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

• ES of Salivary Gland
• Salivary Gland Epithelioid Sarcoma

What is Epithelioid Sarcoma of Salivary Gland? (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)
• Epithelioid Sarcoma of Salivary Gland is a very uncommon high-grade malignancy that mostly involves the major salivary gland (specifically the parotid gland). The tumor is diagnosed in a wide age group of children and adults
• The cause of formation of this salivary gland tumor is generally unknown. No definitive risk factors are observed, but epithelioid sarcomas are known to be associated with trauma and genetic abnormalities
• The presenting signs and symptoms of Epithelioid Sarcoma of Salivary Gland may include a slowly-growing facial lump and pain while eating/chewing. Involvement of the other major salivary glands, such as the submandibular and sublingual, have not been reported
• The treatment for Epithelioid Sarcoma of Salivary Gland involves surgery followed by radiation therapy, in most cases. With prompt diagnosis and adequate treatment, the prognosis of Epithelioid Sarcoma of Salivary Gland is generally better than ES affecting other body sites 

Who gets Epithelioid Sarcoma of Salivary Gland? (Age and Sex Distribution)

• Even though epithelioid sarcomas are mostly observed in teens and young adults, Epithelioid Sarcoma of Salivary Gland has been recorded in very young children (less than 6 years) and older adults (above 70 years)
• Even though both genders are affected, a male predominance is noted
• No ethnic or racial preference has been observed
• About 10% of salivary gland malignancies are sarcomas; an epithelioid sarcoma is extremely uncommon among them

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

No risks are evident, or have been identified for Epithelioid Sarcoma of Salivary Gland.

• However, some have reported external injury or trauma as a possible predisposing factor for epithelioid sarcoma, in general
• It is researched that the tumor formation may be affected by certain genetic factors

The following general risk factors are noted for salivary gland cancers:

• Exposure to ionizing radiation (which may be from natural or artificial sources)
• Occupations involving woodwork, plumbing, and mining (asbestos)
• Working in manufacturing industry related to certain materials, such as rubber products

It is important to note that having a risk factor does not mean that one will get the condition. A risk factor increases one’s 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 of Salivary Gland? (Etiology)

The exact cause of Epithelioid Sarcoma of Salivary Gland 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.

• In general, it is known that cancers form when normal, healthy cells begin transforming into abnormal cells - these cancer cells grow and divide uncontrollably (and lose their ability to die), resulting in the formation of a mass or a tumor
• The transformation of normally healthy cells into cancerous cells may be the result of genetic mutations. Mutations allow the cancer cells to grow and multiply uncontrollably to form new cancer cells
• These tumors can invade nearby tissues and adjoining body organs, and even metastasize and spread to other regions of the body

What are the Signs and Symptoms of Epithelioid Sarcoma of Salivary Gland?

Epithelioid Sarcoma of Salivary Gland signs and symptoms include:

• They may grow at a slow rate and appear as painless inflammatory irregular (or nodular) lesions below the skin surface. The tumor is about 3-4 cm size
• Presence of visible swelling, typically on the side of the face, that is slowly increasing in size
• Initially, the overlying skin may not appear inflamed or present changes in texture or color
• As the tumor progress and becomes larger in size, changes in the overlying skin including ulceration may be noted
• Involvement of the parotid glands are seen in a vast majority of the cases; the tumor may be present as a painless mass in the gland
• Malignant tumors may cause neurological signs and symptoms, such as facial muscle weakness and pain, due to facial nerve involvement
• Persistent facial pain at the site of swelling of the tumor; this requires an immediate checkup by a healthcare provider

How is Epithelioid Sarcoma of Salivary Gland Diagnosed?

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

• Complete evaluation of family (medical) history, along with a thorough physical examination
• Plain X-ray of the head and neck
• Ultrasound scan of the affected salivary gland
• CT or CAT scan with contrast of the head and neck may show a well-defined mass. This radiological procedure creates detailed 3-dimensional images of structures inside the body
• MRI scans of head and neck: Magnetic resonance imaging (MRI) uses a magnetic field to create high-quality pictures of certain parts of the body, such as tissues, muscles, nerves, and bones. These high-quality pictures may reveal the presence of the tumor

Although the above modalities can be used to make an initial diagnosis, a tissue biopsy of the tumor is necessary to make a definitive diagnosis to begin treatment. The tissue for diagnosis can be procured in multiple different ways which include: 

• Fine needle aspiration (FNA) biopsy of the tumor: A FNA biopsy may not be helpful, because one may not be able to visualize the different morphological areas of the tumor. Hence, a FNA biopsy as a diagnostic tool has certain limitations, and an open surgical biopsy is preferred
• Salivary gland core biopsy of the tumor
• Salivary gland open biopsy of the tumor

Tissue biopsy of the tumor:

• A tissue biopsy of the nodule is performed and sent to a laboratory for a pathological examination. A pathologist examines the biopsy under a microscope. After putting together clinical findings, special studies on tissues (if needed) and with microscope findings, the pathologist arrives at a definitive diagnosis. Examination of the biopsy under a microscope by a pathologist is considered to be gold standard in arriving at a conclusive diagnosis
• Biopsy specimens are studied initially using Hematoxylin and Eosin staining. The pathologist then decides on additional studies depending on the clinical situation
• Sometimes, the pathologist may perform special studies, which may include immunohistochemical stains, molecular testing, and very rarely, electron microscopic studies to assist in the diagnosis

Note: An important differential diagnosis is extrarenal rhabdoid tumor, before a definite diagnosis of epithelioid sarcoma is arrived at.

