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Primary retroperitoneal PEComa: an incidental finding
  1. Bárbara Monteiro Marinho,
  2. António Gâmboa Canha,
  3. Donzília Sousa Silva and
  4. José Davide Pinto Silva
  1. Department of Surgery, HEBIPA - Hepatobiliary and Pancreatic Unit, Hospital de Santo António, Centro Hospitalar Universitário do Porto, Porto, Portugal
  1. Correspondence to Dr Bárbara Monteiro Marinho; barbaramarinho_12{at}


Perivascular epithelioid cell neoplasm (PEComa) is a rare type of tumour, and primary retroperitoneal PEComa is rarer still. Although pulmonary lymphangioleiomyomatosis (LAM), angiomyolipomas and clear cell ‘sugar’ tumours of the lung are well described, relatively little is known about other members of the PEComa family. We describe a case of an asymptomatic retroperitoneal PEComa, lymphangioleiomyoma type, which appeared in a previously healthy middle-aged woman as an incidental finding, in a CT scan performed in the context of spontaneous pneumothorax. The patient underwent surgical excision of the tumour and the histopathological and immunohistochemical analysis of the surgical specimen made the definitive diagnosis. Although rare, reports of isolated retroperitoneal lymphangioleiomyoma and primary retroperitoneal PEComas NOS (not otherwise specified) are described in the literature, normally associated with pulmonary LAM. The patient’s pulmonary imaging was normal. Short-term re-examination did not detect any recurrence. We also provide a literature review of this rare group of tumours.

  • Surgical oncology
  • General surgery
  • Pneumothorax
  • Lung function

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  • Contributors Supervised by JDPS. Patient was under the care of AGC and DSS. Report was written by BMM.

  • Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.

  • Case reports provide a valuable learning resource for the scientific community and can indicate areas of interest for future research. They should not be used in isolation to guide treatment choices or public health policy.

  • Competing interests None declared.

  • Provenance and peer review Not commissioned; externally peer reviewed.