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Malignant PEComa

MedGen UID:
824908
•Concept ID:
C3839062
•
Neoplastic Process
Synonyms: PEComa, malignant; Perivascular epithelioid tumor, malignant
SNOMED CT: Perivascular epithelioid tumor, malignant (703605001); PEComa, malignant (703605001)

Definition

A usually large and aggressive tumor with perivascular epithelioid cell differentiation characterized by the presence of marked nuclear atypia, pleomorphism, increased mitotic activity, necrosis, and infiltrative margins. The most common metastatic sites are liver, lungs, lymph nodes, and bone. [from NCI]

Professional guidelines

PubMed

Addition of Antiestrogen Treatment in Patients with Malignant PEComa Progressing to mTOR Inhibitors.
Sanfilippo R, Fabbroni C, Fucà G, Fumagalli E, Morosi C, Sbaraglia M, Gronchi A, Collini P, Dei Tos AP, Casali PG
Clin Cancer Res 2020 Oct 15;26(20):5534-5538. Epub 2020 Jun 30 doi: 10.1158/1078-0432.CCR-20-1191. PMID: 32605908
Medical treatment of advanced malignant perivascular epithelioid cell tumors.
Fabbroni C, Sbaraglia M, Sanfilippo R
Curr Opin Oncol 2020 Jul;32(4):301-306. doi: 10.1097/CCO.0000000000000649. PMID: 32541317
A retrospective study of patients with malignant PEComa receiving treatment with sirolimus or temsirolimus: the Royal Marsden Hospital experience.
Benson C, Vitfell-Rasmussen J, Maruzzo M, Fisher C, Tunariu N, Mitchell S, Al-Muderis O, Thway K, Larkin J, Judson I
Anticancer Res 2014 Jul;34(7):3663-8. PMID: 24982384

Recent clinical studies

Etiology

A Clinicopathologic and Molecular Characterization of Uterine Sarcomas Classified as Malignant PEComa.
Anderson WJ, Dong F, Fletcher CDM, Hirsch MS, Nucci MR
Am J Surg Pathol 2023 May 1;47(5):535-546. Epub 2023 Mar 1 doi: 10.1097/PAS.0000000000002028. PMID: 36856023
nab-Sirolimus for Patients With Malignant Perivascular Epithelioid Cell Tumors.
Wagner AJ, Ravi V, Riedel RF, Ganjoo K, Van Tine BA, Chugh R, Cranmer L, Gordon EM, Hornick JL, Du H, Grigorian B, Schmid AN, Hou S, Harris K, Kwiatkowski DJ, Desai NP, Dickson MA
J Clin Oncol 2021 Nov 20;39(33):3660-3670. Epub 2021 Oct 12 doi: 10.1200/JCO.21.01728. PMID: 34637337Free PMC Article
Dia-gnostic Challenges and Extraordinary Treatment Response in Rare Malignant PEComa Tumor of the Kidney.
Huľová S, Sycova-Mila Z, Macák D, Janega P, Chovanec M, Mardiak J, Mego M
Klin Onkol 2018 Fall;31(6):448-452. doi: 10.14735/amko2018448. PMID: 30545225
Update on the imaging of malignant perivascular epithelioid cell tumors (PEComas).
Phillips CH, Keraliya AR, Shinagare AB, Ramaiya NH, Tirumani SH
Abdom Radiol (NY) 2016 Feb;41(2):368-76. doi: 10.1007/s00261-015-0568-8. PMID: 26867923
Imaging features of primary and metastatic malignant perivascular epithelioid cell tumors.
Tirumani SH, Shinagare AB, Hargreaves J, Jagannathan JP, Hornick JL, Wagner AJ, Ramaiya NH
AJR Am J Roentgenol 2014 Feb;202(2):252-8. doi: 10.2214/AJR.13.10909. PMID: 24450662

