Kong, XiangyiLi, DongmeiKong, YanguoZhong, Dingrong2017-03-282017Kong, Xiangyi, Dongmei Li, Yanguo Kong, and Dingrong Zhong. 2017. “Malignant adenohypophysis spindle cell oncocytoma with repeating recurrences and a high Ki-67 index.” Medicine 96 (4): e5657. doi:10.1097/MD.0000000000005657. http://dx.doi.org/10.1097/MD.0000000000005657.http://nrs.harvard.edu/urn-3:HUL.InstRepos:31731769Abstract Adenohypophysis spindle cell oncocytoma (ASCO) is a rare tumor recently reported by Roncaroli et al in 2002. This tumor is considered a grade I tumor by the World Health Organization. We report a rare case of malignant ASCO with repeating recurrences and a high Ki-67 index—a challenging diagnosis guided by clinical presentations, radiological signs, and postoperative pathological tests. We represent a 30-year-old man who had suffered from headaches, diplopia, and impaired visual field and acuity. His magnetic resonance imaging revealed an abnormal sellar mass and was originally misdiagnosed as a pituitary macroadenoma. We present detailed analysis of the patient's disease course and review relevant literature. When surgically treated, the specimen revealed a typical histopathology pattern of ASCO. The tumor recurred for several times and the patient underwent 3 surgeries and 1 γ-knife treatment, which was accompanied by a continuously increasing Ki-67 index. This is the first reported case of malignant ASCO (WHO III–IV grade). Despite its rarity, ASCO should be considered in the differential diagnosis of sellar lesions that mimic pituitary adenomas.en-USObservational Studyadenohypophysis spindle cell oncocytomaKi-67 indexmalignantrecurrentJournal Article2017-03-2810.1097/MD.0000000000005657