PEComa

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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.
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:

Clear cell sarcoma.

Images:

IHC

Melanocytic markers
- HMB-45.^[1]
- Melan A (Mart 1).
- Mitf.
Myogenic markers
- Calponin.
- Actin.^[1]
- Myosin.

See also

Tuberous sclerosis.

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/.

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