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Perivascular epithelioid cell tumour-mimicking retroperitoneal leiomyosarcoma
  1. Shirin Siddiqi1 and
  2. Lusine Mesropyan2
  1. 1Surgery, Conemaugh Health System, Johnstown, Pennsylvania, USA
  2. 2Surgery, Banner University Medical Center South, Tucson, Arizona, USA
  1. Correspondence to Dr Shirin Siddiqi; ssiddiqi{at}


A young man in his 40s was evaluated in the emergency department for abdominal and right flank pain. A CT scan of the abdomen and pelvis showed a solid, well-circumscribed lesion measuring 7.1×8.1×5.4 cm, which was arising from the retroperitoneum and extending from the third portion of the duodenum towards the right kidney. A percutaneous core biopsy was obtained, demonstrating an atypical smooth muscle neoplasm suggestive of a low-grade leiomyosarcoma. The patient underwent surgery for an en-block resection of the mass and the final pathology confirmed a perivascular epithelioid cell neoplasm without significant pleomorphism, mitosis or necrosis. Our case adds to the small number of perivascular epithelioid cell tumour cases reported in the literature and we present it in order to increase our understanding of this tumour and to assist in its appropriate diagnosis and management.

  • Musculoskeletal and joint disorders
  • Radiology
  • Gastrointestinal surgery
  • Surgical oncology

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  • Contributors LM: conception of work, revising for final approval, accountable for the accuracy of the submission. SS: drafting manuscript, literature search, patient communication, editing for final approval, accountable for the accuracy of the submission.

  • 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.