Overview

NCI Definition: A soft tissue mesenchymal tumor with perivascular epithelioid cell differentiation. Representative examples include angiomyolipoma, clear cell-sugar-tumor of the lung, and lymphangioleiomyomatosis. [1]

Significant Genes in PEComa

AKT1 +

AKT2 +

AKT3 +

CRKL +

EGFR +

ERBB2 +

IRS2 +

KIT +

MET +

MLH1 +

MLH3 +

MSH2 +

MSH3 +

MSH6 +

MTOR +

PDGFRA +

PIK3CA +

PIK3CG +

PIK3R1 +

PIK3R2 +

PMS1 +

PMS2 +

PTEN +

RICTOR +

RNF43 +

RPTOR +

TSC1 +

TSC2 +

Disease Details

Synonyms
Neoplasm with Perivascular Epithelioid Cell Differentiation, Tumor with Perivascular Epithelioid Cell Differentiation, Perivascular Epithelioid Cell Neoplasm
Parent(s)
Soft Tissue Neoplasm
Children
Angiomyolipoma, Lymphangioleiomyomatosis, and Uterine Corpus PEComa
OncoTree Name
Perivascular Epithelioid Cell Tumor
OncoTree Code
PECOMA

References

1. National Cancer Institute. NCI Thesaurus Version 18.11d. https://ncit.nci.nih.gov/ncitbrowser/ [2018-08-28]. [2018-09-21].

2. The AACR Project GENIE Consortium. AACR Project GENIE: powering precision medicine through an international consortium. Cancer Discovery. 2017;7(8):818-831. Dataset Version 6. This dataset does not represent the totality of the genetic landscape; see paper for more information.

3. All assertions and clinical trial landscape data are curated from primary sources. You can read more about the curation process here.