背景:血管周围上皮样细胞肿瘤(PEComas)包括一组罕见的间充质肿瘤,具有双重黑素细胞和肌肉分化。肝脏PEComas罕见且难以诊断,他们的行为还不清楚。
方法:这里,在过去的5年中,我们从我们和合作中心的档案中报告了总共5例肝脏和肝周PEComas病例。进行了详细的组织学评估。使用了一组完整的免疫组织化学染色,并使用分离探针对TFE3基因进行了荧光原位杂交分析。
结果:所有这些患者都是女性,平均年龄为44岁。病变位于肝右叶:3例,左肝叶:一例,胃肝韧带1例。术前临床诊断为肝细胞癌(HCC),局灶性结节增生,血管瘤,转移,胃肠道间质瘤,分别。4例进行了手术切除,没有进一步的辅助治疗。组织病理学检查和随后的免疫表型分型揭示了PEComa的诊断。对4例TFE3基因重排进行荧光原位杂交分析。
结论:本系列强调了肝或肝周PEComas的准确组织学诊断对于防止不必要的积极治疗非常重要,与原发性肝细胞癌或肝样/上皮样转移性肿瘤不同。
BACKGROUND: Perivascular epithelioid cell tumors (PEComas) encompass a group of rare mesenchymal neoplasms, with dual melanocytic and muscular differentiation. Hepatic PEComas are rare and difficult to diagnose, and their behavior is still unclear.
METHODS: Herein, we report a total of five cases of hepatic and perihepatic PEComas over a period of the last 5 years from our and collaborating center\'s archive. A detailed histological evaluation was done. A comprehensive panel of immunohistochemical stains was used and fluorescence in-situ hybridization analysis was performed for the TFE3 gene using break-apart probes.
RESULTS: All these patients were women, with an average age of presentation of 44 years. The lesions were in the right hepatic lobe: three cases, the left hepatic lobe: one case, and gastrohepatic ligament: one case. The preoperative clinicoradiological diagnoses were hepatocellular carcinoma (HCC), focal nodular hyperplasia, hemangioma, metastasis, and gastrointestinal stromal tumor, respectively. Surgical excision was performed in four cases with no further adjuvant therapy. Histopathological examination and subsequent immunophenotyping revealed a diagnosis of PEComa. Fluorescence in-situ hybridization analysis was performed for TFE3 gene rearrangement in four cases.
CONCLUSIONS: This series highlights the fact that accurate histological diagnosis of hepatic or perihepatic PEComas is important to prevent unnecessary aggressive treatment, unlike primary hepatocellular carcinomas or hepatoid/epithelioid metastatic tumors.