Epithelioid sarcoma is a rare type of sarcoma that usually occurs in the fingers, hands, or forearms. Symptoms include a small, hard lump under the skin that can mimic other conditions. Treatment involves radical resection and radiation therapy, with a risk of recurrence and metastases. Prognosis depends on tumor size, age, and sex of the patient.
The term sarcoma generally refers to cancers of the body’s connective tissue, such as fat, muscle, bone, cartilage, or the deep layer of skin. An epithelioid sarcoma is a rare type of sarcoma that occurs most often in the fingers, hands, or forearms, but can also occur in the lower extremities or anywhere in the soft tissue of the body. These tumors are frequently found in young adults, with a defined age range of approximately 20-40 years, and are more common in males.
An epithelioid sarcoma is usually first noticed as a small, hard lump under the skin that isn’t painful. Tumors that are close to the skin’s surface may eventually open up and look like a sore or wound. Deeper tumors might start putting pressure on the nerves, causing pain or numbness and weakness. In general, there tends to be a lag between the first appearance of symptoms and the diagnosis of epithelioid sarcoma because symptoms can be quite variable and can mimic many other conditions. These other conditions are often assumed before the rare epithelioid tumor is suspected.
The prognosis depends mainly on the size of the tumor and on the age and sex of the patient. Larger tumors, especially those associated with initiation of blood vessel development or vasculature, tend to have a worse outcome. An advanced age at the time of diagnosis can also suggest a poor outcome. Furthermore, males are not only more likely to develop epithelioid sarcoma, but also tend to have a worse prognosis than females with the same diagnosis.
Epithelioid sarcomas tend to grow slowly. The main danger of these tumors is their tendency to recur and spread via lymph node metastases. The lungs are a common site of metastases, followed by bone and the brain.
Because of the tendency of these tumors to recur, treatment steps often involve radical resection, which refers to the surgical removal of a considerable amount of normal tissue along with the removal of the actual tumor. Amputation may be recommended for very large tumors, especially if they occur in the fingers or toes, although most doctors would prefer to avoid this option. Radical resection or amputation is often followed by radiation therapy in hopes of eliminating any remaining cancer cells that were not removed with the tumor. These patients need to be followed closely for many years to make sure the cancer has not come back or spread.
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