J Korean Pediatr Soc.  2001 Dec;44(12):1475-1479.

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

• Eun SH
• Jang SH
• Han MH
• Chung MJ
• Hwang PH

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.

Keyword

CNS atypical teratoid/rhabdoid tumor

MeSH Terms

Biopsy
Cellular Structures
Central Nervous System*
Child
Drug Therapy
Female
Humans
Infant
Medulloblastoma*
Neuroectodermal Tumors, Primitive
Pediatrics
Population Characteristics
Prognosis
Recurrence
Rhabdoid Tumor
Seizures
Vomiting