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目的对肺硬化性血管瘤(PSH)与神经内分泌肿瘤之间的关系进行探讨。方法对1例罕见的肺硬化性血管瘤合并类癌(类癌在硬化性血管瘤中)进行光镜及免疫组化观察,并结合文献进行分析。结果患者为中年男性,临床表现为异位ACTH综合征及肺部阴影,行肿物及肺叶切除术。大体表现为近肺膜下类圆形结节,边界清楚。镜下可见结节中心约2/3区域为典型类癌样区:条索状、小梁状肿瘤细胞巢被纤细的胶原纤维所分隔,瘤细胞AE1/AE3、CgA、Syn、TTF-1和ACTH弥漫(+),vimentin(-);而1/3似环状或新月体样包绕中心区的部分为典型PSH样区,包括实片状及乳头状结构等。PSH内有两型肿瘤细胞:一型为形态一致的圆形细胞成片排列,TTF-1和vimentin弥漫(+),AE1/AE3、CgA和Syn(-);另一型为腺腔或乳头表面被覆的立方上皮细胞,AE1/AE3和TTF-1(+),vimentin、CgA和Syn(-)。类癌与PSH在交界处相互移行,分界不清,,有时可见两者间有一拉长的挤压变扁的腺性腔隙,两种瘤细胞均位于两侧的腺上皮下,但仍有灶性肿瘤细胞团可越过腺腔而在对侧相互交叉。结论PSH与神经内分泌细胞及其肿瘤之间或多或少存在着某种内在联系;PSH合并类癌可能与PSH起源的原始呼吸道上皮细胞多向分化有关。
Objective To investigate the relationship between pulmonary sclerosing hemangioma (PSH) and neuroendocrine tumors. Methods One case of rare sclerosing hemangioma with carcinoid (carcinoid in sclerosing hemangioma) was observed by light microscopy and immunohistochemistry combined with the literature. Results The patient was middle-aged male. The clinical manifestations were ectopic ACTH syndrome, pulmonary shadow, tumor and lobectomy. The general performance of subepithelial type of circular nodules, the border is clear. Microscopically, about 2/3 of the nodules were typical carcinoid-like areas: the nests of trabecular and trabecular tumor cells were separated by slender collagen fibers. The tumor cells AE1 / AE3, CgA, Syn, TTF-1 and ACTH diffuse (+), vimentin (-); and 1/3 like ring or crescent-like part of the central area surrounding the typical PSH-like areas, including the real flake and papillary structures. There are two types of tumor cells in the PSH: one is a circular arrangement of morphologically uniform cells, filled with (+), AE1 / AE3, CgA and Syn (-); TTF-1 and vimentin; Surface-covered cubic epithelial cells, AE1 / AE3 and TTF-1 (+), vimentin, CgA and Syn (-). Carcinoid and PSH at the junction of the migration of each other, the boundary is unclear, and sometimes there can be seen between the two elongated squeeze flattened glandular cavity, both tumor cells are located in both sides of the glandular epithelium, but there are still Focal tumor cell clusters can cross the gland lumen and cross each other on the contralateral side. Conclusion PSH and neuroendocrine cells and their tumors are more or less some intrinsic relationship exists; PSH-associated carcinoid may be related to the original multi-directional differentiation of the origin of the respiratory tract epithelial cells of PSH.