Epithelioid Sarcoma

Common Name(s)

Epithelioid Sarcoma

Epithelioid sarcoma is a rare cancer that most often occurs in the soft tissue of the fingers, hands and forearms of young adults.   It may also be found in the legs, trunk, head or neck regions.  It is rare in young children and adults, and it occurs more frequently in men.   Epithelioid sarcoma begins as a painless, firm growth or bump that may be accompanied by an open wound (ulceration) in the skin covering the growth.   It is considered an aggressive cancer because it has a high chance of regrowing after treatment (a recurrence), or spreading to surrounding tissues or more distant parts of the body (a metastasis).   Epithelioid sarcoma is first treated with surgery to remove all the cancer cells (wide local excision).   Amputation of part of the affected limb may be needed in severe cases.   Radiation therapy or chemotherapy may also be used to destroy any cancer cells not removed during surgery.
 

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Advocacy and Support Organizations

 

Condition Specific Organizations

Following organizations serve the condition "Epithelioid Sarcoma" for support, advocacy or research.

There are currently no organizations listed in Disease InfoSearch that support this condition. Create a listing.

 

 

General Support Organizations

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Scientific Literature

Articles from the PubMed Database

Research articles describe the outcome of a single study. They are the published results of original research.
The terms "Epithelioid Sarcoma" returned 88 free, full-text research articles on human participants. First 3 results:

Epithelioid sarcoma arising from the temporal space: A case report.
 

Author(s): Yong Seek Kim, Hyo Sung Kwak, Gyung Ho Chung, Yo Na Kim, Seungbae Hwang

Journal: Medicine (Baltimore). 2018 Sep;97(38):e12529.

 

Epithelioid sarcoma is a malignant soft tissue tumor arising from mesenchymal tissue and usually occurs in the extremities. The tumor involving the head and neck region is extremely rare. We present radiologically well-documented case of an epithelioid sarcoma arising from the temporal space.

Last Updated: 31 Dec 1969

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Long period of relative quiescence in distal-type epithelioid sarcoma of the forearm with recurrence after surgery: A case report.
 

Author(s): Xiupeng Zhang, Biying Jiang, Xiuying Shi, Chuifeng Fan

Journal: Medicine (Baltimore). 2018 Sep;97(36):e12276.

 

Epithelioid sarcoma (ES) is a rare malignant mesenchymal tumor that only accounts for 0.6% to 1.0% of all cases of sarcomas. ES with a relative quiescent state of more than 10 years is extremely rare.Here, we present a rare case of ES in the forearm of a 17-year-old girl. The patient ...

Last Updated: 31 Dec 1969

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Pseudomyogenic (epithelioid sarcoma-like) hemangioendothelioma - a rare vascular neoplasm with deceptive morphology and distinctive immunophenotype.
 

Author(s): F F Cheo, K Sittampalam

Journal: Malays J Pathol. 2017 Dec;39(3):305-309.

 

Pseudomyogenic (epithelioid sarcoma-like) hemangioendothelioma is a rare, low grade vascular (endothelial) neoplasm typically presenting as multicentric, superficial to deep nodules in extremities with a slight tendency of affecting young adult males. We report a case of pseudomyogenic ...

Last Updated: 31 Dec 1969

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Reviews from the PubMed Database

Review articles summarize what is currently known about a disease. They discuss research previously published by others.
The terms "Epithelioid Sarcoma" returned 13 free, full-text review articles on human participants. First 3 results:

[Pleural epithelioid sarcoma: about a case and review of the literature].
 

Author(s): Hajar Ouahbi, Youssra Akasbi, Karima Oualla, Bouchra Amara, Achraf Chatar, Siham Tizniti, Hind Fatemi, Fatima Zahra Lemrabet, Samia Arifi, Nawfal Mellas

Journal:

 

Proximal epithelioid sarcoma (PES) originating from the pleura is a clinical entity rarely reported in the literature. We report the case of a young patient with immediately metastatic proximal epithelioid sarcoma (PES) treated at the Department of Medical Oncology, Fes. Treatment ...

