Pituitary Atypical Teratoid Rhabdoid Tumor in a Patient with MEN-1 Syndrome and Prolactinoma: A Case Report

Miranda Stiewig-Rapp; Camille Andre; Dana Sheely

doi:10.53941/ijctm.2025.1000029

Abstract

Atypical teratoid/rhabdoid tumor (AT/RT) is a rare, aggressive central nervous system (CNS) neoplasm, typically diagnosed in children, with fewer than 100 adult cases reported. Pituitary prolactinomas, more common in adults, have been rarely associated with AT/RT, particularly in the context of multiple endocrine neoplasia type 1 (MEN-1) syndrome. We present a 38-year-old female with MEN-1 syndrome, recurrent pituitary prolactinoma, and AT/RT. She presented with altered mental status, headache, and seizure-like activity. CT imaging revealed a residual invasive pituitary tumor with significant hemorrhaging. Post-operative pathology confirmed the presence of both a pituitary prolactinoma and AT/RT. Molecular analysis of the AT/RT demonstrated a loss of INI-1 and a homozygous deletion of CDKN2A/2B. The patient was re-admitted a month later with signs of aggressive tumor recurrence and transitioned to hospice care. This case is one of only two documented instances of concurrent pituitary prolactinoma and AT/RT, and it is further complicated by the patient’s MEN-1 syndrome. This report highlights the rare occurrence of AT/RT in adults and its potential association with prior radiation therapy for pituitary adenomas, raising questions about the impact of MEN-1 syndrome on AT/RT pathogenesis.

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