Revisiting Preoperative Diagnostic Clues for Adult Primary Sellar Atypical Teratoid/Rhabdoid Tumor: A Rare Entity Mimicking Pituitary Adenoma.

Abstract

Primary sellar atypical teratoid/rhabdoid tumor (AT/RT) is a rare, fast-growing, and aggressive embryonal tumor in adults. Awareness of potential "red flags" of this disease is important but difficult for clinicians during preoperative assessment. The clinical and radiological data of 3 adult patients diagnosed with primary sellar AT/RT at our institute were retrospectively analyzed. Additionally, the clinical characteristics of 75 individual cases of primary sellar AT/RT identified from 46 publications were reviewed. Sex, age, sellar compression symptoms, magnetic resonance imaging (MRI) characteristics, and pituitary hormone levels of 3 patients from our institute and 75 cases from the literature were analyzed (n = 78). The cohort consisted of 5 (6.4%) males and 73 (94%) females, ranging in age from 20 to 80 years, with a mean age of 47 years. Fifty-three (68%) patients suffered from headaches, 11 (14%) of whom experienced severe headaches or progressive headaches. Forty-one (53%) patients presented with ophthalmoplegia, manifesting symptoms such as ptosis and diplopia. MRI revealed that 42 (54%) patients had sellar masses invading the cavernous sinus, with 11 (14%) cases showing bilateral cavernous sinus involvement. Pituitary-related hormone abnormality was found in 36 (46%) patients, including 19 (24%) patients with hypopituitarism. Primary sellar AT/RT is an aggressive yet rare malignancy in adults. Rapidly progressive sellar compression symptoms and aggressive MRI characteristics provide crucial clues for the preoperative presumptive diagnosis of this disease.