软组织多形性玻璃样变血管扩张性肿瘤1例并文献复习  

Pleomorphic hyalinizing angiectatic tumor of soft parts:A case report and literature review

作　　者：郑银海 李广柳 李伟滔 朱莹 叶伟标 ZHENG Yinhai;LI Guangliu;LI Weitao;ZHU Ying;YE Weibiao(First School of Clinical Medicine,Guangdong Medical University,Zhanjiang Guangdong 524023;Department of Pathology,Dongguan People’s Hospital of Guangdong Medical University,Dongguan Guangdong 523059,China)

机构地区：[1]广东医科大学第一临床医学院,广东湛江524023 [2]广东医科大学附属东莞市人民医院病理科,广东东莞523059

出　　处：《临床与病理杂志》2024年第10期1458-1464,共7页Journal of Clinical and Pathological Research

基　　金：国家自然科学基金(82103269);广东省医学科研基金(C2018054)。

摘　　要：为探讨软组织多形性玻璃样变血管扩张性肿瘤(pleomorphic hyalinizing angiectatic tumor of soft parts,PHAT)的临床病理特征和诊断要点,本文对1例发生在腹膜后的PHAT患者进行组织形态学观察、免疫组织化学检查和分子检测,并检索相关文献。患者为男性,因下腹部不适1年余,电子计算机断层扫描(computed tomography,CT)示下腹部占位入院,行肿物切除术。大体肿物呈结节状,大小为24 cm×19 cm×12 cm,边界相对清晰,切面灰红色,可见大小不等的出血性囊腔。镜下观察显示:肿物内有丰富的呈簇状分布的扩张性血管,血管壁可见厚的玻璃样变物质。血管之间见呈片状分布的梭形或多形性瘤细胞,部分细胞具有明显异型性,核内假包涵体可见,但核分裂罕见。间质内见多少不等的炎症细胞和含铁血黄素沉着。免疫组织化学显示:肿瘤细胞表达波形蛋白(vimentin)、抗原分化簇34(cluster of differentiation 34,CD34)、缺氧诱导因子-1α(hypoxia-inducible factor 1-alpha,HIF-1α)和血管内皮生长因子(vascular endothelial growth factor,VEGF)。反转录聚合酶链反应(reverse transcription polymerase chain reaction,RT-PCR)检测显示:与癌旁组织相比,肿瘤组织HIF-1α和VEGF mRNA表达上调(均P<0.05)。PHAT是一种分化方向未定的具有低度恶性潜能的软组织肿瘤,其发病年龄范围和累及部位较广,肿瘤以含有扩张的玻璃样变的薄壁血管簇、在血管之间有呈片状分布的梭形或多形性瘤细胞、瘤细胞表达vimentin和CD34为特征,因瘤细胞具有多形性而容易被误诊为软组织肉瘤,熟悉其组织学形态和免疫表型特征有助于诊断和鉴别诊断。To investigate the clinical pathological characteristics and diagnostic essentials of pleomorphic hyalinizing angiectatic tumor(PHAT),this study analyzed the histological morphology,immunohistochemical findings,and molecular tests of a retroperitoneal PHAT case,along with a literature review.The patient,a male,presented with over one year of lower abdominal discomfort.Computed tomography(CT)revealed a mass in the lower abdomen.Tumor resection was performed.Macroscopically,the tumor was nodular,measuring 24 cm×19 cm×12 cm,with a relatively clear boundary and a gray-red cut surface containing hemorrhagic cystic spaces of varying sizes.Microscopic examination revealed clusters of dilated blood vessels with thick hyalinized walls.Between these vessels were sheets of spindle or pleomorphic tumor cells,some exhibiting marked atypia and intranuclear pseudoinclusions.However,mitotic figures were rare.The stroma displayed varying degrees of inflammatory cell infiltration and hemosiderin deposition.Immunohistochemically,tumor cells expressed vimentin,cluster of differentiation 34(CD 34),hypoxia-inducible factor-1α(HIF-1α),and vascular endothelial growth factor(VEGF).Reverse transcription polymerase chain reaction(RT-PCR)showed upregulation of HIF-1αand VEGF mRNA expression in tumor tissue.PHAT is a soft tissue tumor of indeterminate differentiation with low malignant potential,affecting a wide age range and various anatomical locations.Histologically,it is characterized by clusters of dilated hyalinized thin-walled blood vessels,sheets of spindled or pleomorphic tumor cells between these vessels,and immunoreactivity for vimentin and CD 34.The pleomorphism of tumor cells may lead to misdiagnosis as a soft tissue sarcoma.Familiarity with its histological and immunophenotypic features aids in its diagnosis and differential diagnosis.

关 键 词：软组织多形性玻璃样变血管扩张性肿瘤 波形蛋白 抗原分化簇34 缺氧诱导因子-1Α 血管内皮生长因子 

分 类 号：R73[医药卫生—肿瘤]