Intracranial desmoplastic small-cell tumor: Report of a case Journal Article


Authors: Tison, V.; Cerasoli, S.; Morigi, F.; Ladanyi, M.; Gerald, W. L.; Rosai, J.
Article Title: Intracranial desmoplastic small-cell tumor: Report of a case
Abstract: We report a case of desmoplastic small-cell tumor occurring in the CNS in relation to the tentorium in a 24-year-old man. Morphologically, the neoplasm had the typical appearance of small, round tumor cells of primitive appearance growing as well-defined nests separated by abundant desmoplastic stroma. The diagnosis was confirmed through the demonstration of immunoreactivity for keratin, desmin, and neuron-specific enolase and the detection by Southern blot analysis of a unique gene resulting from the fusion of the WT1 gene in chromosome 11 and the EWS gene in chromosome 22. This is the first documented instance of the occurrence of this tumor type at a distance from a mesothelial-lined surface.
Keywords: immunohistochemistry; adult; human tissue; case report; cisplatin; methotrexate; brain tumor; brain neoplasms; magnetic resonance imaging; tumor localization; etoposide; gene product; cloning, molecular; sarcoma, ewing's; chromosome 11; desmin; small cell carcinoma; neuron specific enolase; chromosome 22; cns; keratin; wilms tumor; 1 (2 chloroethyl) 3 (2,6 dioxo 3 piperidyl) 1 nitrosourea; humans; prognosis; human; male; article; intrathecal drug administration; desmoplastic small-cell tumor; tentorium; cerebellum tentorium
Journal Title: American Journal of Surgical Pathology
Volume: 20
Issue: 1
ISSN: 0147-5185
Publisher: Lippincott Williams & Wilkins  
Date Published: 1996-01-01
Start Page: 112
End Page: 117
Language: English
DOI: 10.1097/00000478-199601000-00013
PUBMED: 8540602
PROVIDER: scopus
DOI/URL:
Notes: Article -- Export Date: 22 November 2017 -- Source: Scopus
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  1. Juan Rosai
    181 Rosai
  2. William L Gerald
    375 Gerald
  3. Marc Ladanyi
    1326 Ladanyi