Hepatic perivascular epithelioid cell tumor (PEComa): Clinicopathological Findings and Prognosis-A Systematic Review
Alireza Abdollahi1, Masoomeh Safaei2
1Department of Pathology, Tehran University of Medical Sciences, Tehran, Iran.
Background & Objective:
Hepatic Perivascular epithelioid cell tumors (PEComas) are rare and can complicate diagnosis due to their low incidence, possible malignant potential and misdiagnosis as melanoma and hepatocellular carcinoma (HCC). The purpose of this systematic review and meta-analysis was to investigate the clinicopathological traits, diagnostic indicators, and prognostic implications associated with hepatic PEComas.
Methods:
We conducted a systematic literature search in PubMed, Web of Science, and Scopus using relevant keywords. Studies were included if they assessed clinicopathological findings or prognostic outcomes of hepatic PEComas. Data extraction was performed using a standardized sheet and data were pooled using RStudio.
Results:
The initial search retrieved 1,172 articles, from which 286 duplicates were removed. Ultimately, 15 studies met our eligibility criteria for inclusion in the meta-analysis. The mean patient age was 45.42 years. Misdiagnosis as HCC occurred in 18% of cases. The pooled malignancy rate was 8.6% with 6% of cases demonstrating tumor progression. Immunohistochemistry analysis revealed HMB-45 positivity in 98.8% (95% CI: 97% - 100%) and Smooth Muscle Actin positivity in 95.2% (95% CI: 81% - 98%) of cases. The mean tumor size was 67.1 mm (95% CI: 52.86 - 81.34), with 4.7% (95% CI: 3% - 8%) showing invasive features.
Conclusion:
Hepatic PEComas are predominantly benign but can pose diagnostic challenges due to their resemblance to HCC and melanoma. Despite generally favorable outcomes, long-term follow-up is crucial due to the risk of recurrence. Further prospective studies utilizing standardized diagnostic criteria are warranted to refine management strategies and identify prognostic indicators.
