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. The sex, age, sellar compression symptoms, MR imaging characteristics, and pituitary hormone levels of the 3 patients from our institute and the 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. 53 (68%) patients suffered from headaches, 11 (14%) of whom experienced severe headaches or progressive headaches. 41 (53%) patients presented with ophthalmoplegia, manifesting symptoms such as ptosis and diplopia. MR imaging revealed that 42 (54%) patients had sellar masses invading the cavernous sinus (CS), with 11 (14%) cases showing bilateral CS 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 MR imaging characteristics provide crucial clues for the preoperative presumptive diagnosis of this disease.
Wu et al. (Wed,) studied this question.