Abstract：
Pseudomyogenic hemangioendothelioma (PMH) is a rare tumor of vascular origin with intermediate malignant potential which commonly presents as a subcutaneous and soft-tissue mass with or without concurrent bone involvement. However, PMH presenting as primary multifocal bone lesions is rare. Histomorphologically, it mimicks other epithelioid tumors and cytokeratin expression in PMH can prompt an erroneous diagnosis of metastatic carcinoma, especially in an elderly patient. Diligent histopathological examination and judicious immunohistochemistry panel can guide to the correct diagnosis. Due to its rarity, the optimal therapeutic strategy has not been established till date. We present a rare case of PMH of primary bone with multifocal bony disease in a 23-year-old male who presented with severe bone pains. The patient has been managed with four weekly denosumab, and the disease is stable with symptomatic relief after 6 months.
摘要：
假肌源性血管内皮瘤（PMH）是一种罕见的血管源性肿瘤，具有中等恶性潜能，通常表现为皮下和软组织肿块，有或没有并发骨受累。然而,表现为原发性多灶性骨病变的PMH很少见。组织形态,它模仿其他上皮样肿瘤和细胞角蛋白在PMH的表达可以提示转移癌的错误诊断，尤其是老年患者。勤奋的组织病理学检查和明智的免疫组织化学小组可以指导正确的诊断。由于它的稀有性,到目前为止，最佳治疗策略尚未建立。我们介绍了一名23岁男性患有严重骨痛的原发性骨PMH并伴有多灶性骨病的罕见病例。病人已经接受了四次每周一次的denosumab治疗，6个月后病情稳定，症状缓解。
