrum is exemplified by rhabdoid meningiomas, which are designated in the World Health Organization Classification of Tumours as high grade, despite only a subset following an aggressive clinical course. Patient management decisions are further exacerbated by high rates of interobserver variability, biased against missing possibly aggressive tumors...
April 1, 2017: Neuro-oncology
Anna S Berghoff, Philip Kresl, Michal Bienkowski, Christian Koelsche, Ursula Rajky, Johannes A Hainfellner, Matthias Preusser
INTRODUCTION: NAB2-STAT6 gene fusion is a molecular characteristic of solitary fibrous tumors (SFT) and hemangiopericytoma, underscoring their definition as one diagnostic entity. NAB2-STAT6 fusion is associated with nuclear relocation of STAT6 protein that can be detected by immunohistochemistry. We evaluated the diagnostic value of STAT6 expression in meningeal tumors. METHODS: 77 meningeal tumors (17/77 (22.0%) SFT/hemangiopericytoma, 11/77 meningothelial meningioma, 10/77 atypical meningioma 8/77 chordoid meningioma, 9/77 fibroblastic meningioma, 10/77 transitional meningioma, 3/77 rhabdoid meningioma and 9/77 anaplastic meningioma) were included...
March 2017: Clinical Neuropathology
Hildegard Kehrer-Sawatzki, Said Farschtschi, Victor-Felix Mautner, David N Cooper
Schwannomatosis is characterized by the predisposition to develop multiple schwannomas and, less commonly, meningiomas. Despite the clinical overlap with neurofibromatosis type 2 (NF2), schwannomatosis is not caused by germline NF2 gene mutations. Instead, germline mutations of either the SMARCB1 or LZTR1 tumour suppressor genes have been identified in 86% of familial and 40% of sporadic schwannomatosis patients. In contrast to patients with rhabdoid tumours, which are due to complete loss-of-function SMARCB1 mutations, individuals with schwannomatosis harbour predominantly hypomorphic SMARCB1 mutations which give rise to the synthesis of mutant proteins with residual function that do not cause rhabdoid tumours...
February 2017: Human Genetics
Mateusz Bujko, Marcin M Machnicki, Emilia Grecka, Nataliia Rusetska, Ewa Matyja, Paulina Kober, Tomasz Mandat, Małgorzata Rydzanicz, Rafał Płoski, Romuald Krajewski, Wieslaw Bonicki, Tomasz Stokłosa, Janusz A Siedlecki
BACKGROUND: Rhabdoid meningioma is rare aggressive meningioma histological subtype that develops predominantly through progression from less malignant tumors. Owing to its low incidence, this tumor's biological background is unknown. The aim of this study was to profile somatic mutations in 4 meningioma samples from the same patient, derived previously from 4 subsequent tumor resections. CASE DESCRIPTION: A 58-year-old woman presented with recurrent meningioma progressing from atypical to rhabdoid subtype...
January 2017: World Neurosurgery
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...
June 27, 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
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