All issues > Volume 44(12); 2001

Case Report

J Korean Pediatr Soc. 2001;44(12):1475-1479. Published online December 15, 2001.

A Case of Central Nervous System Atypical Teratoid/Rhabdoid Tumor of The 4th Ventricle : A Highly Malignant Tumor in Infancy and Childhood Frequently Mistaken for Medulloblastoma

So Hee SH Eun¹, Sung Hee SH Jang¹, Myoung Hee MH Han¹, Myoung Ja MJ Chung², Pyoung Han PH Hwang¹

¹Department of Pediatrics, College of Medicine, Chonbuk National University, Chonju, Korea
²Department of Pathology, College of Medicine, Chonbuk National University, Chonju, Korea

Correspondence Pyoung Han PH Hwang ,Email: hwaph@moak.chonbuk.ac.kr

Abstract

Central nervous system atypical teratoid/rhabdoid tumor(CNS rhabdoid tumor) is a rare malignancy of uncertain origin. It typically occurs in infants and young children and comprises only a small fraction of pediatrics CNS malignancies. The tumor contains a large spindled cell component as classical rhabdoid morphology and focal areas resembling primitive neuroectodermal tumor. The tumor is defined histopathologically by the presence of rhabdoid cells, but contains considerable heterogeneity of the cell type, including the frequent presence of primitive neuroectodermal tumor. The prognosis for children with CNS rhabdoid tumor is dismal. We experienced a case of a three-year-old female who had been transferred to our hospital for seizure and vomiting. She was diagnosed as CNS atypical teratoid/rhabdoid tumor by biopsy. In spite of surgery and intensive postoperative multi-agents chemotherapy, she developed a local recurrence around the operation site at six months after surgery. We present this case with a brief review of related literatures.

Keywords :CNS atypical teratoid/rhabdoid tumor