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Related Concept Videos

Peritoneum01:21

Peritoneum

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The peritoneum is a vital membrane that lines the abdominal cavity and covers most of the organs within it. It plays a crucial role in protecting the organs, providing a smooth surface for their movement, and facilitating various physiological processes. Understanding the anatomy and function of the peritoneum is essential for comprehending the complexities of the abdominal region.
Anatomy of the Peritoneum
The peritoneum is divided into two layers: the parietal peritoneum and the visceral...
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Updated: Aug 22, 2025

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Primary retroperitoneal PEComa: an incidental finding.

Bárbara Monteiro Marinho1, António Gâmboa Canha2, Donzília Sousa Silva2

  • 1Department of Surgery, HEBIPA - Hepatobiliary and Pancreatic Unit, Hospital de Santo António, Centro Hospitalar Universitário do Porto, Porto, Portugal barbaramarinho_12@hotmail.com.

BMJ Case Reports
|November 11, 2022
PubMed
Summary
This summary is machine-generated.

This study reports a rare case of retroperitoneal PEComa (perivascular epithelioid cell neoplasm) in a woman, discovered incidentally. Surgical removal and analysis confirmed the diagnosis, with no recurrence noted.

Keywords:
General surgeryLung functionPneumothoraxSurgical oncology

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Area of Science:

  • Oncology
  • Pathology
  • Rare Tumors

Background:

  • Perivascular epithelioid cell neoplasms (PEComas) are rare tumors with limited understanding of non-pulmonary manifestations.
  • Pulmonary lymphangioleiomyomatosis (LAM) and other PEComas are documented, but primary retroperitoneal PEComas are exceptionally infrequent.

Observation:

  • A case of an asymptomatic retroperitoneal PEComa, lymphangioleiomyoma type, was incidentally found in a middle-aged woman via CT scan for spontaneous pneumothorax.
  • The patient was previously healthy, and the retroperitoneal mass was an incidental finding.
  • Pulmonary imaging was normal, and no recurrence was detected on short-term follow-up.

Findings:

  • Histopathological and immunohistochemical analysis confirmed the diagnosis of retroperitoneal PEComa.
  • The tumor was surgically excised.
  • Literature review of rare retroperitoneal PEComas, often associated with pulmonary LAM, was provided.

Implications:

  • This case expands the understanding of PEComa presentations, particularly isolated retroperitoneal occurrences.
  • Highlights the importance of thorough histopathological and immunohistochemical evaluation for rare tumors.
  • Contributes to the literature on PEComas, emphasizing their diverse locations and associations.