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

• Salivary Gland Angiosarcoma

What is Angiosarcoma of Salivary Gland? (Definition/Background Information)

• Angiosarcoma of soft tissue is an infrequent, aggressive, but often rapidly developing tumor, without signs and symptoms in the early stages. The tumor arises from the vascular linings, which involve the blood vessels
• They are regarded as a type of high-grade malignant cancer that spreads and infiltrates the surrounding tissues and organs. “High-grade” means that the tumor is very aggressive
• Angiosarcoma of Salivary Gland is a very uncommon malignancy that mostly involves the major salivary gland (specifically the parotid and the submandibular gland). The tumor is usually diagnosed in middle-aged and older adult males
• The cause of formation of this salivary gland tumor is unknown. But the tumor is thought to occur as a reactive process influenced by factors that include exposure to carcinogens and radiation
• The presenting signs and symptoms of Angiosarcoma of Salivary Gland may include a slowly-growing facial lump and pain while eating/chewing. Neurological symptoms, such as facial muscle weakness, may be noted
• There are no well-formed treatment protocols for Angiosarcoma of Salivary Gland. However, a combination treatment of surgery and radiation therapy are usually employed
• With prompt diagnosis and adequate treatment, the prognosis of Angiosarcoma of Salivary Gland is good, even though it is a high-grade sarcoma. However, the prognosis also depends upon various factors including tumor stage and size, overall health status of the individual, and other factors

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

• Typically, only about 1.5% of all benign and malignant tumors of the salivary glands are sarcomas
• Angiosarcoma of Salivary Gland may affect a wide range of age groups, but it normally peaks after the age of 50 years. Most cases are observed between age 60-70 years
• There is no gender predominance noted and both males and females are affected
• No ethnic or racial preference has been observed

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

The risk of Angiosarcoma of Salivary Gland is linked to the following factors:

• A previously performed radiotherapy at the head and neck region for other cancer conditions
• Chronic obstruction of the lymph vessels, called lymphedema, due to mastectomy (surgical removal of the breast or the lymph nodes under the armpit; a treatment for breast cancer)
• Exposure to chemical and environmental contaminants, such as arsenic, thorium dioxide, vinyl chloride, dioxin, and many other agents
• Presence of foreign materials in the body, such as graft material (plastic, steel), bone wax, polyester fiber (Dacron, from sutures), etc., which are sometimes known to act as carcinogenic agents
• Angiosarcoma of soft tissue has also been associated to occur in patients diagnosed with genetic disorder including neurofibromatosis (type 1), Klippel-Trenaunay syndrome, and Maffucci syndrome
• Individuals affected by immune deficiency disorders, such as AIDS, have a higher risk of developing the tumor
• Some angiosarcomas are known to be associated with congenital hemangiomas (arising in the parotid gland)

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 Angiosarcoma of Salivary Gland? (Etiology)

The exact cause and mechanism of formation of Angiosarcoma of Salivary Gland, is unknown. They are thought to occur as a reactive process, owing to some preexisting factor(s).

• These preexisting factors may include:
  • Radiation exposure
  • Chronic lymphedema
  • Exposure to chemicals and environmental pollutants
  • Presence of foreign materials in the body for long periods (such as an implant or graft)
• 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 Angiosarcoma of Salivary Gland?

Angiosarcoma of Salivary Gland signs and symptoms include:

• Presence of a firm and visible swelling, typically on the side of the face (near the ears and jaw), that is slowly increasing in size
• Most tumors are around 2-3 cm in diameter, while larger tumors may exceed 6 cm
• 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
• The soft tissue malignant tumors initially grow at a moderate rate, and then suddenly start progressing rapidly, during the later stages. In most cases, the initial tumor growth periods are asymptomatic
• Involvement of the major salivary glands (parotid followed by the submandibular) are seen in most of the cases
• Extremely rarely, other major and minor salivary glands may be involved
• In some individuals bleeding disorders, anemia (decreased red blood cells), internal bleeding, liver damage, etc. may be observed - these developments may be sudden or spontaneous
• Enlarged lymph nodes
• 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 Angiosarcoma of Salivary Gland Diagnosed?

A diagnosis of Angiosarcoma 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

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 Angiosarcoma of Salivary Gland?

Complications of Angiosarcoma 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)
• Tumor recurrence following surgical treatment
• Tumor metastasis to local and distant sites
• 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 Angiosarcoma of Salivary Gland Treated?

There are no well-established treatment protocols available to treat Angiosarcoma of Salivary Gland, since it is a very rare tumor. Nevertheless, a combination of surgery and radiation therapy are generally advocated. The treatment may also depend upon the stage, overall health, age, and subtype of the tumor.

The treatment measures for Salivary Gland Angiosarcoma include the following:

• 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 origin of the tumor
• Following surgery, radiation therapy is usually provided for tumors of the head and neck region
• 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 Angiosarcoma of Salivary Gland be Prevented?

Current medical research has not established a method of preventing the formation of Angiosarcoma of Salivary Gland.

• Adopting a lifestyle that avoids prolonged exposure to pollutants and carcinogens, either at home or at work, may help in preventing cancer development
• 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 metastasizing potential and chances of recurrence, often several years of active follow-up and vigilance is recommended

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

• The prognosis of Angiosarcoma of Salivary Gland is generally good with prompt diagnosis and treatment, since early recognition of the condition is possible in many cases. The best treatment practice for angiosarcoma involves a combination of radiotherapy and appropriate surgical intervention
• Factors that favor better outcomes include small tumor sizes and tumor involvement of the parotid glands (followed by the submandibulars). Tumors affecting minor salivary glands and sublingual (major salivary) glands have generally poorer outcomes
• Unfavorable prognostic indicators include advanced disease stage, older age of the patient, high cell division rate, and large tumor sizes
• 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 Angiosarcoma 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/