肺硬化性血管瘤中合并类癌的临床病理观察  

Carcinoid in pulmonary sclerosing hemangioma:a case report and review of literature

作　　者：师杰[1] 杨堤[1] 孟芝兰[1] 

机构地区：[1]中国医学科学院中国协和医科大学北京协和医院病理科

出　　处：《诊断病理学杂志》2009年第4期273-276,共4页Chinese Journal of Diagnostic Pathology

摘　　要：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 the pulmonary sclerosing hemangioma （PSH） with neuroendocrine tumors. Methods A rare case of earcinoid in the pulmonary sclerosing hemangioma （PSH） was studied with conventional histological and stains, as well as literature review. Results A 41-year-old man presented with ectopic ACTH syndrome. CT scans showned a peripheral nodule in the fight lower lobe, and then produced pulmonary lobectomy. Macroscopically, a well-circumscribed nodule presented near the pleura. Microscopically, 2/3 of the nodule near the center appeared as carcinoid tumor： uniform cells were arranged in nests or cords, and separated by collagen fibrovascular stroma. The tumor cells were diffusely positive for AE1/AE3, CgA, Syn, TTF-1 and ACTH, and negative for virnentin. The rest of the nodule surrounding the center resembled a circle or sicrescent and presented as a PSH： it composed of two cell types arranged in papillary and solid patten with scattered cuboidal surface cells forming small tubules. The one type was round stmmal cell, which was positive for TTF-1, vimentin and negative for AE1/AE3, CgA, Syn and ACTH, and another was surface cell which was positive for AE1/AE3, TTF-1 and negative for vhnentin, CgA, Syn and ACTH. The two tumors overlapped at the borderline, sometimes there might be a crushing glandular cavity between them and the tumor nests of the two beneath the epithelium, though several nests could cross the glandular cavity to the other side. Conclusion PSH does not origin from neuroendocrine cell, but there might be some relationship between them more or less; carcinoid in PSH might be concerned with the possible origination from primitive, undifferentiated respiratory epithelium, and has potential to differentiate into respiratory epithelium and neuroendocrine cell.

关 键 词：硬化性血管瘤 类癌 异位ACTH综合征 

分 类 号：R734.2[医药卫生—肿瘤]