Astroblastoma with rhabdoid features and favorable long-term outcome: report of a case with a 12-year follow-up

Fathi, Ali-Reza; Novoa, Eva; El-Koussy, Marwan; Kappeler, Andreas; Mariani, Luigi; Vajtai, Istvan (2008). Astroblastoma with rhabdoid features and favorable long-term outcome: report of a case with a 12-year follow-up. Pathology, research and practice, 204(5), pp. 345-51. München: Elsevier 10.1016/j.prp.2007.12.003

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Astroblastoma is a historically traded microscopic diagnosis to denote a rare neuroepithelial tumor of uncertain nosology, involving a distinctive pattern of pseudorosette arrangement of neoplastic cells. While displaying some glial properties, the latter shall not - by definition - be either reducible to or part of any conventional glioma type. We report on clinicopathologic correlations in a case of astroblastoma involving an extensive rhabdoid phenotype of tumor cells. The male patient was operated on at the age of 53 and 59 years for a left parietal tumor measuring 5.8 cm in diameter at the first presentation. On magnetic resonance imaging and angiography, both the primary and its recurrence were discrete, highly vascularized, and contrast-enhancing. The second surgery was complemented with radiotherapy of 66 Gy, followed by chemotherapy with Temozolomide. Twelve years into clinical history, the patient has stable minimal residual disease at the age of 65. A review of pathology samples from both surgeries showed well-differentiated astroblastoma according to current standards, with an MIB-1 labeling index of 1% and 4%, respectively. Neither of the specimens involved cellular anaplasia, overt mitotic activity, microvascular proliferation, or palisading necrosis. Most tumor cells harbored paranuclear filamentous rhabdoid inclusions that were immunostained for vimentin and, in part, also for GFAP. No polyantigenic reactivity was observed. This example contributes another facet to the spectrum of the so-called composite rhabdoid tumors. Involving a low-grade parent neoplasm, it also further substantiates the incipient perception that the rhabdoid phenotype neither is a peculiar but nonspecific convergence point of anaplastic evolution, nor are such lesions indiscriminately bound for a relentless course.

Item Type:

Journal Article (Original Article)

Division/Institute:

04 Faculty of Medicine > Department of Radiology, Neuroradiology and Nuclear Medicine (DRNN) > Institute of Diagnostic and Interventional Neuroradiology
04 Faculty of Medicine > Service Sector > Institute of Pathology

UniBE Contributor:

El-Koussy, Marwan; Kappeler, Andreas and Vajtai, Istvan

ISSN:

0344-0338

ISBN:

18280055

Publisher:

Elsevier

Language:

English

Submitter:

Factscience Import

Date Deposited:

04 Oct 2013 14:59

Last Modified:

04 May 2014 23:17

Publisher DOI:

10.1016/j.prp.2007.12.003

PubMed ID:

18280055

Web of Science ID:

000255888200008

URI:

https://boris.unibe.ch/id/eprint/25436 (FactScience: 58546)

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