Adrenal and Extra-Adrenal Nonfunctioning Composite Pheochromocytoma/Paraganglioma with Immunohistochemical Ectopic Hormone Expression: Comparison of Two Cases
Adrenal and Extra-Adrenal Nonfunctioning Composite Pheochromocytoma/Paraganglioma with Immunohistochemical Ectopic Hormone Expression: Comparison of Two Cases
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肾上腺和肾上腺外无功能复合性嗜铬细胞瘤/副神经节瘤伴免疫组织化学异位激素表达：两例比较
DOI：
10.1159/000317312
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发表时间：
2010-01-01
影响因子：
1.6
通讯作者：
Zhou, Qiao
中科院分区：
文献类型：
--
作者：
Gong, Jing;Wang, Xinxin;Zhou, Qiao
肾上腺复合性嗜铬细胞瘤是罕见的，其中大部分是功能性的，而肾上腺外复合性副神经节瘤是极其罕见的。我们描述并比较一个后腹膜外肾上腺复合性副神经节瘤和一个肾上腺复合性嗜铬细胞瘤的临床病理和免疫组化特征。两个肿瘤都没有功能，实验室检查显示没有生化异常。两个肿瘤均由典型的副神经节瘤/嗜铬细胞瘤和神经节细胞瘤组成。除了典型的免疫组化表型特征的每个组成部分，这两个肿瘤显示局灶性生长抑素染色，肾上腺肿瘤也是区域性胰岛素和催乳素阳性。尽管这种异常的免疫组化表达，相关的临床症状或实验室异常是不存在的。这些肿瘤有助于排除临床上无症状、无功能的复合性嗜铬细胞瘤或副神经节瘤的罕见发生，并伴有异常的激素表达。
Adrenal composite pheochromocytoma is rare, most of which is functional, and extra-adrenal composite paraganglioma is extremely rare. We describe and compare the clinicopathological and immunohistochemical features of a retroperitoneal extra-adrenal composite paraganglioma and an adrenal composite pheochromocytoma. Both tumors were nonfunctioning and laboratory tests revealed no biochemical abnormalities. Both tumors were composed of typical paraganglioma/pheochromocytoma closely admixed with ganglioneuroma component. In addition to typical immunohistochemical phenotypes characteristic of each component, both tumors showed focal staining of somatostatin, and the adrenal tumor was also regionally positive for insulin and prolactin. Despite this aberrant immunohistochemical expression, relevant clinical symptoms or laboratory abnormalities were absent. These tumors serve to exemplify the extremely rare occurrences of clinically silent, nonfunctioning composite pheochromocytoma or paraganglioma with aberrant expression of hormones.