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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

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
Fabbroni C, Sbaraglia M, Sanfilippo R
Curr Opin Oncol 2020 Jul;32(4):301-306. doi: 10.1097/CCO.0000000000000649. PMID: 32541317
Italiano A, Delcambre C, Hostein I, Cazeau AL, Marty M, Avril A, Coindre JM, Bui B
Ann Oncol 2010 May;21(5):1135-7. Epub 2010 Mar 9 doi: 10.1093/annonc/mdq044. PMID: 20215136

Recent clinical studies

Etiology

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
Liu L, Dehner C, Grandhi N, Lyu Y, Borcherding DC, Chrisinger JSA, Zhang X, Luo J, Tao Y, Parkes A, Bui NQ, Davis EJ, Milhem MM, Monga V, Weiss M, Tine BV, Hirbe AC
Genes (Basel) 2022 Oct 24;13(11) doi: 10.3390/genes13111932. PMID: 36360169Free PMC Article
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
Shon W, Kim J, Sukov W, Reith J
Br J Dermatol 2016 Mar;174(3):617-20. Epub 2015 Nov 24 doi: 10.1111/bjd.14173. PMID: 26385848
Agaimy A, Wünsch PH
Pathol Res Pract 2006;202(1):37-41. Epub 2005 Dec 15 doi: 10.1016/j.prp.2005.10.008. PMID: 16343800

Diagnosis

Röhrl A, Hillmann A, Weber M
Dtsch Arztebl Int 2024 Mar 22;121(6):195. doi: 10.3238/arztebl.m2023.0017. PMID: 38666672Free PMC Article
Ascione A, Martignoni G, d'Amati G, Della Rocca C, Graziano P, Pernazza A
Pathologica 2022 Aug;114(4):312-315. Epub 2022 Sep 8 doi: 10.32074/1591-951X-762. PMID: 36083244Free PMC Article
Fabbroni C, Sbaraglia M, Sanfilippo R
Curr Opin Oncol 2020 Jul;32(4):301-306. doi: 10.1097/CCO.0000000000000649. PMID: 32541317
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
Machado I, Cruz J, Lavernia J, Rayon JM, Poveda A, Llombart-Bosch A
Int J Surg Pathol 2017 Sep;25(6):543-549. Epub 2017 May 1 doi: 10.1177/1066896917701245. PMID: 28459168

Therapy

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
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
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
Fabbroni C, Sbaraglia M, Sanfilippo R
Curr Opin Oncol 2020 Jul;32(4):301-306. doi: 10.1097/CCO.0000000000000649. PMID: 32541317
Machado I, Cruz J, Lavernia J, Rayon JM, Poveda A, Llombart-Bosch A
Int J Surg Pathol 2017 Sep;25(6):543-549. Epub 2017 May 1 doi: 10.1177/1066896917701245. PMID: 28459168

Prognosis

Testa S, Bui NQ, Ganjoo KN
Cancer Res Commun 2023 Jul;3(7):1212-1223. Epub 2023 Jul 12 doi: 10.1158/2767-9764.CRC-23-0139. PMID: 37448552Free PMC Article
Liu L, Dehner C, Grandhi N, Lyu Y, Borcherding DC, Chrisinger JSA, Zhang X, Luo J, Tao Y, Parkes A, Bui NQ, Davis EJ, Milhem MM, Monga V, Weiss M, Tine BV, Hirbe AC
Genes (Basel) 2022 Oct 24;13(11) doi: 10.3390/genes13111932. PMID: 36360169Free PMC Article
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
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
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

Clinical prediction guides

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
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
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
Liu L, Dehner C, Grandhi N, Lyu Y, Borcherding DC, Chrisinger JSA, Zhang X, Luo J, Tao Y, Parkes A, Bui NQ, Davis EJ, Milhem MM, Monga V, Weiss M, Tine BV, Hirbe AC
Genes (Basel) 2022 Oct 24;13(11) doi: 10.3390/genes13111932. PMID: 36360169Free PMC Article
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

Recent systematic reviews

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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