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Peripheral epithelioid sarcoma

MedGen UID:
232131
Concept ID:
C1333306
Neoplastic Process
Synonyms: Classic Epithelioid Sarcoma; Conventional Epithelioid Sarcoma; conventional epithelioid sarcoma; Distal-Type Epithelioid Sarcoma; distal-type epithelioid sarcoma; Peripheral Epithelioid Sarcoma; peripheral epithelioid sarcoma
 
Monarch Initiative: MONDO:0004060

Definition

An epithelioid sarcoma involving the extremities. It usually presents as nodular masses in the dermis and subcutaneous tissues or in the tendons and fascia. It frequently recurs and metastasizes to other anatomic sites. The most common sites of metastasis are the lungs, lymph nodes, bones, and brain. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVPeripheral epithelioid sarcoma

Professional guidelines

PubMed

Virgen CG, Kelkar N, Tran A, Rosa CM, Cruz-Topete D, Amatya S, Cornett EM, Urits I, Viswanath O, Kaye AD
Biomed Pharmacother 2022 Dec;156:113871. Epub 2022 Oct 19 doi: 10.1016/j.biopha.2022.113871. PMID: 36272265
Tariq MU, Din NU, Abdul-Ghafar J, Park YK
Diagn Pathol 2021 Apr 20;16(1):32. doi: 10.1186/s13000-021-01095-2. PMID: 33879215Free PMC Article
Spunt SL, Million L, Chi YY, Anderson J, Tian J, Hibbitts E, Coffin C, McCarville MB, Randall RL, Parham DM, Black JO, Kao SC, Hayes-Jordan A, Wolden S, Laurie F, Speights R, Kawashima E, Skapek SX, Meyer W, Pappo AS, Hawkins DS
Lancet Oncol 2020 Jan;21(1):145-161. Epub 2019 Nov 27 doi: 10.1016/S1470-2045(19)30672-2. PMID: 31786124Free PMC Article

Recent clinical studies

Etiology

Reed DR, Grohar P, Rubin E, Binitie O, Krailo M, Davis J, DuBois SG, Janeway KA
Pediatr Blood Cancer 2023 Sep;70 Suppl 6(Suppl 6):e30583. Epub 2023 Jul 27 doi: 10.1002/pbc.30583. PMID: 37501549Free PMC Article
Schaefer IM, Hong K, Kalbasi A
Am Soc Clin Oncol Educ Book 2020 May;40:445-462. doi: 10.1200/EDBK_280729. PMID: 32421448Free PMC Article
Gronchi A, Palmerini E, Quagliuolo V, Martin Broto J, Lopez Pousa A, Grignani G, Brunello A, Blay JY, Tendero O, Diaz Beveridge R, Ferraresi V, Lugowska I, Merlo DF, Fontana V, Marchesi E, Braglia L, Donati DM, Palassini E, Bianchi G, Marrari A, Morosi C, Stacchiotti S, Bagué S, Coindre JM, Dei Tos AP, Picci P, Bruzzi P, Casali PG
J Clin Oncol 2020 Jul 1;38(19):2178-2186. Epub 2020 May 18 doi: 10.1200/JCO.19.03289. PMID: 32421444
Spunt SL, Million L, Chi YY, Anderson J, Tian J, Hibbitts E, Coffin C, McCarville MB, Randall RL, Parham DM, Black JO, Kao SC, Hayes-Jordan A, Wolden S, Laurie F, Speights R, Kawashima E, Skapek SX, Meyer W, Pappo AS, Hawkins DS
Lancet Oncol 2020 Jan;21(1):145-161. Epub 2019 Nov 27 doi: 10.1016/S1470-2045(19)30672-2. PMID: 31786124Free PMC Article
Gronchi A, Ferrari S, Quagliuolo V, Broto JM, Pousa AL, Grignani G, Basso U, Blay JY, Tendero O, Beveridge RD, Ferraresi V, Lugowska I, Merlo DF, Fontana V, Marchesi E, Donati DM, Palassini E, Palmerini E, De Sanctis R, Morosi C, Stacchiotti S, Bagué S, Coindre JM, Dei Tos AP, Picci P, Bruzzi P, Casali PG
Lancet Oncol 2017 Jun;18(6):812-822. Epub 2017 May 9 doi: 10.1016/S1470-2045(17)30334-0. PMID: 28499583

