J Korean Med Sci.  2002 Oct;17(5):723-726. 10.3346/jkms.2002.17.5.723.

Primary Intracranial Atypical Teratoid/Rhabdoid Tumor in a Child: A Case Report

• Chung YN
• Wang KC
• Shin SH
• Kim NR
• Chi JG
• Min KS
• Cho BK

• KMID: 1127273
• DOI: http://doi.org/10.3346/jkms.2002.17.5.723

Abstract

Rhabdoid tumors of the central nervous system are rare malignancies. Primary central nervous system atypical teratoid/rhabdoid tumors (ATT/RhTs) mostly occur during early childhood and are almost invariably fatal. These tumors show similar histological and radiological features to primitive neuroectodermal tumor-medulloblastoma (PNET-MB) but have different biological behaviors. We report a case of primary intracranial ATT/RhT in the posterior cranial fossa of a child. Preoperative radiological diagnosis was PNET-MB, but pathological diagnosis is ATT/ RhT. The case involved a 16-month-old baby boy who presented with severe headache, vomiting, and gait disturbance. He was treated by surgical resection, chemotherapy, and radiotherapy. Despite aggressive therapy, he died 19 months after diagnosis. Clinical, radiological, and histopathological features of primary intracranial ATT/RhT are discussed with a special emphasis on the differential diagnosis from PNET-MB.

Keyword

Atypical Teratoid/Rhabdoid Tumor; Primitive Neuroectodermal Tumor; Medulloblastoma; Child

MeSH Terms

Brain Neoplasms/*diagnosis/pathology
Diagnosis, Differential
Humans
Infant
Magnetic Resonance Imaging
Male
Medulloblastoma/diagnosis
Neuroectodermal Tumors, Primitive/diagnosis
Rhabdoid Tumor/*diagnosis/pathology
Teratoma/*diagnosis/pathology
Tomography, X-Ray Computed