J Korean Child Neurol Soc.  2004 Nov;12(2):235-240.

A Case of an Atypical Teratoid/Rhabdoid Tumor of the Cerebellum

• Kim KS
• Jung CG
• Kim YM
• Ko KO
• Lee YH

Abstract

Primary central nervous system atypical teratoid/rhabdoid tumors are rare and extremely aggressive malignancies of early childhood. These tumors are most common in infants less than 2 years of age. Diagnosis is based on distinctive light microscopic and immunohistochemical findings, coupled with a molecular genetic analysis. A histologic features of these tumors are epithelial and/or mesenchymal components in addition to rhabdoid, with or without neuroepithelial fields. The expression of the epithelial membrane antigen, vimentin, and the smooth muscle actin are characteristic of these tumors. Treatment includes surgery, chemotherapy, and radiotherapy. Prognosis is poor despite of an aggressive therapy. We report one case of an atypical teratoid/rhabdoid tumor in a young child. She is an 18 month-old-girl who presented with central type facial palsy. After extensive surgery she improved transiently but relapsed immediately. Her condition was not permitted to receive radiotherapy or chemotherapy. Thereafter, with phrenic nerve palsy, she suffered from recurrent episodes of pneumonia and respiratory difficulties. Finaly, she was expired three months after the diagnosis and treatment.

Keyword

Atypical teratoid/rhabdoid tumor

MeSH Terms

Actins
Central Nervous System
Cerebellum*
Child
Diagnosis
Drug Therapy
Facial Paralysis
Humans
Infant
Molecular Biology
Mucin-1
Muscle, Smooth
Paralysis
Phrenic Nerve
Pneumonia
Prognosis
Radiotherapy
Vimentin
Actins
Mucin-1
Vimentin