Last Updated: 31 Dec 1969

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ALK oncoproteins in atypical inflammatory myofibroblastic tumours: novel RRBP1-ALK fusions in epithelioid inflammatory myofibroblastic sarcoma.
 

Author(s): Jen-Chieh Lee, Chien-Feng Li, Hsuan-Ying Huang, Mei-Jun Zhu, Adrián Mariño-Enríquez, Chung-Ta Lee, Wen-Bin Ou, Jason L Hornick, Jonathan A Fletcher

Journal: J. Pathol.. 2017 Feb;241(3):316-323.

 

ALK oncogenic activation mechanisms were characterized in four conventional spindle-cell inflammatory myofibroblastic tumours (IMT) and five atypical IMT, each of which had ALK genomic perturbations. Constitutively activated ALK oncoproteins were purified by ALK immunoprecipitation ...

Last Updated: 31 Dec 1969

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Proximal-type epithelioid sarcoma: a new case report and literature review.
 

Author(s): Abdelmoughit Echchaoui, Yassine Sadrati, Youssef Elbir, Abderrahim Elktaibi, Malika Benyachou, Samir El Mazouz, Nour-Eddine Gharib, Abdellah Abbassi

Journal:

 

Proximal-type epithelioid sarcoma is a rare soft tissue neoplasm which arises from the more proximal part of body and occurs more often in young people; the definite diagnosis depends mainly on the pathological examination; early detection and complete excision remain the foundation ...

Last Updated: 31 Dec 1969

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Clinical Trial Information This information is provided by ClinicalTrials.gov

Doxorubicin With Upfront Dexrazoxane Plus Olaratumab for the Treatment of Advanced or Metastatic Soft Tissue Sarcoma
 

Status: Recruiting

Condition Summary: Sarcoma, Soft Tissue; Soft Tissue Sarcoma; Undifferentiated Pleomorphic Sarcoma; Leiomyosarcoma; Liposarcoma; Synovial Sarcoma; Myxofibrosarcoma; Angiosarcoma; Fibrosarcoma; Malignant Peripheral Nerve Sheath Tumor; Epithelioid Sarcoma

 

Last Updated: 11 Oct 2018

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A Phase II, Multicenter Study of the EZH2 Inhibitor Tazemetostat in Adult Subjects With INI1-Negative Tumors or Relapsed/Refractory Synovial Sarcoma
 

Status: Recruiting

Condition Summary: Malignant Rhabdoid Tumors (MRT); Rhabdoid Tumors of the Kidney (RTK); Atypical Teratoid Rhabdoid Tumors (ATRT); Selected Tumors With Rhabdoid Features; Synovial Sarcoma; INI1-negative Tumors; Malignant Rhabdoid Tumor of Ovary; Renal Medullary Carcinoma; Epithelioid Sarcoma; Poorly Differentiated Chordoma (or Other Chordoma With Sponsor Approval); Any Solid Tumor With an EZH2 GOF Mutation

 

Last Updated: 18 May 2018

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Ribociclib and Doxorubicin in Treating Patients With Metastatic or Advanced Soft Tissue Sarcomas That Cannot Be Removed by Surgery
 

Status: Recruiting

Condition Summary: Metastatic Angiosarcoma; Metastatic Epithelioid Sarcoma; Metastatic Fibrosarcoma; Metastatic Leiomyosarcoma; Metastatic Liposarcoma; Metastatic Malignant Peripheral Nerve Sheath Tumor; Metastatic Synovial Sarcoma; Metastatic Undifferentiated Pleomorphic Sarcoma; Myxofibrosarcoma; Pleomorphic Rhabdomyosarcoma; Stage III Soft Tissue Sarcoma; Stage IV Soft Tissue Sarcoma; Undifferentiated (Embryonal) Sarcoma

 

Last Updated: 9 May 2018

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