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Epithelioid Sarcoma (ES)

Last updated May 26, 2018

Approved by: Maulik P. Purohit MD MPH


Very high magnification microscopic pathology image showing epithelioid sarcoma, proximal subtype. H&E stain.

What are the other Names for this Condition? (Also known as/Synonyms)

  • Adult Epithelioid Sarcoma
  • Epithelioid Cell Sarcoma
  • Epithelioid Cell Synovial Sarcoma

What is Epithelioid Sarcoma? (Definition/Background Information)

  • An Epithelioid Sarcoma (ES) is a type of soft tissue tumor involving the epithelioid cells (which line the blood vessels) and connective tissues. It has a high malignancy potential (capacity to metastasize)
  • Though its occurrence is rare, ES frequently affects soft tissues just below the skin (termed superficial), or deep inside the body organs, occurring as a solitary tumor. Tumors at the superficial locations are known to ulcerate the overlying skin
  • ES has been observed in the upper and lower limbs, and on the trunk. Teenagers and young adults are most commonly affected. Usually, an individual does not experience pain or tenderness at the tumor sites

Who gets Epithelioid Sarcoma? (Age and Sex Distribution)

  • Epithelioid Sarcoma may be present in individuals of all age group, but the peak age range is between 10-39 years (from teenage to young-adult period). Infants, young children, and the elderly, are rarely affected
  • There is a slight male predilection; the male to female gender ratio is 1.8:1
  • No particular racial/ethnic preference is seen

What are the Risk Factors for Epithelioid Sarcoma? (Predisposing Factors)

  • No risks are evident, or have been identified for Epithelioid Sarcoma occurrence
  • However, some have reported external injury or trauma as a possible predisposing factor
  • It is researched that the tumor formation may be affected by certain genetic factors too

It is important to note that having a risk factor does not mean that one will get the condition. A risk factor increases ones chances of getting a condition compared to an individual without the risk factors. Some risk factors are more important than others.

Also, not having a risk factor does not mean that an individual will not get the condition. It is always important to discuss the effect of risk factors with your healthcare provider.

What are the Causes of Epithelioid Sarcoma? (Etiology)

  • The exact cause and mechanism of Epithelioid Sarcoma formation, is unknown. They are thought to occur spontaneously
  • It is suggested that genetic factors or trauma (of any sort), may have a role in their formation

What are the Signs and Symptoms of Epithelioid Sarcoma?

The presentations are based on the location of the tumor. Epithelioid Sarcoma signs and symptoms include:

  • Most tumors are asymptomatic. However, in others, a wide-range of behavior is observed
  • They may grow at a slow rate and appear as painless inflammatory irregular (or nodular) lesions below the skin surface. They may also be present as deep-seated tumors
  • Lesions just below the skin appear like a badly-healed wound
  • Most common location of ES is the finger, hand, forearm, foot, knee, leg, thigh, and trunk. Other locations include the spine, penis, and female genitalia
  • On the hands and legs, muscle contracture and nerve compression may occur, causing muscular- and nerve-related signs and symptoms

How is Epithelioid Sarcoma Diagnosed?

A diagnosis of Epithelioid Sarcoma is made using the following tools:

  • Physical exam with evaluation of the individual’s medical history
  • MRI scan, PET scan, of the affected region
  • Histopathological studies conducted on a biopsy specimen: A pathologist examines the tissue under a microscope before arriving at a definitive diagnosis. A microscopic examination by a pathologist is considered to be gold standard in diagnosis of the tumor
  • Genetic analysis through molecular testing, helps differentiate ES from other different tumor types

Many clinical conditions may have similar signs and symptoms. Your healthcare provider may perform additional tests to rule out other clinical conditions to arrive at a definitive diagnosis.

What are the possible Complications of Epithelioid Sarcoma?

Complications are dependent on the site and severity of the tumor. These include:

  • Recurrence of Epithelioid Sarcoma after surgery (there is an 80% chance of recurrence)
  • Metastasizing of tumor into lungs, lymph nodes, and scalp
  • Bones may undergo demineralization; sometimes limb amputation may be required
  • Damage to vital nerves, blood vessels, and surrounding structures during surgery
  • Side effects from chemotherapy (such as toxicity), radiation therapy

How is Epithelioid Sarcoma Treated?

Treatment measures for Epithelioid Sarcoma include the following:

  • Wide surgical excision of ES with removal of the entire lesion is adopted, if necessary. If the lesion is not fully removed, then it might recur
  • Embolization is used to provide temporary relief from the symptoms, and reduce blood loss during a surgical procedure
  • High-dose radiation therapy is used after the surgery to destroy the remaining present tumor cells. Chemotherapy has not shown any particular benefit in treating ES
  • Aggressive lesions may require multi-disciplinary management
  • Bone tumors may require the use of bone grafts. Amputations may be recommended for lesions on the limbs
  • Post-operative care is important; minimum activity level is to be ensured until the surgical wound heals
  • Follow-up care with regular screening and check-ups are important

How can Epithelioid Sarcoma be Prevented?

  • Current medical research has not established a way of preventing Epithelioid Sarcoma
  • Regular medical screening at periodic intervals with blood tests, scans, and physical examinations are mandatory for those who have already endured ES; due to its metastasizing potential and chances of recurrence
  • Often several years of active vigilance is necessary

What is the Prognosis of Epithelioid Sarcoma? (Outcomes/Resolutions)

  • Epithelioid Sarcoma is a rare, high-mortality tumor, with metastasizing/malignancy potential (metastatic rate at 45%)
  • The long-term favorable prognosis depends on a combination of factors such as; young age of patient, female gender, and location of tumor at the extremities (distal)
  • A negative outcome is guided by factors, such as age at diagnosis, the size of the tumor (larger than 2 cm), location of the tumor (axial or proximal), and if biopsy of the specimen reveals necrosis with high vascularity

Additional and Relevant Useful Information for Epithelioid Sarcoma:

Epithelioid Sarcoma is often confused with many benign and malignant sarcomas. Hence, an accurate diagnosis is essential for prompt and definite treatment action.

What are some Useful Resources for Additional Information?

References and Information Sources used for the Article:

Helpful Peer-Reviewed Medical Articles:

Reviewed and Approved by a member of the DoveMed Editorial Board
First uploaded: June 26, 2013
Last updated: May 26, 2018