Background: Perivascular epithelioid cell tumors (PEComas) are a rare group of mesenchymal neoplasms with distinctive histological and immunohistochemical features. This systematic review aims to characterize the clinical presentation, diagnostic approach, treatment, and outcomes of adult patients with hepatic PEComa. Methods: We performed a systematic literature search for English-language articles regarding hepatic PEComas using the terms (perivascular epithelioid cell tumor) OR (PEComa) AND (liver) OR (hepatic), up to 25 May 2025. Results: A total of 145 studies encompassing 281 patients were included in the analysis. Most studies originated from Asia. The mean age at diagnosis was 46 years (IQR: 35.25–53.75) with a female predominance. The underlying comorbidities were uncommon among the reported cases, and more than half were asymptomatic at presentation. The tumor presented as a single liver lesion in almost 9 out of 10 patients. Surgical excision was the primary treatment, and diagnosis in 74% of patients was made with positive immunohistochemistry for markers such as HMB-45 and smooth muscle actin. A malignant phenotype was reported in 30 cases. The median follow-up duration was 24 months (IQR: 12–48); recurrence occurred in 17 patients, and disease-related mortality occurred in 8 patients. Conclusions: Primary hepatic PEComa is a rare liver tumor with mostly benign clinical behavior and non-specific presentation. Future studies are needed to support clinician decisions regarding this entity and improve patient care.
背景：血管周上皮样细胞肿瘤（PEComa）是一组罕见的间叶源性肿瘤，具有独特的组织学和免疫组化特征。本系统综述旨在描述成人肝PEComa患者的临床表现、诊断方法、治疗及预后。方法：我们系统检索了截至2025年5月25日发表的英文文献，检索词为（血管周上皮样细胞肿瘤）或（PEComa）与（肝脏）或（肝性）的组合。结果：共纳入145项研究，涵盖281例患者。多数研究来自亚洲。诊断时平均年龄为46岁（四分位距：35.25–53.75），女性居多。报告病例中基础合并症少见，超过半数患者就诊时无症状。近九成患者表现为单发肝脏病灶。手术切除是主要治疗方式，74%的患者通过HMB-45和平滑肌肌动蛋白等标志物阳性免疫组化确诊。30例报告为恶性表型。中位随访时间为24个月（四分位距：12–48）；17例患者出现复发，8例患者死于本病。结论：原发性肝PEComa是一种罕见的肝脏肿瘤，多呈良性临床过程且表现不具特异性。未来需要更多研究以支持临床医生对此类疾病的诊疗决策并改善患者照护。
肿瘤(癌症)患者之家