PEComa
PEComa is a family of tumours derived from perivascular epithelioid cells (PECs).
General
- Associated with abnormalities in TSC1 and TSC2 - the genes involved in tuberous sclerosis.[1]
The PEComa family
- Angiomyolipoma.
- Lymphangioleiomyomatosis.
- Clear-cell myomelanocytic tumour of ligamentum teres/falciform ligament.
- Abdominopelvic sarcoma of perivascular epitheloid cells.[2]
- Clear-cell sugar tumour (CCST).
- Primary extrapulmonary sugar tumour.
Microscopic
Features:[1]
- Epithelioid morphology.
- Clear or granular cytoplasm.
- Central oval (or round) nucleus.
- Indistinct/small nucleolus.
DDx:
Images:
IHC
See also
References
- ↑ 1.0 1.1 1.2 1.3 Martignoni G, Pea M, Reghellin D, Zamboni G, Bonetti F (February 2008). "PEComas: the past, the present and the future". Virchows Arch. 452 (2): 119–32. doi:10.1007/s00428-007-0509-1. PMC 2234444. PMID 18080139. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2234444/.
- ↑ Bonetti, F.; Martignoni, G.; Colato, C.; Manfrin, E.; Gambacorta, M.; Faleri, M.; Bacchi, C.; Sin, VC. et al. (Jun 2001). "Abdominopelvic sarcoma of perivascular epithelioid cells. Report of four cases in young women, one with tuberous sclerosis.". Mod Pathol 14 (6): 563-8. doi:10.1038/modpathol.3880351. PMID 11406657.