• Patient

    Generally appears during middle age, with a female predominance3

  • Microscope

    Consists of epithelioid cells that are associated with blood vessel walls and usually express both melanocytic and smooth muscle markers4

  • Map marker

    Malignant PEComas demonstrate local invasion and/or metastatic spread1

  • Earth

    Globally, ≤1 diagnosis per 1,000,000 people annually5

May appear anywhere in the body, most frequently at visceral, retroperitoneal, and abdominopelvic sites.3,6-9

PEcoma sites


Misdiagnosis may occur due to overlapping immunohistochemical and cytogenetic markers with other sarcomas and solid tumors1,2,10

Misdiagnoses include1,2,10

— Leiomyosarcoma

— Malignant melanoma
— Unclassified pleomorphic sarcoma
— Rhabdomyosarcoma


Advanced malignant PEComa is associated with a poor prognosis11

Up to 72% of patients with malignant PEComas develop metastatic disease within 12 months1,4,10

Median survival in advanced malignant (metastatic or unresectable) PEComa is estimated at 16 months4*

*Median survival of 16 months reflects data gathered from (n=7) patients who succumbed to disease with unresectable or metastatic malignant PEComa. Analysis was sourced from reported cases found in a literature review via a search of the PubMed database.4

The Role of mTOR

PEcoma cell

The Role of mTOR

  • Advanced malignant PEComa is primarily an mTOR-driven disease1
  • Overactivation of the mTOR pathway leads to uncontrolled cell growth and survival1,12
  • PEComas commonly have alterations in TSC1 or TSC2 genes1,13

GIST=gastrointestinal stromal tumor; mTOR=mechanistic target of rapamycin; RCC=renal cell carcinoma; TSC1=tuberous sclerosis complex-1; TSC2=tuberous sclerosis complex-2.

References: 1. Ben-Ami E, Hornick JL, Wagner H, Wagner AJ. The potential of emerging new therapeutics for the treatment of perivascular epithelioid cell tumors (PEComa). Expert Opin Orphan Drugs. 2018;6(9):537-543. 2. Chen Z, Han S, Wu J, et al. A systematic review: perivascular epithelioid cell tumor of gastrointestinal tract. Medicine (Baltimore). 2016;95(28):e3890. doi:10.1097/MD.0000000000003890 3. Sanfilippo R, Jones RL, Blay JY, et al. Role of chemotherapy, VEGFR inhibitors, and mTOR inhibitors in advanced perivascular epithelioid cell tumors (PEComas). Clin Cancer Res. 2019;25(17):5295-5300. doi:10.1158/1078-0432.CCR-19-0288 4. Bleeker JS, Quevedo JF, Folpe AL. "Malignant" perivascular epithelioid cell neoplasm: risk stratification and treatment strategies. Sarcoma. 2012;2012:541626. doi:10.1155/2012/541626 5. Stacchiotti S, Frezza AM, Blay JY, et al. Ultra-rare sarcomas: a consensus paper from the Connective Tissue Oncology Society community of experts on the incidence threshold and the list of entities. Cancer. 2021;127(16):2934-2942. doi:10.1002/cncr.33618 6. Wagner AJ, Ravi V, Riedel RF, et al. nab-Sirolimus for patients with malignant perivascular epithelioid cell tumors. J Clin Oncol. 2021;39(33):3660-3670. doi:10.1200/JCO.21.01728 7. Bourgmayer A, Nannini S, Bonjean P, Kurtz JE, Malouf GG, Gantzer J. Natural history and treatment strategies of advanced PEComas: a systematic review. Cancers (Basel). 2021;13(20):5227. doi:10.3390/cancers13205227 8. What is sarcoma? Sarcoma Foundation of America. Accessed March 8, 2023. 9. Zhang S, Chen F, Huang X, et al. Perivascular epithelial cell tumor (PEComa) of the pancreas: a case report and review of literature. Medicine (Baltimore). 2017;96(22):e7050. doi:10.1097/MD.0000000000007050 10. Tirumani SH, Shinagare AB, Hargreaves J, et al. Imaging features of primary and metastatic malignant perivascular epithelioid cell tumors. AJR Am J Roentgenol. 2014;202(2):252-258. doi:10.2214/AJR.13.10909 11. Armah HB, Parwani AV. Perivascular epithelioid cell tumor. Arch Pathol Lab Med. 2009;133(4):648-654. doi:10.5858/133.4.648 12. Gonzalez-Angulo AM, Meric-Bernstam F, Chawla S, et al. Weekly nab-rapamycin in patients with advanced nonhematologic malignancies: final results of a phase I trial. Clin Cancer Res. 2013;19(19):5474-5484. doi:10.1158/1078-0432.CCR-12-3110 13. Agaram NP, Sung YS, Zhang L, et al. Dichotomy of genetic abnormalities in PEComas with therapeutic implications. Am J Surg Pathol. 2015;39(6):813-825. doi:10.1097/PAS.0000000000000389