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[鞍内神经节细胞瘤合并生长激素分泌型垂体腺瘤。病例报告]

[Intrasellar gangliocytoma associated with growth hormone-producing pituitary adenoma. Case report].

作者信息

Romero Lorena, Arcos Andrea, Bautista María Dolores, Domínguez Miguel, Medina Juan M, Arráez Miguel A

机构信息

Servicio de Neurocirugía, Hospital Regional Universitario Carlos Haya, Málaga, España.

出版信息

Neurocirugia (Astur). 2012 Nov;23(6):264-9. doi: 10.1016/j.neucir.2012.05.002. Epub 2012 Sep 7.

Abstract

INTRODUCTION

Intrasellar gangliocytomas are uncommon entities which, unusually, may be found in association with hormone-releasing pituitary adenomas.

CASE REPORT

The patient was a 49-year-old female who presented a sellar lesion with associated acromegaly. A trans-sphenoidal tumour was removed, with no medical improvement. Histopathological analysis revealed a gangliocytoma with an associated somatotroph adenoma.

DISCUSSION

We found 85 cases of intrasellar gangliocytomas with associated hormone-releasing pituitary adenomas reported in the literature, with the following distribution: 50 growth hormone-releasing (GH) cases (59%), 15 mixed (GH and prolactin-releasing) cases (17%), 11 prolactin-releasing cases (13%), 7 adrenocorticotropic hormone-releasing (ACTH) cases (8%) and 2 corticotropin hormone-releasing (CRH) cases (2%).

CONCLUSIONS

Mixed gangliocytomas-adenomas are uncommon entities. Association with growth hormone-releasing cases is more frequent and the most common presentation is among middle-aged females. Diagnosis is histopathological. Identification of this entity is important because it may lead to a limitation in therapeutic response in incomplete resections.

摘要

引言

鞍内神经节细胞瘤是一种罕见的病变,不同寻常的是,它可能与分泌激素的垂体腺瘤相关。

病例报告

患者为一名49岁女性,表现为鞍区病变并伴有肢端肥大症。经蝶窦肿瘤切除术,但病情未改善。组织病理学分析显示为神经节细胞瘤合并生长激素分泌型腺瘤。

讨论

我们在文献中发现85例鞍内神经节细胞瘤合并分泌激素的垂体腺瘤的病例,分布如下:50例生长激素释放型(GH)(59%),15例混合型(GH和催乳素释放型)(17%),11例催乳素释放型(13%),7例促肾上腺皮质激素释放型(ACTH)(8%)和2例促肾上腺皮质激素释放激素型(CRH)(2%)。

结论

神经节细胞瘤-腺瘤混合型是罕见的病变。与生长激素释放型病例相关更为常见,最常见的表现是在中年女性中。诊断依靠组织病理学。识别这种病变很重要,因为它可能导致不完全切除术后治疗反应受限。

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