A rare astrocytic tumor with rhabdoid features

Nagai, Shoichi; Kurimoto, Masanori; Ishizawa, Shin; Hayashi, Nakamasa; Hamada, Hideo; Kamiyama, Hironaga; Endo, Shunro

doi:10.1007/s10014-008-0241-9

Cited by 16 publications

(32 citation statements)

References 17 publications

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“…15 The immunohistochemical profile of rhabdoid GB is varied, with GFAP, EMA, vimentin, and neurofilament being common markers. 9,10,17,[19][20][21]32 Positive immunohistochemical staining of neurofilament and overexpression of vimentin offer possible markers by which to differentiate it from its parent disease, with which it shares some morphological features. 19,27 Rhabdoid GBs can be differentiated from other rhabdoid tumors of the CNS by the presence of INI-1, the hSNF5/INI-1 tumor suppressor gene product.…”

Section: Discussionmentioning

confidence: 99%

Clinicopathological characteristics and treatment of rhabdoid glioblastoma

Babu¹,

Hatef²,

McLendon³

et al. 2013

JNS

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Object Rhabdoid glioblastoma (GB) is an exceedingly rare tumor in which some of the tumor cells possess rhabdoid features such as eccentric nuclei, abundant eosinophilic cytoplasm, and pseudopapillary formations. These tumors are exceptionally aggressive, and leptomeningeal dissemination is common. In the 9 previously reported cases, the longest survival was only 9 months, with a median survival of 17.8 weeks. The authors report the clinicopathological characteristics of 4 cases of rhabdoid GB and demonstrate the utility of intensive temozolomide and adjuvant therapy in these tumors. The authors also review the literature to provide the most comprehensive understanding of these rare tumors to date. Methods A retrospective review was performed of patients treated for GB at the Duke University Medical Center between 2004 and 2012. One of two experienced neuropathologists identified 4 cases as being rhabdoid GBs. Immunohistochemistry and fluorescence in situ hybridization analyses were performed in all cases. Kaplan-Meier analysis was used to assess overall survival, with the log-rank test being used to evaluate differences between survival curves. An extensive review of the literature was also performed. Results The median age of patients with rhabdoid GB was 30 years. Clinical presentation varied with location, with headache being a presenting symptom in 90% of patients. All lesions were supratentorial, and 45.5% of the cases involved the temporal lobe. Leptomeningeal dissemination occurred in 63.6% of patients, with 1 patient having extracranial metastasis to the scalp and lungs. Fluorescence in situ hybridization revealed epidermal growth factor receptor gain or amplification in all study cases. The median survival in the authors' cohort was significantly higher than that of all previously reported cases (27.5 vs 4.5 months, p = 0.003). Postoperative treatment in the authors' cohort included radiotherapy with concurrent temozolomide, bevacizumab, interleukin 13, CCNU, and/or etoposide. Conclusions Enhanced survival in the authors' 4 patients suggests that the current standard of care for the treatment of GB may be beneficial in rhabdoid GB cases, with postoperative radiotherapy and concomitant temozolomide treatment followed by adjuvant therapy. Due to the rapid tumor dissemination associated with these lesions, aggressive and timely therapy is warranted, with frequent surveillance and/or continued therapy despite stable disease. Additionally, patients should undergo full craniospinal imaging to monitor the development of distant metastatic disease.

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Section: Discussionmentioning

confidence: 99%

Clinicopathological characteristics and treatment of rhabdoid glioblastoma

Babu¹,

Hatef²,

McLendon³

et al. 2013

JNS

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“…Rhabdoid tumor is characterized by large, morphologically monomorphous and relatively noncohesive rhabdoid cells 1–8 . It was first described as one subtype of Wilms' tumors of the kidney, presenting with rhabdomyosarcomatoid features 1,9 .…”

Section: Introductionmentioning

confidence: 99%

“…Atypical teratoid/rhabdoid tumor (AT/RT) is a representative tumor with rhabdoid cells in the CNS, 2,3 which usually arises in young children and with a highly aggressive process. Besides AT/RT, a supratentorial rhabdoid tumor, containing regions of glioblastoma, was reported and designated as rhabdoid glioblastoma 4–8 . This tumor had a specific rhabdoid morphological feature and poor prognosis.…”

Section: Introductionmentioning

confidence: 99%

Rhabdoid glioblastoma: Case report and literature review

Wang

2010

Neuropathology

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Rhabdoid glioblastoma is a recently described entity in which a glioblastoma is associated with a rhabdoid component. Although rhabdoid glioblastoma has not appeared in the new World Health Organization classification of tumors of the CNS, it has a specific morphological feature and highly aggressive clinic process. Up to now, there have been six cases of rhabdoid glioblastoma reported in the literature. We report rhabdoid glioblastoma in the right front temporal lobe from a 31-year-old Chinese man. This tumor consisted of rhabdoid tumor cells with an eccentric nucleus and an eosinophilic cytoplasm. The tumor had an area appearing to be glioblastoma with microvascular proliferation and necrosis, and lacked a primitive neuroectodermal tumor component, and a mesenchymal component. Vimentin, epithelial membrane antigen, GFAP and integrase interactor (INI-1) expression were found in the tumor cells. Genetic abnormalities which include monosomy or a deletion of chromosome 22 were not found in this tumor. After 3 months post-surgery, the tumor was widespread in leptomeningia and the patient died. In conclusion, rhabdoid glioblastoma is a rare glioblastoma with poor prognosis; the differential diagnosis contained other rhabdoid tumors. This case will contribute to the profile of rhabdoid glioblastoma with typical morphology and immunophenotype, genetic and clinic features.

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“…Among these, AT/RT is the most frequent one, is usually found in infancy and childhood, and consists of rhabdoid, primitive neuroepithelial, and mesenchymal components [4]. rGBs are said to be more aggressive than typical GBs and have worse prognosis [5][6][7][8][9][10][11]. Diagnosis relies mainly on pathology, while imaging findings seem to be nonspecific as judged from a limited number of cases.…”

Section: Introductionmentioning

confidence: 99%

“…Pathological diagnosis is primarily histological, where immunohistochemistry and genetic testing is used to further characterize tumor and confirm the diagnosis [10]. So far, only 21 cases of rGBs have been reported as single case reports or small groups of patients [1,3,[5][6][7][8][9][10][11][12][13][14]. In these reports, clinicopathologic and genetic features have been emphasized and detailed evaluations of imaging findings have not been presented.…”

Section: Introductionmentioning

confidence: 99%