Department of Neurosurgery, University of Tuebingen, 72076, Tuebingen, Germany.
Pituitary. 2012 Dec;15 Suppl 1:S10-6. doi: 10.1007/s11102-010-0262-3.
Pituicytoma is an exceptionally rare low-grade glioma (WHO grade I) of the neurohypophysis and infundibulum. We are reporting the case of a 48-year-old man who presented with severe Cushing's syndrome. Endocrinological evaluation unequivocally confirmed pituitary-dependent Cushing's syndrome (=Cushing's disease). Cranial MR-imaging displayed a conspicuous area in the dorsal and basal pituitary gland and a minimal bulging of the pituitary gland paramedian of the pituitary stalk on the right side. Transsphenoidal inspection revealed a small tumor in the basal and dorsal pituitary gland. Surprisingly, the definite postoperative histopathological diagnosis of the removed tumor was pituicytoma and not pituitary adenoma. Hence, the microadenoma responsible for Cushing's disease was not yet removed and persistent hypercortisolism necessitated transsphenoidal re-operation. During re-operation, hemihypophysectomy was performed on the right side. The non-tumorous specimen of the adeno-hypophysis showed signs of Crooke's hyalinization consistent with Cushing's disease. Undetectable postoperative ACTH- and cortisol levels provided clear evidence that the underlying ACTH-source was successfully removed during re-operation. Coincidence of pituicytoma and pituitary-dependent Cushing's disease has not previously been reported.
垂体细胞瘤是一种极其罕见的低级别神经垂体和漏斗胶质瘤（WHO 分级 I）。我们报告了一例 48 岁男性，表现为严重库欣综合征。内分泌评估明确证实了垂体依赖性库欣综合征（=库欣病）。颅磁共振成像显示在垂体的背侧和基底部有一个明显的区域，在垂体柄右侧的垂体旁有一个微小的隆起。经蝶窦检查显示在垂体的基底部和背侧有一个小肿瘤。令人惊讶的是，切除肿瘤的明确术后组织病理学诊断是垂体细胞瘤，而不是垂体腺瘤。因此，导致库欣病的微腺瘤尚未被切除，持续的皮质醇增多症需要经蝶窦再次手术。在再次手术中，对右侧进行了半垂体切除术。非肿瘤性的腺垂体标本显示与库欣病一致的 Crooke 透明变性的迹象。术后 ACTH 和皮质醇水平无法检测到，这清楚地表明在再次手术中成功切除了潜在的 ACTH 来源。垂体细胞瘤和垂体依赖性库欣病的同时发生以前没有报道过。
