Perivascular epitheloid cell tumour (PEComa) of the retroperitoneum – a rare tumor with uncertain malignant behaviour: a case report
2009

Rare Tumor in the Abdomen: A Case Report

Sample size: 1 publication Evidence: low

Author Information

Author(s): Koenig Alexandra M, Quaas Alexander, Ries Thorsten, Yekebas Emre F, Gawad Karim A, Vashist Yogesh K, Burdelski Christoph, Mann Oliver, Izbicki Jakob R, Erbersdobler Andreas

Primary Institution: University Medical Centre of Hamburg-Eppendorf

Hypothesis

What are the characteristics and potential malignancy of a perivascular epitheloid cell tumor (PEComa) in the retroperitoneum?

Conclusion

The lung lesions observed may represent metastases from the PEComa rather than independent tumors, highlighting the need for further understanding of this rare tumor type.

Supporting Evidence

  • The tumor was characterized by a proliferation of perivascular cells with an epitheloid phenotype.
  • Histological analysis suggested a malignant potential due to focal necrosis and a 10% proliferative index.
  • Postoperative scans showed multiple lung lesions compatible with lymphangioleiomyomatosis.

Takeaway

This study talks about a rare tumor found in a woman's abdomen that might spread to her lungs, and doctors need to learn more about it to understand how dangerous it can be.

Methodology

The tumor was surgically removed and analyzed using histological and immunohistochemical techniques.

Limitations

The lung lesions were not biopsied, making it impossible to definitively determine their origin.

Participant Demographics

A 27-year-old woman.

Digital Object Identifier (DOI)

10.1186/1752-1947-3-62

Want to read the original?

Access the complete publication on the publisher's website

View Original Publication