Diagnosis

A Clinicopathologic and Molecular Characterization of Uterine Sarcomas Classified as Malignant PEComa.
Anderson WJ, Dong F, Fletcher CDM, Hirsch MS, Nucci MR
Am J Surg Pathol 2023 May 1;47(5):535-546. Epub 2023 Mar 1 doi: 10.1097/PAS.0000000000002028. PMID: 36856023
Uterine PEComas: correlation between melanocytic marker expression and TSC alterations/TFE3 fusions.
Bennett JA, Ordulu Z, Pinto A, Wanjari P, Antonescu CR, Ritterhouse LL, Oliva E
Mod Pathol 2022 Apr;35(4):515-523. Epub 2021 Jun 15 doi: 10.1038/s41379-021-00855-1. PMID: 34131293Free PMC Article
Medical treatment of advanced malignant perivascular epithelioid cell tumors.
Fabbroni C, Sbaraglia M, Sanfilippo R
Curr Opin Oncol 2020 Jul;32(4):301-306. doi: 10.1097/CCO.0000000000000649. PMID: 32541317
Ischiorectal fossa: benign and malignant neoplasms of this "ignored" radiological anatomical space.
Faria SC, Elsherif SB, Sagebiel T, Cox V, Rao B, Lall C, Bhosale PR
Abdom Radiol (NY) 2019 May;44(5):1644-1674. doi: 10.1007/s00261-019-01930-7. PMID: 30955068
Update on the imaging of malignant perivascular epithelioid cell tumors (PEComas).
Phillips CH, Keraliya AR, Shinagare AB, Ramaiya NH, Tirumani SH
Abdom Radiol (NY) 2016 Feb;41(2):368-76. doi: 10.1007/s00261-015-0568-8. PMID: 26867923

Therapy

Gynecologic perivascular epithelioid cell tumors (PEComas): a review of recent evidence.
Levin G, Capella MP, Meyer R, Brezinov Y, Gotlieb WH
Arch Gynecol Obstet 2024 Jun;309(6):2381-2386. Epub 2024 Apr 25 doi: 10.1007/s00404-024-07510-5. PMID: 38664269Free PMC Article
Phase II Trial of nab-Sirolimus in Patients With Advanced Malignant Perivascular Epithelioid Cell Tumors (AMPECT): Long-Term Efficacy and Safety Update.
Wagner AJ, Ravi V, Riedel RF, Ganjoo K, Van Tine BA, Chugh R, Cranmer L, Gordon EM, Hornick JL, Du H, Ding L, Schmid AN, Navarro WH, Kwiatkowski DJ, Dickson MA
J Clin Oncol 2024 May 1;42(13):1472-1476. Epub 2024 Mar 1 doi: 10.1200/JCO.23.02266. PMID: 38427923Free PMC Article
nab-Sirolimus for Patients With Malignant Perivascular Epithelioid Cell Tumors.
Wagner AJ, Ravi V, Riedel RF, Ganjoo K, Van Tine BA, Chugh R, Cranmer L, Gordon EM, Hornick JL, Du H, Grigorian B, Schmid AN, Hou S, Harris K, Kwiatkowski DJ, Desai NP, Dickson MA
J Clin Oncol 2021 Nov 20;39(33):3660-3670. Epub 2021 Oct 12 doi: 10.1200/JCO.21.01728. PMID: 34637337Free PMC Article
Medical treatment of advanced malignant perivascular epithelioid cell tumors.
Fabbroni C, Sbaraglia M, Sanfilippo R
Curr Opin Oncol 2020 Jul;32(4):301-306. doi: 10.1097/CCO.0000000000000649. PMID: 32541317
Dia-gnostic Challenges and Extraordinary Treatment Response in Rare Malignant PEComa Tumor of the Kidney.
Huľová S, Sycova-Mila Z, Macák D, Janega P, Chovanec M, Mardiak J, Mego M
Klin Onkol 2018 Fall;31(6):448-452. doi: 10.14735/amko2018448. PMID: 30545225

