Primary rhabdoid tumor of the brain in an adult

Rezanko, Türkan; Tunakan, Mine; Kahraman, Aslil; Sucu, Hasan Kamil; Gelal, Fazıl; Akkol, Ismail

doi:10.1111/j.1440-1789.2006.00624.x

Cited by 30 publications

(15 citation statements)

References 17 publications

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“…AT/RT was first recognized as a malignant intracranial rhabdoid tumor in an adult patient in 1992 by Horn et al 30 From then on, approximately 30 cases of adult AT/RT have been reported. [18][19][20][21][22][23][24][25][26][27][28][29][30] In contrast to the pediatric neoplasm, most adult CNS rhabdoid tumors are supratentorial, with only 3 tumors located in the cerebellum and 2 in the spinal cord. Most cases were diagnosed on the basis of characteristic histologic and immunohistochemical features.…”

Section: Discussionmentioning

confidence: 99%

“…17 To our knowledge, only 29 cases of primary AT/RT of the CNS in adults have been reported (On-line Table). [18][19][20][21][22][23][24][25][26][27][28][29][30] The On-line Table summarizes the clinical and imaging data from our patients and from those in the literature.…”

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confidence: 99%

“…10,18,19,24 To our knowledge, there are only limited case reports describing the imaging features of this entity in adults. 8,9,18,19,[21][22][23][24][25][26][27][28][29][30][31] The goal of this study was to characterize the CT and MR imaging features of CNS AT/RT in 5 adults. …”

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Atypical Teratoid/Rhabdoid Tumors in Adult Patients: CT and MR Imaging Features

Han¹,

Qiu²,

Xie³

et al. 2010

AJNR Am J Neuroradiol

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SUMMARY:Primary AT/RT is a rare highly malignant tumor of the CNS, usually occurring in children younger than 5 years of age. The objective of this study was to characterize the CT and MR imaging findings in a series of 5 adult patients with pathologically proved AT/RT. All 5 AT/RTs were supratentorial. In 2 patients who underwent nonenhanced CT, the tumors appeared isoattenuated, and 1 of the 2 tumors contained calcifications. Solid portions of the tumors on MR imaging were isointense on T1-weighted, T2-weighted, and FLAIR images, and 1 case showed restricted diffusion on DWI. The tumors also demonstrated a bandlike rim of strong enhancement surrounding a central cystic area on contrast-enhanced T1-weighted imaging. One tumor was associated with destruction of the calvaria. Although AT/RTs can have nonspecific findings, the tumors in our series were large and isointense on T1-weighted, T2-weighted, and FLAIR images with central necrosis and prominent rim enhancement.ABBREVIATIONS: ADC ϭ apparent diffusion coefficient; AT/RT ϭ atypical teratoid/rhabdoid tumor; CNS ϭ central nervous system; DWI ϭ diffusion-weighted imaging; FLAIR ϭ fluid-attenuated inversion recovery; L ϭ left; mRNA ϭ messenger ribonucleic acid; N/A ϭ not available, ND ϭ not depicted; OS ϭ overall survival; PNET ϭ primitive neuroectodermal tumor; PR ϭ partial resection; R ϭ right; TR ϭ total resection A lthough AT/RT was first described as a rare, highly malignant childhood neoplasm of the kidney, it has also been reported at several extrarenal sites including the skin, chest wall, liver, thymus, optic nerve, and the CNS. [1][2][3][4][5][6][7][8][9][10][11][12][13][14][15][16] When found in the CNS, it usually arises from the posterior fossa and is most commonly found in children younger than 5 years of age.17 To our knowledge, only 29 cases of primary AT/RT of the CNS in adults have been reported (On-line Table). 18-30The On-line Table summarizes the clinical and imaging data from our patients and from those in the literature.The prognosis of patients with an AT/RT is poor, especially in patients younger than 3 years of age, with a survival time that averages 15 months in children and 38 months in adults. 10,18,19,24 To our knowledge, there are only limited case reports describing the imaging features of this entity in adults. 8,9,18,19,[21][22][23][24][25][26][27][28][29][30][31] The goal of this study was to characterize the CT and MR imaging features of CNS AT/RT in 5 adults. Materials and Methods PatientsThis study was approved by our institutional review board in accordance with standards of the National Institutes of Health. Five consecutive adult patients who received a diagnosis of AT/RT from January 2001 to October 2009 were included in the study. All patients had undergone surgical resection of their tumor and had received postoperative radiation therapy and chemotherapy. The resection specimens were collected and analyzed at 1 central pathology laboratory. Definitive diagnosis was based on histologic and immunohistochemical evaluation...