Diagnosis

Reed DR, Grohar P, Rubin E, Binitie O, Krailo M, Davis J, DuBois SG, Janeway KA
Pediatr Blood Cancer 2023 Sep;70 Suppl 6(Suppl 6):e30583. Epub 2023 Jul 27 doi: 10.1002/pbc.30583. PMID: 37501549Free PMC Article
Stoneham S, Hunter A, Raahimi M, Livesey A, Mitchell CD, Keohane S
Clin Exp Dermatol 2023 Jul 21;48(8):866-872. doi: 10.1093/ced/llad166. PMID: 37127912
Kleinschmidt-DeMasters BK, Gilani A
Clin Neuropathol 2023 Mar-Apr;42(2):54-65. doi: 10.5414/NP301512. PMID: 36708209
Nacev BA, Jones KB, Intlekofer AM, Yu JSE, Allis CD, Tap WD, Ladanyi M, Nielsen TO
Nat Rev Cancer 2020 Oct;20(10):608-623. Epub 2020 Aug 11 doi: 10.1038/s41568-020-0288-4. PMID: 32782366Free PMC Article
Mavrogenis AF, Panagopoulos GN, Angelini A, Lesenský J, Vottis C, Megaloikonomos PD, Kokkalis ZT, Kontogeorgakos V, Ruggieri P, Papagelopoulos PJ
Eur J Orthop Surg Traumatol 2017 Aug;27(6):747-762. Epub 2017 Jun 5 doi: 10.1007/s00590-017-1984-y. PMID: 28585186

Therapy

Reed DR, Grohar P, Rubin E, Binitie O, Krailo M, Davis J, DuBois SG, Janeway KA
Pediatr Blood Cancer 2023 Sep;70 Suppl 6(Suppl 6):e30583. Epub 2023 Jul 27 doi: 10.1002/pbc.30583. PMID: 37501549Free PMC Article
Gronchi A, Palmerini E, Quagliuolo V, Martin Broto J, Lopez Pousa A, Grignani G, Brunello A, Blay JY, Tendero O, Diaz Beveridge R, Ferraresi V, Lugowska I, Merlo DF, Fontana V, Marchesi E, Braglia L, Donati DM, Palassini E, Bianchi G, Marrari A, Morosi C, Stacchiotti S, Bagué S, Coindre JM, Dei Tos AP, Picci P, Bruzzi P, Casali PG
J Clin Oncol 2020 Jul 1;38(19):2178-2186. Epub 2020 May 18 doi: 10.1200/JCO.19.03289. PMID: 32421444
Spunt SL, Million L, Chi YY, Anderson J, Tian J, Hibbitts E, Coffin C, McCarville MB, Randall RL, Parham DM, Black JO, Kao SC, Hayes-Jordan A, Wolden S, Laurie F, Speights R, Kawashima E, Skapek SX, Meyer W, Pappo AS, Hawkins DS
Lancet Oncol 2020 Jan;21(1):145-161. Epub 2019 Nov 27 doi: 10.1016/S1470-2045(19)30672-2. PMID: 31786124Free PMC Article
Acosta AE, Vélez CS
Curr Treat Options Oncol 2017 Aug 10;18(9):56. doi: 10.1007/s11864-017-0498-5. PMID: 28795284
Gronchi A, Ferrari S, Quagliuolo V, Broto JM, Pousa AL, Grignani G, Basso U, Blay JY, Tendero O, Beveridge RD, Ferraresi V, Lugowska I, Merlo DF, Fontana V, Marchesi E, Donati DM, Palassini E, Palmerini E, De Sanctis R, Morosi C, Stacchiotti S, Bagué S, Coindre JM, Dei Tos AP, Picci P, Bruzzi P, Casali PG
Lancet Oncol 2017 Jun;18(6):812-822. Epub 2017 May 9 doi: 10.1016/S1470-2045(17)30334-0. PMID: 28499583

