Geddes J F, Jansen G H, Robinson S F, Gömöri E, Holton J L, Monson J P, Besser G M, Révész T
Department of Morbid Anatomy, St. Bartholomew's and the Royal London School of Medicine and Dentistry, UK.
Am J Surg Pathol. 2000 Apr;24(4):607-13. doi: 10.1097/00000478-200004000-00017.
Hamartomatous or neoplastic ganglion cells in the sella turcica are an unusual cause of symptoms. They have been reported in association with a functioning or nonfunctioning pituitary adenoma, with pituitary cell hyperplasia, and occasionally as masses unassociated with an adenoma, again with variable endocrinologic findings. Fewer than 50 cases of intrasellar ganglion cell lesions have been reported in the literature, only six of them associated with Cushing's syndrome. We describe the clinicopathologic features of another eight patients, three of whom presented with acromegaly, four with apparently nonfunctioning adenohypophyseal masses, and one with Cushing's syndrome. On histology, six of them were found to have sparsely granulated growth hormone (GH)-producing adenomas with ganglion cell areas, one appeared to have a gangliocytoma not associated with an adenoma, whereas the eighth had a ganglion cell lesion in the posterior pituitary. The morphologic and immunohistochemical findings suggest that the ganglion cell component of seven of these tumors has resulted from neuronal differentiation in a GH-producing adenoma, despite the lack of demonstrable adenoma in one case. A true sellar "gangliocytoma" or hamartoma of ectopic hypothalamic-type neurons appears to be a rarer explanation for the presence of ganglion cells in a pituitary biopsy.
蝶鞍内的错构瘤性或肿瘤性神经节细胞是症状的不寻常病因。它们曾被报道与功能性或无功能性垂体腺瘤、垂体细胞增生相关,偶尔也作为与腺瘤无关的肿块出现,内分泌学表现也各不相同。文献中报道的蝶鞍内神经节细胞病变病例少于50例,其中仅6例与库欣综合征相关。我们描述了另外8例患者的临床病理特征,其中3例表现为肢端肥大症,4例表现为明显无功能的腺垂体肿块,1例表现为库欣综合征。组织学检查发现,其中6例为稀疏颗粒型生长激素(GH)分泌腺瘤伴神经节细胞区域,1例似乎为与腺瘤无关的神经节细胞瘤,而第8例在后叶有神经节细胞病变。形态学和免疫组化结果表明,尽管其中1例未发现明显腺瘤,但这些肿瘤中有7例的神经节细胞成分是由分泌GH的腺瘤中的神经元分化形成的。真正的蝶鞍“神经节细胞瘤”或异位下丘脑型神经元错构瘤似乎是垂体活检中出现神经节细胞的较罕见原因。