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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

Pharmacological management of cancer pain: Novel therapeutics.
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
The many faces of solitary fibrous tumor; diversity of histological features, differential diagnosis and role of molecular studies and surrogate markers in avoiding misdiagnosis and predicting the behavior.
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
A risk-based treatment strategy for non-rhabdomyosarcoma soft-tissue sarcomas in patients younger than 30 years (ARST0332): a Children's Oncology Group prospective study.
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

Dermatofibrosarcoma Protuberans in Children.
Sleiwah A, Wright TC, Chapman T, Dangoor A, Maggiani F, Clancy R
Curr Treat Options Oncol 2022 Jun;23(6):843-854. Epub 2022 Apr 8 doi: 10.1007/s11864-022-00979-9. PMID: 35394606
Neoadjuvant Chemotherapy in High-Risk Soft Tissue Sarcomas: Final Results of a Randomized Trial From Italian (ISG), Spanish (GEIS), French (FSG), and Polish (PSG) Sarcoma Groups.
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
A risk-based treatment strategy for non-rhabdomyosarcoma soft-tissue sarcomas in patients younger than 30 years (ARST0332): a Children's Oncology Group prospective study.
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
Histotype-tailored neoadjuvant chemotherapy versus standard chemotherapy in patients with high-risk soft-tissue sarcomas (ISG-STS 1001): an international, open-label, randomised, controlled, phase 3, multicentre trial.
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
microRNAs and Soft Tissue Sarcomas.
Fujiwara T, Kunisada T, Takeda K, Ozaki T
Adv Exp Med Biol 2015;889:179-99. doi: 10.1007/978-3-319-23730-5_10. PMID: 26659002

Diagnosis

Sarcoma of the Lung and Mediastinum.
Yoshida KI, Yoshida A
Surg Pathol Clin 2024 Jun;17(2):243-255. Epub 2023 Dec 15 doi: 10.1016/j.path.2023.11.008. PMID: 38692808
The epigenomics of sarcoma.
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
Tumors of the hand.
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
Cutaneous Malignant Peripheral Nerve Sheath Tumor.
Luzar B, Falconieri G
Surg Pathol Clin 2017 Jun;10(2):337-343. Epub 2017 Mar 14 doi: 10.1016/j.path.2017.01.008. PMID: 28477884
Pediatric soft tissue sarcomas.
Stein-Wexler R
Semin Ultrasound CT MR 2011 Oct;32(5):470-88. doi: 10.1053/j.sult.2011.03.006. PMID: 21963167

Therapy

Children's Oncology Group's 2023 blueprint for research: Bone tumors.
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
Neoadjuvant Chemotherapy in High-Risk Soft Tissue Sarcomas: Final Results of a Randomized Trial From Italian (ISG), Spanish (GEIS), French (FSG), and Polish (PSG) Sarcoma Groups.
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
A risk-based treatment strategy for non-rhabdomyosarcoma soft-tissue sarcomas in patients younger than 30 years (ARST0332): a Children's Oncology Group prospective study.
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
Dermatofibrosarcoma Protuberans.
Acosta AE, Vélez CS
Curr Treat Options Oncol 2017 Aug 10;18(9):56. doi: 10.1007/s11864-017-0498-5. PMID: 28795284
Histotype-tailored neoadjuvant chemotherapy versus standard chemotherapy in patients with high-risk soft-tissue sarcomas (ISG-STS 1001): an international, open-label, randomised, controlled, phase 3, multicentre trial.
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

A risk-based treatment strategy for non-rhabdomyosarcoma soft-tissue sarcomas in patients younger than 30 years (ARST0332): a Children's Oncology Group prospective study.
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
microRNA and Bone Cancer.
Nugent M
Adv Exp Med Biol 2015;889:201-30. doi: 10.1007/978-3-319-23730-5_11. PMID: 26659003
microRNAs and Soft Tissue Sarcomas.
Fujiwara T, Kunisada T, Takeda K, Ozaki T
Adv Exp Med Biol 2015;889:179-99. doi: 10.1007/978-3-319-23730-5_10. PMID: 26659002
Diagnostically Challenging Epithelioid Soft Tissue Tumors.
James AW, Dry SM
Surg Pathol Clin 2015 Sep;8(3):309-29. doi: 10.1016/j.path.2015.05.002. PMID: 26297059
Pediatric soft tissue sarcomas.
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

SWI/SNF complex-deficient soft tissue neoplasms: An update.
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
Neoadjuvant Chemotherapy in High-Risk Soft Tissue Sarcomas: Final Results of a Randomized Trial From Italian (ISG), Spanish (GEIS), French (FSG), and Polish (PSG) Sarcoma Groups.
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
A risk-based treatment strategy for non-rhabdomyosarcoma soft-tissue sarcomas in patients younger than 30 years (ARST0332): a Children's Oncology Group prospective study.
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
microRNA and Bone Cancer.
Nugent M
Adv Exp Med Biol 2015;889:201-30. doi: 10.1007/978-3-319-23730-5_11. PMID: 26659003
microRNAs and Soft Tissue Sarcomas.
Fujiwara T, Kunisada T, Takeda K, Ozaki T
Adv Exp Med Biol 2015;889:179-99. doi: 10.1007/978-3-319-23730-5_10. PMID: 26659002

Recent systematic reviews

(18) F-FDG PET and PET/CT as a diagnostic method for Ewing sarcoma: A systematic review and meta-analysis.
Seth N, Seth I, Bulloch G, Siu AHY, Guo A, Chatterjee R, MacManus M, Donnan L
Pediatr Blood Cancer 2022 Mar;69(3):e29415. Epub 2021 Oct 28 doi: 10.1002/pbc.29415. PMID: 34709700
Malignant peripheral nerve sheath tumour of the oral and maxillofacial region-A systematic review.
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
Prognosis and risk factors for malignant peripheral nerve sheath tumor: a systematic review and meta-analysis.
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
Synovial Sarcoma of the Nerve-Clinical and Pathological Features: Case Series and Systematic Review.
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
Primary Intracranial pPNET/Ewing Sarcoma: Diagnosis, Management, and Prognostic Factors Dilemma-A Systematic Review of the Literature.
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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