Prognosis

Uterine PEComas: correlation between melanocytic marker expression and TSC alterations/TFE3 fusions.
Bennett JA, Ordulu Z, Pinto A, Wanjari P, Antonescu CR, Ritterhouse LL, Oliva E
Mod Pathol 2022 Apr;35(4):515-523. Epub 2021 Jun 15 doi: 10.1038/s41379-021-00855-1. PMID: 34131293Free PMC Article
nab-Sirolimus for Patients With Malignant Perivascular Epithelioid Cell Tumors.
Wagner AJ, Ravi V, Riedel RF, Ganjoo K, Van Tine BA, Chugh R, Cranmer L, Gordon EM, Hornick JL, Du H, Grigorian B, Schmid AN, Hou S, Harris K, Kwiatkowski DJ, Desai NP, Dickson MA
J Clin Oncol 2021 Nov 20;39(33):3660-3670. Epub 2021 Oct 12 doi: 10.1200/JCO.21.01728. PMID: 34637337Free PMC Article
Malignant Pleural PEComa: A Previously Undocumented Site.
Au RTM, Ahmed ADB, Ho BCS, Teo CHY, Chuah KL
Int J Surg Pathol 2020 Aug;28(5):549-552. Epub 2019 Dec 22 doi: 10.1177/1066896919893074. PMID: 31865805
Primary Ovarian Malignant PEComa: A Case Report.
Westaby JD, Magdy N, Fisher C, El-Bahrawy M
Int J Gynecol Pathol 2017 Jul;36(4):400-404. doi: 10.1097/PGP.0000000000000331. PMID: 27684885
Update on the imaging of malignant perivascular epithelioid cell tumors (PEComas).
Phillips CH, Keraliya AR, Shinagare AB, Ramaiya NH, Tirumani SH
Abdom Radiol (NY) 2016 Feb;41(2):368-76. doi: 10.1007/s00261-015-0568-8. PMID: 26867923

Clinical prediction guides

Clinical and ultrasound features of uterine perivascular epithelioid cell tumors: case series and literature review.
Wang R, Luo H, Cao W
Ultrasound Obstet Gynecol 2024 Nov;64(5):687-695. Epub 2024 Oct 12 doi: 10.1002/uog.29116. PMID: 39395194
A retrospective clinical analysis of 11 cases of PEComa from different sites.
Yan J, Zhou D, Wang Y, Yang B, Wang Y, Zhang K, Zhang S, Zhang B, Meng Q, Lv Q
World J Surg Oncol 2024 Apr 30;22(1):116. doi: 10.1186/s12957-024-03349-x. PMID: 38689335Free PMC Article
A Clinicopathologic and Molecular Characterization of Uterine Sarcomas Classified as Malignant PEComa.
Anderson WJ, Dong F, Fletcher CDM, Hirsch MS, Nucci MR
Am J Surg Pathol 2023 May 1;47(5):535-546. Epub 2023 Mar 1 doi: 10.1097/PAS.0000000000002028. PMID: 36856023
Characteristics of mismatch repair deficiency in sarcomas.
Doyle LA, Nowak JA, Nathenson MJ, Thornton K, Wagner AJ, Johnson JM, Albrayak A, George S, Sholl LM
Mod Pathol 2019 Jul;32(7):977-987. Epub 2019 Feb 14 doi: 10.1038/s41379-019-0202-3. PMID: 30765880
Malignant perivascular epithelioid cell tumor in children: description of a case and review of the literature.
Alaggio R, Cecchetto G, Martignoni G, Bisogno G, Cheng L, Sperlì D, d'Amore ES, Dall'Igna P
J Pediatr Surg 2012 Jun;47(6):e31-40. doi: 10.1016/j.jpedsurg.2012.02.023. PMID: 22703822

Recent systematic reviews

Gynecologic perivascular epithelioid cell tumors (PEComas): a review of recent evidence.
Levin G, Capella MP, Meyer R, Brezinov Y, Gotlieb WH
Arch Gynecol Obstet 2024 Jun;309(6):2381-2386. Epub 2024 Apr 25 doi: 10.1007/s00404-024-07510-5. PMID: 38664269Free PMC Article

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