Extraventricular neurocytoma at the sellar region: Report of 8 cases and literature review

Abstract

BackgroundExtraventricular neurocytoma at the sellar region (EVNSR) is an exceedingly rare tumor. Given the paucity of specificity and its peculiar nature, our study aimed to characterize the clinical, imaging, and pathological features, including treatment and clinical outcomes of this tumor. MethodEight patients with pathologically confirmed EVNSR at Beijing Tiantan Hospital from 2012 to 2020 were retrospectively analyzed. Additionally, 7 cases from the prior detailed literature were also retrieved. FindingsAmong the 8 patients from Beijing Tiantan Hospital, 2 males and 6 females with an average age of 45.3 (range, 8–61). Vision impairment and headache were the most common complaints at presentation. Preoperative diagnoses were pituitary adenoma (n = 6), meningioma (n = 1), and oligodendroglioma (n = 1). Treatment included subtotal tumor resection (n = 3), partial resection (n = 5), adjuvant therapy covered radiotherapy (n = 2), and gamma knife surgery (n = 3). The clinical outcomes of these 8 cases were stable (n = 5), survival after progression (n = 1), and death after recurrence (n = 2). ConclusionsEVNSRs are extremely rare tumors, and most have benign prognoses after appropriate treatment. Due to the unique location, total removal of the tumor is challenging; And adjuvant radiation therapy for eligible patients is recommended. Regular imaging review is also advised. Future studies with more patients are needed to elucidate the biological nature of EVNSRs.