Gangliocytomas in the sellar region

Abstract

Gangliocytomas occurring in the sellar region are extremely rare. We examined a cohort of these tumors to examine their clinical presentations and prognoses. Between January 2000 and December 2012, 23 patients were diagnosed with sellar region gangliocytomas in Huashan Hospital. These patients were retrospectively reviewed for medical histories, endocrinological examinations, preoperative magnetic resonance imaging (MRI), pathological findings and follow-ups. Endocrinological tests revealed elevated prolactin (PRL) levels in 10 cases (43.5%) and elevated growth hormone (GH) and insulin-like growth factor 1 (IGF-1) levels in 9 cases (39.1%). Scattered ganglion cells admixed with adenomatous components were observed in 16 cases (69.6%). In the remaining 7 cases (30.4%), only fragments with ganglion cells dispersed in the fibrillar matrix without adenohypophyseal components were detected. Immunohistochemistry revealed PRL-positive adenomas in 6 cases (26.1%) and GH-positive adenomas in 8 cases (34.8%). The average follow-up period was 4.2 years (range: 1–12.7 years). Gross total resection was achieved in 20 cases (87.0%). One patient recurred five years after tumor resection (4.3%). One patient died of acute myocardial infarction six years after operation. Gangliocytomas located in the sellar region may represent a unique immunopathological entity. The surgical results and prognoses of the gangliocytomas were comparable with those of pituitary adenomas.