肝脏非特异性血管周上皮样细胞肿瘤:附1例报告(英文)

Hepatic perivascular epithelioid cell tumors-not otherwise specified: a case report

血管周上皮样细胞分化的肿瘤是一组罕见的和血管关系密切的间叶肿瘤,组织学和免疫表型上具有血管周上皮样细胞的特征,联合表达黑色素瘤抗体和肌源性抗体。尽管已有相关病例的报道,如良性血管平滑肌脂肪瘤,但对于其起源及分型仍有争议,需深入探讨。我们报道一例肝脏非特异性血管周上皮样细胞肿瘤,具有不同于一般良性肝脏血管平滑肌脂肪瘤的病理学及免疫组织化学特点。肝脏非特异性血管周上皮样细胞肿瘤可以被新定义为一种具有"潜在恶性风险"或"低度恶性"生物学行为的罕见肿瘤,需收集相关病例及进行长期随访,进一步研究此类肿瘤的生物学行为,更新肝脏血管周上皮样细胞肿瘤的病理分型。

Neoplasms of perivascular epithelioid cells （PEComas） are characterized by epithelioid to spindle cells with eosinophilic to clear cytoplasm, an intimate relationship with blood vessels, and coexpression of myoid and melanocytic immunohistochemical markers. While most reported hepatic PEComas, such as angiomyolipoma （AML）, behave in a benign fashion, emerging PEComas cases without typical characteristics require further clarification. We report a case of primary hepatic perivascular epithelioid cell tumors-not otherwise specified （HPEComas-NOS） with untypical pathological and immunohistochemical features compared to those of the benign hepatic AML cases. HPEComas-NOS may represent a special type of PEComas classified as having ＂malignant potential＂ or at ＂high risk of aggressive behavior＂, suggesting the need for further clarification of hepatic PEComas and long-term follow-up of patients with HPEComas-NOS.