Epithelioid Sarcoma

An uncommon kind of cancer called epithelioid sarcoma begins as an expansion of cells in soft tissue. On the body, it may happen everywhere. The finger, hand, forearm, knee, or lower leg are common places where it begins under the skin.

A nodule, also known as a tiny, solid growth or bump under the skin, may develop as a result of epithelioid sarcoma. Frequently, it doesn't hurt. One growth or more growths might exist. On occasion, the growths lead to skin sores that do not heal.

Teenagers and young adults are frequently affected by epithelioid sarcoma. Yet it can also have an impact on seniors. The growth of epithelioid sarcoma is usually sluggish. After therapy, it frequently returns. Soft tissue sarcoma, also known as epithelioid sarcoma, is a kind of cancer. These tumors develop in the connective tissues of the body.

Soft tissue sarcomas can take many different forms. Soft tissue sarcomas are uncommon, and epithelioid sarcoma is one of them. It is better to receive treatment in a cancer center with experience treating sarcoma patients.

Epithelioid Sarcoma: Causes

Malignant tumors called epithelioid sarcomas have mixed mesenchymal and epithelial cell development. Close to 90% of epithelioid sarcomas have decreased expression of the integrase interactor-1 (INI-1). All healthy nucleated cells express the SWI/SNF chromatin remodeling complex, of which INI-1 is a component. This chromatin remodeling complex modifies nucleosomes to enable DNA transcription, which is crucial for biological activity. INI-1, often referred to as SMARCB1, is located in the 22q11 band on chromosome 22.

In INI-1 deficient epithelioid sarcomas, a variety of gene deletions and rearrangements, including translocations affecting the 22q11 region, are thought to be the cause of carcinogenesis. It's interesting to note that up to 27% of cases of tumors have been linked to past trauma at the tumor’s location. Nonetheless, it stands to reason that prior trauma to the distal extremity is an all too frequent occurrence for additional supposition as to an initiating event.

Epithelioid Sarcoma: Symptoms

The most typical symptom of epithelioid sarcoma is a slow-growing, painless, multinodular swelling of the distal upper extremity that occasionally ulcerates. The dermis, subcutis, or deep fascia may serve as the lesion's primary focus. Typically, it follows tendons and aponeuroses as it invades along the fascial planes. While it is defined by its histologic characteristics rather than by location, there is a proximal variety of epithelioid sarcoma that tends to develop more frequently in the perineal, pubic, genital, and truncal areas.

While masses frequently have a maximum diameter of fewer than 5 centimeters, they can be rather enormous, up to 20 cm, particularly in the proximal variety. Local invasion by epithelioid sarcoma is aggressive, and it often spreads to nearby lymph nodes. It is generally known that distant metastatic spread can affect the lungs and scalp in particular.

Due to the aggressiveness of local recurrences and the potential for metastasis, the clinical behavior of the tumor has perhaps best been characterized as an "indolent, unrelenting clinical course."

Epithelioid Sarcoma: Risk Factors

Several factors play an important role in the development of epithelioid sarcoma which includes −

  • Prior radiation treatment

  • Genetics

  • Autoimmune disorders

  • Lymphedema

  • Chemicals

Epithelioid Sarcoma: Diagnosis

The diagnosis of epithelioid sarcoma is mainly done based on history and some of the tests may be required for confirmation and to rule out underlying causes −

Diagnosis of epithelioid sarcoma might be challenging. It appears that there are far more widespread issues. Such easier-to-treat issues are frequently prioritized first by medical professionals. A skin sore that is not healing, for instance, can be mistaken for an infection.

The following tests and techniques are used to diagnose epithelioid sarcoma −

  • Image-based exams. Imaging procedures capture images of the body. They can display an epithelioid sarcoma's location and size. X-rays, MRIs, CT scans, and PET scans, sometimes known as positron emission tomography tests, may be performed.

  • Obtaining tissue for analysis. An operation called a biopsy is used to take a sample of tissue for laboratory analysis. A needle inserted into the cancer and through the skin may be used to remove the tissue. Surgery may occasionally be required to get the tissue sample. To determine if the sample contains cancer, it is analyzed in a lab. Further information about the cancer cells is revealed by other specialized testing. This data is used by your medical team to develop a treatment strategy.

Epithelioid Sarcoma: Treatment

The treatment is based on the severity of the symptoms. Your doctor may advise conservative or surgical treatment.

Conservative Treatment

Conservative treatment includes −

  • Radiation treatment. Strong energy beams are used in radiation treatment to eliminate cancer cells. Before surgery, radiation treatment may be performed to reduce the tumor. This may increase the likelihood that during surgery, all of the cancer will be removed. After surgery, radiation treatment may be utilized to eradicate any residual cancer cells.

  • Targeted treatment. In targeted treatment, drugs are used to target certain molecules in cancer cells that promote their growth. Targeted therapy can kill cancer cells by inhibiting these substances. If surgery is not an option for you or if other treatments are unsuccessful, targeted therapy may be an alternative.

  • Chemotherapy. Chemotherapy kills cancer cells by using powerful medications. Epithelioid sarcoma that has spread to different bodily areas may be treated with chemotherapy. In cases where surgery is not a possibility, it may also be employed.

  • Scientific tests. Studies of novel medicines are called clinical trials.

Surgical Treatment

Surgery entails the removal of the malignancy as well as some surrounding healthy tissue. To ensure that all cancer cells are eliminated, it is helpful to take some healthy tissue. The likelihood that cancer will return is decreased by eliminating all malignant cells.

Epithelioid Sarcoma: Prevention

Avoiding exposure to risk factors whenever feasible is the only approach to stop certain soft tissue sarcomas from developing. Nevertheless, the majority of sarcomas occur in persons with no recognized risk factors, hence there is currently no known approach to avoid the majority of instances. Moreover, radiation treatment patients typically have few options.


Young individuals with epithelioid sarcoma typically appear with a slow-growing tumor of an extremity. The savvy primary care practitioner must have a high index of suspicion for a tumor that warrants biopsy due to the clinical presentation's deceptively innocuous character. After a disease has been diagnosed, the healthcare team must use an interprofessional approach to determine the best staging, course of therapy, and long-term management of the condition.

Surgery, pathology, radiography, and nursing will all be involved. Complete excision can result in curative results, although there is a significant risk of local recurrence that can be reduced with radiation therapy, especially in tumors larger than 3 cm. Palliative care combined with medical oncology and radiation oncology has largely remained undefined in non-resectable patients and metastatic illnesses.

Dr. Durgesh Kumar Sinha
Dr. Durgesh Kumar Sinha


Updated on: 26-Apr-2023


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