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一名结节病患者的鞍区黄色肉芽肿。

Xanthogranuloma of the sellar region in a patient with sarcoidosis.

作者信息

Sulentić Petra, Cupić Hrvoje, Cerina Vatroslav, Vrkljan Milan

机构信息

Department of Clinical Neuroendocrinology and Pituitary Diseases, University Department of Endocrinology, Diabetes and Metabolic Diseases, Sestre milosrdnice University Hospital, Zagreb, Croatia.

出版信息

Acta Clin Croat. 2010 Mar;49(1):61-5.

PMID:20635586
Abstract

Xanthogranuloma of the sellar region is a very rare brain tumor with favorable prognosis and without reported relapses of purely xanthogranulomatous lesion after complete resection. A case is presented of a 40-year-old male diagnosed with and treated for sarcoidosis, complaining of headache, photophobia and loss of libido. Physical examination revealed generally scarce hairiness, while laboratory investigations showed panhypopituitarism. Expansive sellar and suprasellar mass compressing the floor of the third ventricle and optical chiasm was confirmed by cranial multi-slice computerized tomography (MSCT). Complete resection of the tumor mass using trans-sphenoidal approach was performed. Histopathologic analysis revealed cholesterol clefts, sparse lymphoplasmacellular infiltrates, macrophages, siderophages and foreign body giant cells around cholesterol clefts confirming the diagnosis of xanthogranuloma of the sellar region. Since preoperative diagnosis of xanthogranuloma is very difficult, therapeutic algorithm does not differ from other expansive lesions of the sellar region, but pituitary involvement should always be considered in patient with sarcoidosis since therapeutic management is non-surgical. Follow up MSCT imaging after 6 months revealed a solid, contrast-enhanced mass at the posterior base of the sella.

摘要

鞍区黄色肉芽肿是一种非常罕见的脑肿瘤,预后良好,完全切除后纯黄色肉芽肿性病变未见复发报道。本文报告一例40岁男性,曾被诊断为结节病并接受治疗,现主诉头痛、畏光和性欲减退。体格检查发现毛发普遍稀少,实验室检查显示全垂体功能减退。头颅多层计算机断层扫描(MSCT)证实鞍区及鞍上有占位性肿块,压迫第三脑室底部和视交叉。采用经蝶窦入路对肿瘤肿块进行了完整切除。组织病理学分析显示胆固醇裂隙、稀疏的淋巴细胞和浆细胞浸润、巨噬细胞、含铁血黄素巨噬细胞以及胆固醇裂隙周围的异物巨细胞,证实为鞍区黄色肉芽肿。由于鞍区黄色肉芽肿的术前诊断非常困难,其治疗方案与鞍区其他占位性病变并无不同,但结节病患者应始终考虑垂体受累情况,因为治疗方法并非手术治疗。6个月后的随访MSCT成像显示蝶鞍后底部有一个实性、强化的肿块。

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