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What Is an Epithelioid Sarcoma?

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  • Written By: S. Gadd
  • Edited By: Heather Bailey
  • Last Modified Date: 21 July 2014
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The term sarcoma generally refers to tumors in the connective tissue of the body, such as fat, muscle, bone, cartilage, or the deep skin layer. An epithelioid sarcoma is a rare type of sarcoma that most often occurs in the fingers, hands, or forearms, but can also occur in the lower extremities, or anywhere throughout the soft tissues of the body. These tumors are frequently found in young adults, with a defined age range of about 20-40 years, and are more common in males.

An epithelioid sarcoma is usually first noticed as a small hard mass underneath the skin that is not painful. Tumors that are close to the skin’s surface may eventually open and have the appearance of a sore or a wound. Deeper tumors might begin to exert pressure on nerves, causing either pain or numbness and weakness. In general, there tends to be a gap of time between the first appearance of symptoms and diagnosis of epithelioid sarcoma because the symptoms can be quite variable and can imitate many other conditions. These other conditions are often assumed before the rare epithelioid tumor is suspected.

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The prognosis depends mostly on the size of the tumor and the age and gender of the patient. Larger tumors, especially those that are associated with initiation of blood vessel development, or vascularization, tend to have a worse outcome. An older age at diagnosis may also suggest a poor outcome. In addition, males not only have a higher likelihood of developing an epithelioid sarcoma, but they also tend to have a poorer 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 to spread via lymph node metastasis. The lungs are a common site of metastases, followed by bone and brain.

Due to the tendency of these tumors to recur, treatment steps often involve radical resection, which refers to the surgical removal of a sizeable amount of normal tissue along with 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 rather avoid this option. Radical resection or amputation is often followed by radiation therapy in hopes of getting rid of any remaining cancer cells that were not excised with the tumor. These patients should be closely followed for many years to ensure the tumor has not recurred or spread.

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