Synonyms
Definition
It is a neoplasm with smooth muscle and melanocytic differentiation which arises from perivascular epithelioid cells.
Clinical Features
Incidence
Very uncommon tumors. No association with tuberous sclerosis
Age
Most reported cases between 19 and 48 years
Sex
No sex predilection
Site
Most cases arise in the kidney (angiomyolipoma) that should be described elsewhere, but with anecdotal cases described in the bladder or prostate (Martignoni et al. 2015).
Treatment
Surgery
Outcome
The majority of the cases have shown a benign course with only a few malignant cases with metastatic disease.
Macroscopy
PEComa shows typically a solid well-circumscribed or infiltrative tumor with a size range of 0.5–9 cm in greatest dimension.
Microscopy
The tumor may show two patterns, epithelioid and spindled, with one dominant pattern. Epithelioid pattern is arranged in nests and spindled pattern in fascicles. The epithelioid component is associated with blood vessel. Tumor cells...
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References and Further Reading
Martignoni, G., Pea, M., Zampini, C., et al. (2015). PEComa of the kidney and of genitourinary tract. Seminars in Diagnostic Pathology, 32, 140–159.
Pan, C.-C., Yu, I. T., Yang, A.-H., et al. (2003). Clear cell myomelanocytic tumor of the urinary bladder. The American Journal of Surgical Pathology, 27, 689–692.
Sukov, W. R., Cheville, J. C., Amin, M. B., et al. (2009). Perivascular epithelioid cell tumor (PEComa) of the urinary bladder: Report of 3 cases and review of the literature. The American Journal of Surgical Pathology, 33, 304–308.
Weinreb, I., Howarth, D., Latta, E., et al. (2007). Perivascular epithelioid cell neoplasm (PEComas): Four malignant cases expanding the histopathological spectrum and a description of a unique findings. Virchows Archiv, 450, 463–470.
Williamson, S. R., Bunde, P. J., Montironi, R., & Lopez-Beltran, A. (2013). Malignant perivascular epithelioid cell neoplasm (PEComa) of the urinary bladder with TFE3 gene rearrangement: Clinicopathologic, immunohistochemical, and molecular features. The American Journal of Surgical Pathology, 37, 1619–1626.
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Raspollini, M.R., Lopez-Beltran, A. (2019). Perivascular Epithelioid Cell Tumor. In: van Krieken, J. (eds) Encyclopedia of Pathology. Encyclopedia of Pathology. Springer, Cham. https://doi.org/10.1007/978-3-319-28845-1_4895-1
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DOI: https://doi.org/10.1007/978-3-319-28845-1_4895-1
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