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[Polypoid PEComa: case report and literature review].
Aurélie Maran-Gonzalez1, Pierre Baldet, Valérie Costes
1Laboratoire d'anatomie et cytologie pathologiques, hôpital Lapeyronie, CHU de Montpellier, 371, avenue du Doyen-Gaston-Giraud, 34295 Montpellier cedex 5, France. aurelie.maran@hotmail.fr
Annales De Pathologie
|May 24, 2011
Summary
This report details an 11-year-old female with a rare perivascular epithelioid cell tumor (PEComa) of the rectum. The study reviews malignancy criteria and differential diagnosis for this uncommon mesenchymal tumor.
Area of Science:
- Oncology
- Pathology
- Rare Cancers
Background:
- Perivascular epithelioid cell tumors (PEComas) are rare mesenchymal neoplasms.
- These tumors are characterized by co-expression of myogenic and melanocytic markers.
- PEComas are thought to originate from perivascular cells.
Observation:
- An 11-year-old female presented with a 2 cm ulcerated rectal polyp.
- The polyp was exteriorized via the anus and completely resected.
- Histological examination revealed nests and cords of cells with clear cytoplasm and regular nuclei, lacking atypia.
Findings:
- Immunohistochemical analysis confirmed tumor cells stained positive for HMB45, a melanocytic marker.
- The tumor exhibited low mitotic activity and no necrotic areas.
- This case represents the 18th reported instance of PEComa in the literature.
Implications:
- This case contributes to the understanding of PEComa presentation in pediatric patients.
- The review focuses on critical malignancy criteria for PEComas.
- Accurate differential diagnosis is essential for appropriate management of these rare tumors.