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 of Salivary Gland?

Complications of Epithelioid Sarcoma of Salivary Gland may include:

• Cosmetic concerns (facial disfigurement) and severe emotional stress
• Ulceration and bleeding from the tumor
• Large-sized tumors may compress adjoining tissues and structures resulting in additional complications
• Breathing or swallowing difficulties (if tumor mass obstructs the food-pipe or wind-pipe)
• Recurrence of epithelioid sarcoma after surgery; in general, there is an 80% chance of recurrence of ES
• Bones may be affected and undergo demineralization
• Tumor metastasis to local and distant sites: The lung and lymph nodes are commonly involved
• Surgical complications: Surgery to remove the tumor mass may result in facial nerve palsy and gustatory sweating (Frey syndrome)
• Post-surgical wound infection
• Side effects from chemotherapy (such as toxicity), radiation therapy

How is Epithelioid Sarcoma of Salivary Gland Treated?

A combination of surgery, chemotherapy, and radiation therapy are used to treat Epithelioid Sarcoma of Salivary Gland. The treatment may also depend upon the stage, overall health, age, and subtype of the tumor.

• Wide surgical excision with removal of the entire lesion is the standard treatment mode. A total parotidectomy may be considered if the parotid gland is the primary site of tumor origin
• High-dose radiation therapy is used after the surgery to destroy the remaining present tumor cells. Chemotherapy is not known to be beneficial in treating ES
• When the tumor is at an inaccessible location, or is unsafe for surgical intervention, non-invasive procedures, such as chemotherapy and radiation therapy (using fast neutron-beam), may be considered
• Recurrent salivary gland tumors are also known to respond better to fast neutron-beam radiation therapy than other treatment modes
• Embolization is used to provide temporary relief from the symptoms, and reduce blood loss during a surgical procedure
• Clinical trial therapies (especially for stage IV disease) including therapeutic drugs, radiation, stem cell transplantation, and monoclonal antibodies, either singly or in combination of various therapies
• Post-operative care is important: A minimum activity level is to be ensured until the surgical wound heals
• Follow-up care with regular screening and check-ups are important, to watch for recurrence and any metastatic behavior

How can Epithelioid Sarcoma of Salivary Gland be Prevented?

• Current medical research has not established a method of preventing the formation of Epithelioid Sarcoma of Salivary Gland
• Regular medical screening at periodic intervals with blood tests, radiological scans, and physical examinations, are mandatory for those who have been diagnosed with the tumor
• Due to its high metastasizing potential and chances of recurrence, often several years of active follow-up and vigilance is recommended

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

• The prognosis of Epithelioid Sarcoma of Salivary Gland is generally better than ES at other sites, since early recognition of the condition is possible in many cases. The 5-year overall survival rate is 50-92%
• The long-term favorable prognosis depends on a combination of factors including young age of the patient and female gender
• A negative outcome is guided by factors such as age at diagnosis, the size of tumor (larger than 2 cm), and if biopsy of the specimen reveals necrosis with high vascularity
• The prognosis of salivary gland cancer, in general, depends upon a set of several factors that include:
  • Stage of tumor: With lower-stage tumors, when the tumor is confined to site of origin, the prognosis is usually excellent with appropriate therapy. In higher-stage tumors, such as tumors with metastasis, the prognosis is poor
  • The size of the tumor: Individuals with small-sized tumors fare better than those with large-sized tumors
  • Overall health of the individual: Individuals with overall excellent health have better prognosis compared with those with poor health
  • Age of the individual: Older individuals generally have poorer prognosis than younger individuals
  • Individuals with bulky disease have a poorer prognosis
  • Involvement of the lymph node can adversely affect the prognosis
  • Involvement of vital organs may complicate the condition
  • The surgical respectability of the tumor (meaning, if the tumor can be removed completely)
  • Whether the tumor is occurring for the first time, or is a recurrent tumor. Recurring tumors have worse prognosis compared to tumors that do not recur
  • Response to treatment of salivary gland cancer: Tumors that respond to treatment have better prognosis compared to tumors that do not respond to treatment
  • Progression of the condition makes the outcome worse
• An early diagnosis and prompt treatment of the tumor generally yields better outcomes than a late diagnosis and delayed treatment
• In general, for all salivary gland malignancies the following may be noted:
  • The recurrence rate is between 40-65%
  • Spread of the tumor (metastasis) is seen in 38-65% of the cases
  • And, the death rate lies between 35-65%; most deaths occurring within 3 years of tumor diagnosis

Additional and Relevant Useful Information for Epithelioid Sarcoma of Salivary Gland:

There are 3 major types of salivary glands and these include the following:

• Parotid glands, found on the sides of the face
• Submandibular glands located at the back of mouth, on both sides of the jaw
• Sublingual glands that are seen under the floor of the mouth

The following article link will help you understand other cancers and benign tumors:

http://www.dovemed.com/diseases-conditions/cancer/