Epithelioid sarcoma (patient information)
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Ammu Susheela, M.D. [2]
Overview
Overview
Epithelioid sarcoma is a rare mesenchymal soft tissue tumor. Mutations in the SMARCB1 gene cause epithelioid sarcoma. Tissue biopsy may be helpful in the diagnosis of epithelioid sarcoma. The predominant therapy for epithelioid sarcoma is surgical resection. Adjunctive chemotherapy, radiation, and chemoradiation may be required.
What are the symptoms of Epithelioid sarcoma?
What are the symptoms of Epithelioid sarcoma?
Irregular (or nodular) lesions below the skin surface are the major symptom of epithelioid sarcoma.
What causes Epithelioid sarcoma?
What causes Epithelioid sarcoma?
Mutations in the SMARCB1 gene cause epithelioid sarcoma.
Who is at highest risk?
Who is at highest risk?
The most potent risk factor in the development of epithelioid sarcoma is the presence of family history of epithelioid sarcoma.
Diagnosis
Diagnosis
Tissue biopsy may be helpful in the diagnosis of epithelioid sarcoma.
When to seek urgent medical care?
When to seek urgent medical care?
A person should seek urgent medical care when there are any complications that arise from epithelioid sarcoma.
Treatment options
Treatment options
The predominant therapy for epithelioid sarcoma is surgical resection. Adjunctive chemotherapy, radiation, and chemoradiation may be required.
Where to find medical care for Epithelioid sarcoma?
Where to find medical care for Epithelioid sarcoma?
What to expect (Outlook/Prognosis)?
What to expect (Outlook/Prognosis)?
The prognosis of epithelioid sarcoma is good for females.
Possible complications
Possible complications
- Metastasis
- Contractures
- Nerve compression
- Muscle weakness
- Numbness
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