临床与病理杂志2024，Vol.44Issue(10)：1458-1464,7.DOI:10.11817/j.issn.2095-6959.2024.240314
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
摘要
Abstract
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(CD34),hypoxia-inducible factor-1α(HIF-1α),and vascular endothelial growth factor(VEGF).Reverse transcription polymerase chain reaction(RT-PCR)showed upregulation of HIF-1α and 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 CD34.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Key words
pleomorphic hyalinizing angiectatic tumor of soft parts/vimentin/cluster of differentiation 34/hypoxia-inducible factor-1α/vascular endothelial growth factor引用本文复制引用
郑银海,李广柳,李伟滔,朱莹,叶伟标..软组织多形性玻璃样变血管扩张性肿瘤1例并文献复习[J].临床与病理杂志,2024,44(10):1458-1464,7.基金项目
国家自然科学基金(82103269) （82103269）
广东省医学科研基金(C2018054).This work was supported by the National Natural Science Foundation(82103269)and the Medical Research Fund of Guangdong Province(C2018054),China. （C2018054）