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Section: Discussionmentioning

confidence: 99%

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confidence: 99%

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Atypical Teratoid/Rhabdoid Tumors in Adult Patients: CT and MR Imaging Features

Han¹,

Qiu²,

Xie³

et al. 2010

AJNR Am J Neuroradiol

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“…6,25) Only 25 adult cases of cerebral RTs, including AT/RTs and malignant RTs, have been reported. 1,2,8,10,[13][14][15]19,23,24,27,31,32,38,39,41,42,45) Only 6 cases of epithelioid glioblastoma with a rhabdoid component have been reported. 16,26,28,47) However, the World Health Organization (WHO) Classification of Tumours of the Central Nervous System classifies AT/RTs as embryonal tumors, but does not list malignant RT and rhabdoid glioblastoma as distinct categories.…”

Section: Introductionmentioning

confidence: 99%

Malignant Brain Tumor With Rhabdoid Features in an Adult -Case Report-

Mutou

Hirose

Ikeda

et al. 2011

Neurol. Med. Chir.(Tokyo)

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Rhabdoid tumor (RT) of the central nervous system is an uncommon and aggressive neoplasm that usually affects pediatric patients. Currently, these tumors are classified as malignant RT or atypical teratoid/RT. Another entity of intraparenchymal brain tumor with a rhabdoid component is the extremely rare rhabdoid glioblastoma. A 23-year-old woman presented with a malignant RT in the right thalamus. The tumor was adjacent to the right lateral ventricle and was partially resected. Histological examination revealed prominent proliferation of rhabdoid cells, which is consistent with a diagnosis of malignant RT; the typical features of glioblastoma were not observed. The tumor cells stained positively for integrase interactor-1 and glial fibrillary acidic protein. Therefore, the tumor may have originated from glial components. Genetic analysis using comparative genomic hybridization showed a deoxyribonucleic acid copy-number gain on chromosome 7 but not on chromosome 22. The tumor did not respond to chemotherapy or radiotherapy, and the patient survived for only 4 months after surgery. The present case of malignant RTs shows certain similarities with those of rhabdoid glioblastoma. Further accumulation and analysis of data, including data from genetic analyses, may lead to the identification of a new type of malignant RT.

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“…6,7,10,13,15,17,18 Only single cases have been reported in adults. [22][23][24][25][26][27][28][29][30][31][32] Several large, hospital-based series established an atypical teratoid/rhabdoid tumor prevalence of 1% to 2% among pediatric brain tumors, 8,[33][34][35] but population-based data on the incidence of atypical teratoid/rhabdoid tumors in children are not yet available. However, such data provide important insights into the burden of disease and might contribute to patient care.…”

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Incidence of atypical teratoid/rhabdoid tumors in children

Wöehrer

Slavc

Waldhoer

et al. 2010

Cancer

130

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on behalf of the Austrian Brain Tumor Registry BACKGROUND: Atypical teratoid/rhabdoid tumors are highly malignant embryonal central nervous system (CNS) tumors that were defined as an entity in 1996. As compared with other malignant CNS tumors, their biological behavior is particularly aggressive, but patients may benefit from an intensified treatment. Atypical teratoid/rhabdoid tumors display a complex histomorphology, which renders them prone to misdiagnosis. They occur predominantly in young children, with an estimated prevalence of 1% to 2% among all pediatric CNS tumors. However, populationbased data on the incidence of these tumors are not yet available. METHODS: A nation-wide survey of malignant high-grade CNS tumors (World Health Organization grade III/IV), diagnosed in children (aged birth to 14 years) from 1996 to 2006 was conducted by the Austrian Brain Tumor Registry. A central histopathology review was performed including the assessment of SMARCB1 (INI1) protein status. RESULTS: A total of 311 newly diagnosed, malignant CNS tumors were included. Atypical teratoid/rhabdoid tumors constituted the sixth most common entity (6.1%), referring to an age-standardized incidence rate of 1.38 per 1,000,000 person-years in children. Peak incidence was found in the birth to 2 years age group, where they were as common as CNS primitive neuroectodermal tumors and medulloblastomas. A total of 47.4% of atypical teratoid/rhabdoid tumors were initially diagnosed, whereas 52.6% were retrospectively detected by the central review. The 5-year survival of atypical teratoid/rhabdoid tumor patients was 39.5%, with 66.7% in the correctly diagnosed group versus 15.0% in the not recognized group (P ¼ .0469). CONCLUSIONS: Clinicians and pathologists should be aware of the high incidence of atypical teratoid/rhabdoid tumors in young children to optimize diagnostic and therapeutic management of patients with these tumors. Cancer 2010;116:5725-32. V C 2010 American Cancer Society.KEYWORDS: atypical teratoid/rhabdoid tumor, central nervous system neoplasm, childhood, epidemiology, population-based incidence, survival.Central nervous system (CNS) tumors represent >20% of all childhood malignancies (0-14 years) in developed countries 1,2 and constitute the most common cause of cancer-related death in this age group.

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Primary rhabdoid tumor of the brain in an adult

Cited by 30 publications

References 17 publications

Atypical Teratoid/Rhabdoid Tumors in Adult Patients: CT and MR Imaging Features

Atypical Teratoid/Rhabdoid Tumors in Adult Patients: CT and MR Imaging Features

Malignant Brain Tumor With Rhabdoid Features in an Adult -Case Report-

Incidence of atypical teratoid/rhabdoid tumors in children

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