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多形性透明血管黏液样肿瘤。

Pleomorphic hyalinizing angiectatic tumor of soft parts.

机构信息

Department of Pathology, Taipei City Hospital, Taipei, Taiwan.

出版信息

J Formos Med Assoc. 2010 Aug;109(8):616-20. doi: 10.1016/S0929-6646(10)60100-3.

Abstract

Pleomorphic hyalinizing angiectatic tumor (PHAT) of soft parts is a rare, nonmetastasizing tumor of uncertain lineage which was first reported in 1996. Here, we report a case of PHAT and review the literature. A 49-year-old man presented with a soft and progressively enlarging mass over the right buttock for several years. On suspicion that the mass was a right gluteal lipoma, he underwent surgical excision. The excised lesion measured 14 x 6 x 3.5 cm. It had a variegated appearance with a white-tan to yellowish color on the cut surface. Some punctate hemorrhage and vessel thrombosis were seen. Microscopically, the tumor was a PHAT characterized by clusters of ectatic, fibrin-lined, thin-walled vessels, which were surrounded by a mitotically inert, spindled, pleomorphic, neoplastic stroma that contained a variable inflammatory component. Immunohistochemical study showed that the tumor cells were positive for CD34, and negative for S-100, HMB45 and actin. The patient experienced local recurrence 6 months later. The recurrent tumor was widely excised. No evidence of metastasis was found during the 18 months after the second operation. The recurrent lesion had a microscopic appearance that was similar to the initial lesion.

摘要

软组织多形性嗜酸性血管扩张性肿瘤(PHAT)是一种罕见的、非转移性的、来源不确定的肿瘤,于 1996 年首次报道。在此,我们报告 1 例 PHAT,并复习文献。1 例 49 岁男性,右臀部有一软组织逐渐增大的包块数年,质软。术前考虑为右臀部脂肪肉瘤,行手术切除。切除标本大小为 14cm×6cm×3.5cm,切面呈多彩状,灰白色至黄色,可见点状出血和血管内血栓形成。镜下,肿瘤为 PHAT,由簇状扩张、纤维蛋白样、薄壁血管组成,周围为有丝分裂不活跃的梭形、多形性、肿瘤性间质,其中含有可变的炎症成分。免疫组化染色示肿瘤细胞 CD34 阳性,S-100、HMB45 和肌动蛋白阴性。术后 6 个月患者局部复发,再次行广泛切除术。术后 18 个月无转移证据。复发性肿瘤镜下与初次病变相似。

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