肺血管瘤样纤维组织瘤临床病理分析并文献复习

目的：探讨肺血管瘤样纤维组织细胞瘤(angiomatoid fibrous histiocytoma，AFH)的临床特点、组织病理学及分子病理学特征。方法：收集1例肺AFH，进行光镜观察、免疫组织化学染色及EWSR1基因检测并复习相关文献。结果：镜下观肿瘤周围可见不完整纤维性假包膜，并见不等量淋巴细胞和浆细胞浸润，在肿瘤周围形成淋巴组织套；肿瘤内见散在假血管性腔隙；肿瘤细胞呈漩涡状、束状、席纹状排列，细胞核呈梭形、圆形和卵圆形，有一定异型性，罕见核分裂象与坏死。免疫表型：肿瘤细胞Vimentin和CD99均呈弥漫阳性，Ki-67增殖指数为5%。结论：血管瘤样纤维组织细胞瘤是一种罕见的交界性肿瘤，其诊断主要依靠病理形态学特征，免疫组织化学标记及FISH检测EWSR1基因有助于诊断和鉴别诊断；局部适当扩大切除，术后随访患者是主要处理原则。

Clinicopathologic analyses of pulmonary angiomatoid fibrous histiocytoma and review of the literature

Objective:To investigate the clinical features, histopathological characteristics, and molecular pathological characteristics of pulmonary angiomatoid fibrous histiocytoma (AFH). Methods:One case of pulmonary AFH was collected, light microscopy observation, immunohistochemical staining and EWSR1 gene detection were performed and the literature were reviewed. Results:Under microscopy, there were incomplete ifbrous pseudocapsule and unequal amounts of lymphocytes and plasma cell inifltrated around the tumor tissues, and formed lymphatic sleeve. Pseudo-vascular lacunae scattered in the tumor tisses. hTe tumor cells were swirling, buddle, or fasciculate arrangement, the nuclei were round or oval, some tumor cells with atypia, but rare mitotic and necrosis were detected. hTe immune phenotype:tumor cells Vimentin and CD99 diffuse positive, Ki-67 proliferation index was 5%. Conclusion: Angiomatoid fibrous histiocytoma is a rare borderline tumor and its diagnosis mainly depends on the pathological morphological features, and immunohistochemical markers and FISH EWSR1 detection help diagnosis and differential diagnosis; locally enlarged resection and postoperative follow-up of patients are the main principles.