Intracranial Atypical Teratoid/Rhabdoid Tumor: Retrospective Analysis in a Single Institution

Kim, Eun Kyung; Park, Jeong Ah; Yoon, Jong Hyung; Lee, Soo Hyun; Kang, Hyoung Jin; Shin, Hee Young; Ahn, Hyo Seop

Abstract

PURPOSE
Intracranial atypical teratoid/rhabdoid tumor (ATRT) is an extremely rare and aggressive tumor of early childhood. In this study, we evaluated the clinical characteristics, therapeutic approaches and outcomes of children who were treated at Seoul National University Children's Hospital (SNUCH). METHODS: We reviewed the clinical records of seven patients who were diagnosed as ATRT at SNUCH between January 2000 and July 2005. RESULTS: Of the seven patients, three patients were male and four were female. Median age at diagnosis was 13 months ranging from 3 months to 67 months. The tumors occurred in the infratentorial area in six and at multiple sites in one patient. Metastatic disease at diagnosis was present in two patients. One showed cerebrospinal fluid (CSF) dissemination and the other showed bony metastasis. Tumor excision was performed in all patients, and with the exception of two cases that refused further treatment, five patients received postoperative chemotherpay. One patient with CSF dissemination received radiotherapy and intrathecal chemotherapy as well. Of all the patients who received chemotherapy, two patients died during treatment because of tumor progression. The chemotherapy regimen was changed in three patients during treatment because the tumor showed poor response to chemotherapy. The median length of follow-up for five patients receiving chemotherapy was 6 months and the overall survival (OS) and event free survival (EFS) were 33.3% and 0%, respectively. CONCLUSION: Though various therapeutic approaches have led to improved survival in ATRT, the prognosis of ATRT is dismal compared with other brain tumors. A precise pathologic diagnosis is crucial and intensified treatment modalities should be considered according to the extent of tumor. To establish optimal treatment guidelines, a cooperative prospective study is needed and the efficacy of individual regimens should be analyzed.