Yonsei Med J.  2001 Feb;42(1):142-146. 10.3349/ymj.2001.42.1.142.

Classical malignant rhabdoid tumor of central nervous system in 9-year-old Korean

Affiliations
  • 1Department of Pathology, Medical College of Kosin University, Pusan, Korea. changhkg@ns.kosinmed.or.kr
  • 2Department of Pathology, Yale University School of Medicine, New Haven, Connecticut, USA.

Abstract

A Malignant rhabdoid tumor (MRT) arising in the right temporoparietal lobe of a 9-year-old boy is described along with the results of an immunohistochemical study. The patient initially sought medical attention for a ptosis and right sided headache. The child underwent a subtotal resection of the tumor, followed by radiotherapy and systemic chemotherapy, but died three years after surgery. A MRT, a primary neoplasm of the central nervous system (CNS), is an entity of unknown histogenesis with a dismal prognosis, which only occurs in early childhood. Histologically similar tumors with more varied morphological features have been designated as an atypical teratoid/rhabdoid tumor. However, a classical MRT is extremely rare in the CNS and our case represents a classical CNS MRT.

Keyword

malignant rhabdoid tumor; atypical teratoid/rhabdoid tumor; central nervous system; immunohistochemistry; 9-year-old boy

MeSH Terms

Brain Neoplasms/therapy
Brain Neoplasms/pathology
Brain Neoplasms/metabolism*
Case Report
Child
Glial Fibrillary Acidic Protein/analysis
Human
Immunohistochemistry
Male
Rhabdoid Tumor/therapy
Rhabdoid Tumor/pathology
Rhabdoid Tumor/metabolism*
Vimentin/analysis
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