Brain Tumor Research and Treatment  2014;2(2):108-113

doi:10.14791/btrt.2014.2.2.108

Adult-Onset Sellar and Suprasellar Atypical Teratoid Rhabdoid Tumor Treated with a Multimodal Approach: A Case Report.

Hae Gi PARK 1 ; Jong Hyung YOON ; Se Hoon KIM ; Kwan Ho CHO ; Hyeon Jin PARK ; Sun Ho KIM ; Eui Hyun KIM

Affiliations

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Keywords

Adult-onset; Atypical teratoid rhabdoid tumor; INI1; Sellar; Suprasellar

Country

Republic of Korea

Language

English

Abstract

We report a very rare case of sellar and suprasellar atypical teratoid rhabdoid tumor (ATRT) in a 42-year-old female patient. The tumor was removed subtotally with a transsphenoidal approach. Histopathologic study showed rhabdoid cells with prominent nucleoli and abundant cytoplasm. Immunohistochemistry for INI1 was completely negative in the tumor cells, consistent with ATRT. After surgery, she received radiotherapy including spinal irradiation with proton beam therapy and subsequent chemotherapy, with no evidence of recurrence for more than 2 years. Up to date, this is the 8th case of an adult-onset ATRT in the sellar or suprasellar region. Despite its rarity, ATRTs should be considered in the differential diagnosis of an unclear malignant sellar or suprasellar lesion in adult patients and the treatment strategies for adult ATRT patients could be differentiated from those of pediatric ATRT patients.