Prognosis

Tlemsani C, Larousserie F, De Percin S, Audard V, Hadjadj D, Chen J, Biau D, Anract P, Terris B, Goldwasser F, Pasmant E, Boudou-Rouquette P
Int J Mol Sci 2023 Jan 10;24(2) doi: 10.3390/ijms24021361. PMID: 36674874Free PMC Article
Schaefer IM, Hong K, Kalbasi A
Am Soc Clin Oncol Educ Book 2020 May;40:445-462. doi: 10.1200/EDBK_280729. PMID: 32421448Free PMC Article
Spunt SL, Million L, Chi YY, Anderson J, Tian J, Hibbitts E, Coffin C, McCarville MB, Randall RL, Parham DM, Black JO, Kao SC, Hayes-Jordan A, Wolden S, Laurie F, Speights R, Kawashima E, Skapek SX, Meyer W, Pappo AS, Hawkins DS
Lancet Oncol 2020 Jan;21(1):145-161. Epub 2019 Nov 27 doi: 10.1016/S1470-2045(19)30672-2. PMID: 31786124Free PMC Article
Nugent M
Adv Exp Med Biol 2015;889:201-30. doi: 10.1007/978-3-319-23730-5_11. PMID: 26659003
Stein-Wexler R
Semin Ultrasound CT MR 2011 Oct;32(5):470-88. doi: 10.1053/j.sult.2011.03.006. PMID: 21963167

Clinical prediction guides

Schaefer IM, Hornick JL
Semin Diagn Pathol 2021 May;38(3):222-231. Epub 2020 Jun 5 doi: 10.1053/j.semdp.2020.05.005. PMID: 32646614Free PMC Article
Schaefer IM, Hong K, Kalbasi A
Am Soc Clin Oncol Educ Book 2020 May;40:445-462. doi: 10.1200/EDBK_280729. PMID: 32421448Free PMC Article
Gronchi A, Palmerini E, Quagliuolo V, Martin Broto J, Lopez Pousa A, Grignani G, Brunello A, Blay JY, Tendero O, Diaz Beveridge R, Ferraresi V, Lugowska I, Merlo DF, Fontana V, Marchesi E, Braglia L, Donati DM, Palassini E, Bianchi G, Marrari A, Morosi C, Stacchiotti S, Bagué S, Coindre JM, Dei Tos AP, Picci P, Bruzzi P, Casali PG
J Clin Oncol 2020 Jul 1;38(19):2178-2186. Epub 2020 May 18 doi: 10.1200/JCO.19.03289. PMID: 32421444
Spunt SL, Million L, Chi YY, Anderson J, Tian J, Hibbitts E, Coffin C, McCarville MB, Randall RL, Parham DM, Black JO, Kao SC, Hayes-Jordan A, Wolden S, Laurie F, Speights R, Kawashima E, Skapek SX, Meyer W, Pappo AS, Hawkins DS
Lancet Oncol 2020 Jan;21(1):145-161. Epub 2019 Nov 27 doi: 10.1016/S1470-2045(19)30672-2. PMID: 31786124Free PMC Article
Nugent M
Adv Exp Med Biol 2015;889:201-30. doi: 10.1007/978-3-319-23730-5_11. PMID: 26659003

Recent systematic reviews

Kuhlmann C, Ehrl D, Taha S, Wachtel N, Schmid A, Bronsert P, Zeller J, Giunta RE, Eisenhardt SU, Braig D
Surgery 2023 Jun;173(6):1463-1475. Epub 2023 Apr 1 doi: 10.1016/j.surg.2023.02.026. PMID: 37012145
Schuch LF, Kirschnick LB, de Arruda JAA, Klein IP, Silveira FM, Vasconcelos ACU, Santos-Silva AR, Lopes MA, Carrard VC, Vargas PA, Martins MAT, Wagner VP, Martins MD
Oral Dis 2022 Nov;28(8):2072-2082. Epub 2021 Aug 12 doi: 10.1111/odi.13982. PMID: 34333825
Cai Z, Tang X, Liang H, Yang R, Yan T, Guo W
World J Surg Oncol 2020 Sep 30;18(1):257. doi: 10.1186/s12957-020-02036-x. PMID: 32998743Free PMC Article
Burks SS, Puffer RC, Cajigas I, Valdivia D, Rosenberg AE, Spinner RJ, Levi AD
Neurosurgery 2019 Dec 1;85(6):E975-E991. doi: 10.1093/neuros/nyz321. PMID: 31435657Free PMC Article
Cherif El Asri A, Benzagmout M, Chakour K, Chaoui MF, Laaguili J, Chahdi H, Gazzaz M, El Mostarchid B
World Neurosurg 2018 Jul;115:346-356. Epub 2018 May 3 doi: 10.1016/j.wneu.2018.04.164. PMID: 29729469

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