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神经节细胞瘤:一种罕见的无症状垂体肿瘤的预后

Gangliocytoma: outcome of a rare silent pituitary tumour.

作者信息

Donadille Bruno, Villa Chiara, Gaillard Stephan, Christin-Maitre Sophie

机构信息

Department of Endocrinology, Assistance Publique-Hopital Saint Antoine, Hopitaux de Paris, Paris, France.

Department of Pathological Cytology and Anatomy, Hopital Foch, Suresnes, Île-de-France, France.

出版信息

BMJ Case Rep. 2017 Feb 23;2017:bcr2016218859. doi: 10.1136/bcr-2016-218859.

Abstract

The most common finding in front of a pituitary incidentaloma is a silent pituitary adenoma. We describe a 59 years old woman with a pituitary gangliocytoma and her follow-up after 1 year. Hormonal exploration only evidenced partial corticotropic insufficiency. A trans-sphenoidal surgery was performed due to the tumour's suprasellar expansion. Gangliocytoma is a benign tumour of unknown prevalence, belonging to central nervous system tumour with neuronal differentiation, and 129 cases have been reported in the literature. growth hormone (GH), adrenocorticotropic hormone (ACTH) or prolactin secretions have been reported, as these ganglion cell-like mature neurons are usually mixed with secreting pituitary endocrine cells. We report a case with a pure gangliocytoma devoid of symptomatic endocrine secretion, not surrounded by pituitary endocrine tumour cells. Immunochemistry of the tumour was positive for hypothalamic growth hormone releasing hormone (GHRH) and pituitary hormones, such as GH and ACTH. Hence, this immunoexpression was not associated with peripheral hormonal secretions, suggesting biologically inactive hypothalamopituitary hormones.

摘要

垂体意外瘤最常见的病因是无功能垂体腺瘤。我们报告一例59岁患有垂体神经节细胞瘤的女性患者及其1年的随访情况。激素检查仅显示部分促肾上腺皮质激素功能不全。由于肿瘤向鞍上扩展,遂行经蝶窦手术。神经节细胞瘤是一种患病率未知的良性肿瘤,属于具有神经元分化的中枢神经系统肿瘤,文献中已报道129例。已有生长激素(GH)、促肾上腺皮质激素(ACTH)或催乳素分泌的报道,因为这些神经节样成熟神经元通常与分泌性垂体内分泌细胞混合存在。我们报告一例纯神经节细胞瘤病例,无内分泌分泌症状,且不被垂体内分泌肿瘤细胞包绕。肿瘤免疫组化显示下丘脑生长激素释放激素(GHRH)以及垂体激素如GH和ACTH呈阳性。因此,这种免疫表达与外周激素分泌无关,提示下丘脑 - 垂体激素无生物学活性。

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