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Proximal-type epithelioid sarcoma

MedGen UID:
235511
•Concept ID:
C1335563
•
Neoplastic Process
Synonyms: Central Epithelioid Sarcoma; central epithelioid sarcoma; Large Cell Epithelioid Sarcoma; Large cell epithelioid sarcoma; Proximal epithelioid sarcoma; Proximal-Type Epithelioid Sarcoma; proximal-type epithelioid sarcoma; PTES
SNOMED CT: Proximal epithelioid sarcoma (1290726009); Proximal epithelioid sarcoma (1290886001); Large cell epithelioid sarcoma (1290726009); Large cell epithelioid sarcoma (1290886001)
 
Monarch Initiative: MONDO:0004244

Definition

An epithelioid sarcoma predominantly involving the pelvis, perineum, and genital organs. It tends to have a more aggressive clinical course as compared to the more frequently seen distal-type epithelioid sarcoma. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVProximal-type epithelioid sarcoma

Professional guidelines

PubMed

Treatment and outcomes of epithelioid sarcoma of the spine.
Babu R, Karikari IO, Cummings TJ, Gottfried ON, Bagley CA
J Clin Neurosci 2013 Oct;20(10):1342-5. Epub 2013 Apr 23 doi: 10.1016/j.jocn.2012.12.009. PMID: 23623614

Recent clinical studies

Etiology

Proximal-type epithelioid sarcoma: a new case report and literature review.
Echchaoui A, Sadrati Y, Elbir Y, Elktaibi A, Benyachou M, Mazouz SE, Gharib NE, Abbassi A
Pan Afr Med J 2016;24:238. Epub 2016 Jul 14 doi: 10.11604/pamj.2016.24.238.8535. PMID: 27800093Free PMC Article
Proximal-type epithelioid sarcoma in pubis. Case report and review of the literature.
Manzanares-Campillo Mdel C, Muñoz-Atienza V, Sánchez-García S, Gil-Rendo A, Jara-Sánchez A, Martín-Fernández J
Cir Cir 2011 Nov-Dec;79(6):560-3. PMID: 22169376
Radiologic manifestations of proximal-type epithelioid sarcoma of the soft tissues.
Tateishi U, Hasegawa T, Kusumoto M, Yokoyama R, Moriyama N
AJR Am J Roentgenol 2002 Oct;179(4):973-7. doi: 10.2214/ajr.179.4.1790973. PMID: 12239049
Proximal-type epithelioid sarcoma: a clinicopathologic study of 20 cases.
Hasegawa T, Matsuno Y, Shimoda T, Umeda T, Yokoyama R, Hirohashi S
Mod Pathol 2001 Jul;14(7):655-63. doi: 10.1038/modpathol.3880368. PMID: 11454997
"Proximal-type" epithelioid sarcoma, a distinctive aggressive neoplasm showing rhabdoid features. Clinicopathologic, immunohistochemical, and ultrastructural study of a series.
Guillou L, Wadden C, Coindre JM, Krausz T, Fletcher CD
Am J Surg Pathol 1997 Feb;21(2):130-46. doi: 10.1097/00000478-199702000-00002. PMID: 9042279

Diagnosis

Para-spermatic cord proximal-type epithelioid sarcoma.
Farnan J, Morrison E, Hennessey DB
BMJ Case Rep 2021 Jan 7;14(1) doi: 10.1136/bcr-2019-232385. PMID: 33414109Free PMC Article
Proximal-type epithelioid sarcoma of the oral cavity: a case report and literature review.
Liu Y, Sun B, Yang Y, Zhong L, He X, Wang M, Wang K, Chen L
Oral Surg Oral Med Oral Pathol Oral Radiol 2018 Nov;126(5):e258-e263. Epub 2018 Jun 21 doi: 10.1016/j.oooo.2018.05.049. PMID: 30087080
Proximal-type epithelioid sarcoma: a new case report and literature review.
Echchaoui A, Sadrati Y, Elbir Y, Elktaibi A, Benyachou M, Mazouz SE, Gharib NE, Abbassi A
Pan Afr Med J 2016;24:238. Epub 2016 Jul 14 doi: 10.11604/pamj.2016.24.238.8535. PMID: 27800093Free PMC Article
Proximal-type epithelioid sarcoma of the vulva with INI1 diagnostic utility.
Kim HJ, Kim MH, Kwon J, Kim JY, Park K, Ro JY
Ann Diagn Pathol 2012 Oct;16(5):411-5. Epub 2011 Jul 2 doi: 10.1016/j.anndiagpath.2011.04.002. PMID: 21724432
Proximal-type epithelioid sarcoma in pubis. Case report and review of the literature.
Manzanares-Campillo Mdel C, Muñoz-Atienza V, Sánchez-García S, Gil-Rendo A, Jara-Sánchez A, Martín-Fernández J
Cir Cir 2011 Nov-Dec;79(6):560-3. PMID: 22169376

