Abstract：
OBJECTIVE: Pseudomyogenic hemangioendothelioma (PHE) is a rare intermediate hemangioendothelioma. This article aims to study the clinicopathological features of PHE.
METHODS: We collected the clinicopathological features of 10 new PHE, and examined their molecular pathological features by fluorescence in situ hybridization. In addition, we summarized and analyzed the pathological data of 189 reported cases.
RESULTS: The case group consisted of six men and four women aged 12-83 years (median: 41 years). Five instances occurred in the limbs, three in the head and neck, and two in the trunk. Tumor tissues were composed of spindle cells and round or polygonal epithelioid cells, which were either arranged in sheets or interwoven, along with areas of transitional morphology. Scattered or patchy stromal neutrophil infiltration was observed. Tumor cells had abundant cytoplasm, and some contained vacuoles. The nuclei had mild to moderate atypia, with visible nucleoli, and mitosis was rare. PHE tissues diffusely expressed CD31 and ERG, but not CD34, Desmin, SOX-10, HHV8 or S100, while some samples expressed CKpan, FLI-1 and EMA. INI-1 stain is retained. The proliferation index of Ki-67 is 10-35%. Seven samples were detected by fluorescence in situ hybridization, six of which had breakages in FosB proto-oncogene (AP-1 transcription factor subunit). Two patients experienced recurrence; however, no metastasis or death occurred.
CONCLUSIONS: PHE is a rare soft tissue vascular tumor, which has biologically borderline malignant potential, local recurrence, little metastasis, and good overall survival and prognosis. Immunomarkers and molecular detection are valuable for diagnosis.
METHODS: We collected the clinicopathological features of 10 new PHE, and examined their molecular pathological features by fluorescence in situ hybridization. In addition, we summarized and analyzed the pathological data of 189 reported cases.
RESULTS: The case group consisted of six men and four women aged 12-83 years (median: 41 years). Five instances occurred in the limbs, three in the head and neck, and two in the trunk. Tumor tissues were composed of spindle cells and round or polygonal epithelioid cells, which were either arranged in sheets or interwoven, along with areas of transitional morphology. Scattered or patchy stromal neutrophil infiltration was observed. Tumor cells had abundant cytoplasm, and some contained vacuoles. The nuclei had mild to moderate atypia, with visible nucleoli, and mitosis was rare. PHE tissues diffusely expressed CD31 and ERG, but not CD34, Desmin, SOX-10, HHV8 or S100, while some samples expressed CKpan, FLI-1 and EMA. INI-1 stain is retained. The proliferation index of Ki-67 is 10-35%. Seven samples were detected by fluorescence in situ hybridization, six of which had breakages in FosB proto-oncogene (AP-1 transcription factor subunit). Two patients experienced recurrence; however, no metastasis or death occurred.
CONCLUSIONS: PHE is a rare soft tissue vascular tumor, which has biologically borderline malignant potential, local recurrence, little metastasis, and good overall survival and prognosis. Immunomarkers and molecular detection are valuable for diagnosis.
摘要：
目的：假肌源性血管内皮瘤（PHE）是一种罕见的中间型血管内皮瘤。本文旨在研究PHE的临床病理特征。
方法：我们收集了10个新PHE的临床病理特征，并通过荧光原位杂交检查其分子病理学特征。此外,对189例报告病例的病理资料进行总结分析。
结果：病例组由6名男性和4名女性组成，年龄为12-83岁（中位数：41岁）。五个例子发生在四肢，三个在头部和颈部，还有两个在后备箱里.肿瘤组织由梭形细胞和圆形或多边形上皮样细胞组成，它们要么排成薄片，要么交织在一起，以及过渡形态区域。观察到散在或斑片状的基质中性粒细胞浸润。肿瘤细胞具有丰富的细胞质，还有一些含有空泡。细胞核有轻度到中度的异型,有可见的核仁，有丝分裂是罕见的。PHE组织弥漫性表达CD31和ERG，但不是CD34，Desmin，SOX-10，HHV8或S100，而一些样品表示CKpan，FLI-1和EMA。INI-1染色被保留。Ki-67的增殖指数为10-35%。用荧光原位杂交法检测7份样品,其中6例FosB原癌基因（AP-1转录因子亚基）断裂。两名患者经历了复发;然而，无转移或死亡。
结论：PHE是一种罕见的软组织血管肿瘤，具有生物学边缘恶性潜力，局部复发,小转移,总体生存率和预后良好。免疫标记和分子检测对诊断有价值。
方法：我们收集了10个新PHE的临床病理特征，
结果：病例组由6名男性和4名女性组成，年龄为12-83岁（中位数：41岁）。
结论：PHE是一种罕见的软组织血管肿瘤，
