Sellar Atypical Teratoid/Rhabdoid Tumor: Any Preoperative Diagnostic Clues?

ABSTRACT Objective: Our objectives were to describe a rare adult-onset sellar atypical teratoid/rhabdoid tumor (AT/RT) and to identify preoperative diagnostic clues for this aggressive tumor. Methods: A case report and review of the relevant literature. Results: A 36-year-old female presented with headache for 1 month and blurry vision for 6 days. Imaging identified a large sellar mass with suprasellar extension that was indistinguishable from a pituitary adenoma. Hormonal studies revealed hypopituitarism and hyperprolactinemia. A presumptive diagnosis of pituitary macroadenoma was made, but the histologic, immunohistochemical, electron microscopic, and cytogenetic studies of the tumor supported a diagnosis of AT/RT. Despite multiple surgical resections, radiation, and chemotherapy, the tumor recurred and regrew relentlessly. The patient died 2.5 years after presentation. Conclusion: This case highlights the importance of suspecting aggressive sellar masses in management planning and prognosis counseling. Short duration of headache and visual changes associated with a large sellar mass are diagnostic clues for sellar AT/RT or other rapidly expanding sellar masses. Endocrinologists should pay attention to symptom duration in patients with large sellar masses. Abbreviations: AT/RT atypical teratoid/rhabdoid tumor INI1 integrase interactor 1 MRI magnetic resonance imaging