JoVE - Journal of Visualized Experiments
JoVE Visualize
Contact Us

Malignant perivascular epithelioid cell tumor of the colorectum: Clinicopathological characterization, diagnosis and treatment process of 7 cases

  • 0Department of Pharmacy, The First Affiliated Hospital, Zhejiang University School of Medicine, Hangzhou 310003, Zhejiang Province, China.
World Journal of Gastrointestinal Oncology +

|

February 17, 2025

|

February 17, 2025

View abstract on PubMed

Summary

This summary is machine-generated.

Radical resection is crucial for improving survival in rare colorectal perivascular epithelioid cell tumors (PEComas). Further research is needed to standardize diagnosis and treatment for this aggressive cancer.

Area Of Science

  • Gastroenterology
  • Surgical Oncology
  • Pathology

Background

  • Perivascular epithelioid cell tumor (PEComa) of the colorectum is an exceptionally rare neoplasm.
  • These tumors exhibit variable biological behavior, from benign to highly malignant.
  • Malignant colorectal PEComa carries a poor prognosis with limited treatment consensus.

Purpose Of The Study

  • To comprehensively characterize colorectal PEComa.
  • To establish standardized diagnostic criteria and treatment protocols.

Main Methods

  • Retrospective analysis of seven confirmed cases of malignant colorectal PEComa.
  • Collection of clinicopathological, therapeutic, and follow-up data.
  • Disease staging and progression monitoring using contrast-enhanced computed tomography.

Main Results

  • The study included patients aged 5–73 years, with 71.4% occurring in the colon.
  • Abdominal mass and hematochezia were the primary symptoms; infiltrative growth was the main microscopic indicator of malignancy.
  • Immunohistochemistry showed HMB45 (6/7), melan-A (5/7), and MiTF (3/5) positivity. Radical resection demonstrated superior outcomes compared to a watch-and-wait approach, with a median progression-free survival exceeding 38 months.

Conclusions

  • Radical or extended resection is the cornerstone for achieving prolonged survival in malignant PEComa.
  • There is an urgent need for further research to develop standardized diagnostic and therapeutic guidelines for colorectal PEComa.

Keywords: