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Mixed Gangliocytoma-Pituitary Adenoma: Insights on the Pathogenesis of a Rare Sellar Tumor.

混合性神经节细胞瘤-垂体腺瘤:对一种罕见鞍区肿瘤发生机制的见解

Abstract

Gangliocytomas originating in the sellar region are rare; most are tumors composed of gangliocytic and pituitary adenomatous elements, forming the so-called mixed gangliocytoma-pituitary adenoma. The majority of mixed gangliocytoma adenomas are associated with endocrinopathies, mainly acromegaly and less often Cushing disease and hyperprolactinemia. In the present study, 10 cases of mixed gangliocytoma and somatotroph adenomas were evaluated for patterns of cellular differentiation and expression of lineage-specific transcription factors. The tumors were characterized by immunohistochemistry for pituitary hormones, cytokeratins, Pit-1, and the neuronal markers NeuN, neurofilaments (NFP), and MAP2. Double-labeling immunohistochemistry for Pit-1/GH, Pit-1/NFP, Pit-1/MAP2, and NeuN/GH was performed in 9/10 tumors. Our data demonstrate that both adenomatous and ganglionic cells express the acidophilic lineage transcription factor Pit-1. Although mixed gangliocytomas and somatotroph adenomas show histologically distinct cellular populations, there is at least a small population of cells that coexpress the Pit-1 transcription factor and neuronal-associated cytoskeletal proteins favoring the theory of transdifferentiation of neuroendocrine cells into neuronal elements of these mixed tumors.

摘要

起源于鞍区的神经节细胞瘤极为罕见;大部分肿瘤含有神经节细胞和腺瘤细胞成分,因此也称混合性神经节细胞瘤-垂体腺瘤。大部分混合性神经节细胞瘤-垂体腺瘤与内分泌疾病相关,其中主要为肢端肥大症,较为少见的还有库欣病和高泌乳素血症。本研究对10例混合性神经节细胞瘤-垂体腺瘤病例的细胞分化模式以及谱系特异性转录因子表达进行分析。免疫组织化学方法标记脑垂体激素、细胞角蛋白、垂体特异转录因子-1(Pit-1)、神经标记物NeuN、神经微丝(NFP)和微管相关蛋白2(MAP2)以区分各患者特征。免疫组织化学双标记技术在9例肿瘤中进行Pit-1/GH、Pit-1/NFP、Pit-1/MAP2以及NeuN/GH染色。研究数据显示在腺瘤细胞以及神经节细胞中均存在嗜酸性家族转录因子Pit-1的表达。尽管混合性神经节细胞瘤和生长激素腺瘤在组织结构上显示为不同的细胞群体,但二者都有一小部分细胞同时表达Pit-1转录因子和神经元相关的细胞骨架蛋白,这一发现支持我们的推测:即该混合肿瘤的神经元细胞成分是由神经内分泌细胞诱导分化而来。

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