A rare case of atypical teratoid rhabdoid tumor at the sellar region in an adult: Case report and review of literature.

INTRODUCTION

Atypical teratoid/rhabdoid tumor (AT/RT) of Central Nervous System (CNS) is a rare malignancy, usually confronted in childhood. Although few cases were reported in adults, it seems that there is a preference in supratentorial areas and specifically at the sellar region in middle-aged females with this subgroup presenting distinct features.

RESEARCH QUESTION

We share a case report of a rare intracranial ATRT in the sellar area with lung metastasis in an adult female.

MATERIAL/METHODS: Patient's medical records (laboratory tests, radiology examinations, histopathology report) were reviewed after retrieving an informed consent. A literature search within Pubmed and further "snowballing" was performed with the use of keywords "sellar", "adult", "ATRT" to address the current literature.

RESULTS

We present the case of a 51-year-old woman with headaches and left ptosis, diagnosed with a sellar mass, infiltrating the cavernous sinus. She underwent endoscopic transsphenoidal debulking of the lesion. The pathology report showed an aggressive AT/RT and the patient received radio- and chemo-therapy. On follow up imaging studies, lung metastases were shown and the patient died 7 months after the initial diagnosis.

DISCUSSION & CONCLUSION: Only a few cases of sellar/suprasellar ATRT with lung metastases have been described so far. ATRT should be in the differential diagnosis of fibrous sellar masses in adult women.