Therapy

An unusual and potentially misleading phenotypic change in a primary gastrointestinal stromal tumour (GIST) under imatinib mesylate therapy.
Vassos N, Agaimy A, Schlabrakowski A, Hohenberger W, Schneider-Stock R, Croner RS
Virchows Arch 2011 Mar;458(3):363-9. Epub 2010 Dec 30 doi: 10.1007/s00428-010-1034-1. PMID: 21191613
Proximal-type epithelioid sarcoma of the maxilla with metastasis to the scalp: Cytologic findings of an unusual malignant tumor.
Uschuplich V, Gadre SA, Debski RF, Sahoo S
Diagn Cytopathol 2010 Oct;38(10):744-7. doi: 10.1002/dc.21304. PMID: 20058307
Proximal type epithelioid sarcoma of the scrotum: a source of diagnostic confusion that needs immediate attention: part 2.
Onol FF, Tanidir Y, Kotilŏglu E, Bayramiçli M, Turhal S, Türkeri LN
Eur Urol 2006 Mar;49(3):578-80. doi: 10.1016/j.eururo.2005.12.074. PMID: 16541484
'Proximal-type' epithelioid sarcoma: is Agent Orange still at large?
Chaudhuri A, Harris MD
Ann R Coll Surg Engl 2003 Nov;85(6):410-2. doi: 10.1308/003588403322520816. PMID: 14629885Free PMC Article

Prognosis

Para-spermatic cord proximal-type epithelioid sarcoma.
Farnan J, Morrison E, Hennessey DB
BMJ Case Rep 2021 Jan 7;14(1) doi: 10.1136/bcr-2019-232385. PMID: 33414109Free PMC Article
Proximal-Type Epithelioid Sarcoma: Report of an Unusual Case in the Uterine Cervix.
Suárez-Zamora DA, Barrera-Herrera LE, Rodríguez-Urrego PA, Palau-Lázaro MA
Int J Surg Pathol 2017 Aug;25(5):468-471. Epub 2017 Feb 1 doi: 10.1177/1066896917696744. PMID: 28381140
Proximal-type epithelioid sarcoma: a new case report and literature review.
Echchaoui A, Sadrati Y, Elbir Y, Elktaibi A, Benyachou M, Mazouz SE, Gharib NE, Abbassi A
Pan Afr Med J 2016;24:238. Epub 2016 Jul 14 doi: 10.11604/pamj.2016.24.238.8535. PMID: 27800093Free PMC Article
Proximal-type epithelioid sarcoma -- case report.
Santos LM, Nogueira L, Matsuo CY, Talhari C, Santos M
An Bras Dermatol 2013 May-Jun;88(3):444-7. doi: 10.1590/abd1806-4841.20131922. PMID: 23793215Free PMC Article
Proximal-type epithelioid sarcoma: a clinicopathologic study of 20 cases.
Hasegawa T, Matsuno Y, Shimoda T, Umeda T, Yokoyama R, Hirohashi S
Mod Pathol 2001 Jul;14(7):655-63. doi: 10.1038/modpathol.3880368. PMID: 11454997

Clinical prediction guides

Soft tissue tumours of the penis. The 30-year Istituto Nazionale Tumori di Milano experience.
Colecchia M, Pini GM, Pruneri G, Nicolai N, Servillo S
Pathologica 2024 Feb;116(1):46-54. doi: 10.32074/1591-951X-953. PMID: 38482674Free PMC Article
Proximal-Type Epithelioid Sarcoma in Skull Base: a Pathological Diagnosis Challenge with Other Intracranial Tumors.
Zejun D, Kun Y, Dehong L, Xueling Q
Pathol Oncol Res 2019 Jan;25(1):201-208. Epub 2017 Oct 26 doi: 10.1007/s12253-017-0318-z. PMID: 29076075
SMARCB1 expression in epithelioid sarcoma is regulated by miR-206, miR-381, and miR-671-5p on Both mRNA and protein levels.
Papp G, Krausz T, Stricker TP, Szendrői M, Sápi Z
Genes Chromosomes Cancer 2014 Feb;53(2):168-76. Epub 2013 Nov 5 doi: 10.1002/gcc.22128. PMID: 24327545
Proximal-type epithelioid sarcoma: a clinicopathologic study of 20 cases.
Hasegawa T, Matsuno Y, Shimoda T, Umeda T, Yokoyama R, Hirohashi S
Mod Pathol 2001 Jul;14(7):655-63. doi: 10.1038/modpathol.3880368. PMID: 11454997
"Proximal-type" epithelioid sarcoma, a distinctive aggressive neoplasm showing rhabdoid features. Clinicopathologic, immunohistochemical, and ultrastructural study of a series.
Guillou L, Wadden C, Coindre JM, Krausz T, Fletcher CD
Am J Surg Pathol 1997 Feb;21(2):130-46. doi: 10.1097/00000478-199702000-00002. PMID: 9042279

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