Department of Neurosurgery, Tokyo Women's Medical University, Kawada-cho 8-1 Shinjyuku-ku, Tokyo, 162-8666, Japan,
Brain Tumor Pathol. 2013 Oct;30(4):233-41. doi: 10.1007/s10014-012-0130-0. Epub 2013 Jan 16.
Xanthogranuloma of the sellar region is a rare clinical observation. Although it was included in the World Health Organization (WHO) brain tumor classification in 2000, its clinical features and pathogenesis remain uncertain. We report herein seven cases of xanthogranuloma of the sellar region who underwent transsphenoidal surgery at Tokyo Women's Medical University between 2005 and 2011, and discuss the clinical characteristics of this tumor. Six out of these 7 patients (86 %) presented with endocrinological dysfunction, six (86 %) had headaches, five (71 %) had visual field disturbances, and three (43 %) had diplopia including two with unilateral ptosis. Pathological findings revealed cholesterol clefts, hemosiderin deposits, chronic inflammatory infiltrates, multinucleated giant cells, macrophages, and fibrous proliferation. Of these seven cases, components of Rathke's cleft cyst were observed for six. Xanthogranuloma in the sellar region is suspected of being a terminal stage resulting from a secondary reaction caused by repeated inflammatory change, hemorrhage, and degeneration of a Rathke's cleft cyst.
鞍区黄色肉芽肿是一种罕见的临床观察。尽管它在 2000 年被纳入世界卫生组织(WHO)的脑肿瘤分类,但它的临床特征和发病机制仍不确定。我们在此报告了 7 例在东京女子医科大学接受经蝶窦手术的鞍区黄色肉芽肿患者,讨论了该肿瘤的临床特征。这 7 例患者中,有 6 例(86%)存在内分泌功能障碍,6 例(86%)有头痛,5 例(71%)有视野障碍,3 例(43%)有复视,其中 2 例伴有单侧上睑下垂。病理检查显示胆固醇裂隙、含铁血黄素沉积、慢性炎症浸润、多核巨细胞、巨噬细胞和纤维增生。这 7 例中,有 6 例观察到 Rathke 裂隙囊肿的成分。鞍区黄色肉芽肿可能是 Rathke 裂隙囊肿反复炎症变化、出血和退变引起的继发性反应的终末阶段。