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Background <p>Perivascular epithelioid cell tumours (PEComas) are rare mesenchymal tumours with distinct histological and immunohistochemical features. Diagnosis is challenging, as preoperative imaging often mimics other hepatic and pancreatic neoplasms. This study presents five institutional cases and a&#xa0;systematic review to identify common clinical characteristics, imaging findings, and management strategies.</p> Methods <p>A&#xa0;retrospective review of five histopathologically confirmed cases of hepatic PEComa at Royal North Shore Hospital, Sydney, from 2008–2024 was conducted. A&#xa0;systematic review was performed in accordance with Preferred Reporting Items for Systematic reviews and Meta-Analyses(PRISMA) guidelines, searching EMBASE and PubMed for hepatic and pancreatic PEComas published up to October 2024. Inclusion criteria comprised case reports or case series with histologically confirmed PEComa. The Murad et&#xa0;al. tool was applied to assess quality of the studies.</p> Results <p>Five patients (60% male, median age&#xa0;75&#xa0;years) were identified; two had chronic hepatitis&#xa0;B and two were incidental findings. Imaging demonstrated well-circumscribed, hypervascular masses, with arterial-phase enhancement. All underwent surgical resection, with definitive diagnosis confirmed histologically. The systematic review identified 14&#xa0;additional cases (median age&#xa0;49&#xa0;years, 78.6% female). Tumours ranged from 10&#xa0;to 180 mm, were typically well-defined and hypervascular, and were frequently misdiagnosed as hepatocellular carcinoma or neuroendocrine tumours. All were treated surgically, with no metastases reported.</p> Conclusion <p>Hepatic and pancreatic PEComas are rare tumours that lack specific radiological features and require pathological examination for definitive diagnosis. Surgical excision with clear margins yields excellent outcomes, though prolonged follow-up is recommended. Future studies should focus on the molecular profile and potential targeted therapies, such as mTOR inhibitors, in high-risk or recurrent PEComas.</p>

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Hepatopancreatic perivascular epithelioid cell tumours (PEComa): a single institution series and systematic review

  • 期刊论文

  • 发表日期：

    2025年11月14日

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  • Vincent Liu,
  • Rees Telford,
  • Krishna Kotecha,
  • Alberto Alejandro Boue,
  • Ramesh Damodaran Prabha,
  • Anthony J. Gill,
  • Anubhav Mittal,
  • Jaswinder S. Samra

摘要

Background

Perivascular epithelioid cell tumours (PEComas) are rare mesenchymal tumours with distinct histological and immunohistochemical features. Diagnosis is challenging, as preoperative imaging often mimics other hepatic and pancreatic neoplasms. This study presents five institutional cases and a systematic review to identify common clinical characteristics, imaging findings, and management strategies.

Methods

A retrospective review of five histopathologically confirmed cases of hepatic PEComa at Royal North Shore Hospital, Sydney, from 2008–2024 was conducted. A systematic review was performed in accordance with Preferred Reporting Items for Systematic reviews and Meta-Analyses(PRISMA) guidelines, searching EMBASE and PubMed for hepatic and pancreatic PEComas published up to October 2024. Inclusion criteria comprised case reports or case series with histologically confirmed PEComa. The Murad et al. tool was applied to assess quality of the studies.

Results

Five patients (60% male, median age 75 years) were identified; two had chronic hepatitis B and two were incidental findings. Imaging demonstrated well-circumscribed, hypervascular masses, with arterial-phase enhancement. All underwent surgical resection, with definitive diagnosis confirmed histologically. The systematic review identified 14 additional cases (median age 49 years, 78.6% female). Tumours ranged from 10 to 180 mm, were typically well-defined and hypervascular, and were frequently misdiagnosed as hepatocellular carcinoma or neuroendocrine tumours. All were treated surgically, with no metastases reported.

Conclusion

Hepatic and pancreatic PEComas are rare tumours that lack specific radiological features and require pathological examination for definitive diagnosis. Surgical excision with clear margins yields excellent outcomes, though prolonged follow-up is recommended. Future studies should focus on the molecular profile and potential targeted therapies, such as mTOR inhibitors, in high-risk or recurrent PEComas.