Carcinosarcoma of Salivary Gland

Carcinosarcoma of Salivary Gland

Article
Dental Health
Diseases & Conditions
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Contributed byMaulik P. Purohit MD MPHNov 18, 2019

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

  • Salivary Gland Carcinosarcoma
  • True Malignant Mixed Tumor of Salivary Gland

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

  • Carcinosarcoma of Salivary Gland is an extremely uncommon, but highly-aggressive malignancy of the major salivary glands (mostly) consisting of both carcinomatous and sarcomatous features. It may be observed in children and adults
  • The cause of formation of this salivary gland malignancy is generally unknown, but may be due to genetic factors. No definitive risk factors are observed, but many individuals with Salivary Gland Carcinosarcoma are known to have a history of pleomorphic adenoma (a benign tumor)
  • The presenting signs and symptoms of Carcinosarcoma of Salivary Gland may include a tumor growth forming a lump, associated with pain in some cases, facial paralysis, and eating/chewing difficulties
  • The treatment for Carcinosarcoma of Salivary Gland involves a combination of surgery, chemotherapy, and radiation therapy. However, even with prompt diagnosis and adequate treatment, the prognosis is generally poor, since these are high-grade neoplasms

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

  • Carcinosarcoma of Salivary Gland has been recorded in a wide age category of individuals (age range 14-87 years). The average age at diagnosis is noted to be 58 years
  • Both male and female genders are affected
  • No ethnic or racial preference has been observed
  • The incidence rate of the tumor is very low; only about 60 cases have been reported in the medical literature worldwide

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

Carcinosarcoma of Salivary Gland may be associated with the following factors:

  • Individuals with a history of pleomorphic adenoma tumor recurrence (may be multiple recurrences)
  • Presence of pleomorphic adenoma (carcinosarcoma ex pleomorphic adenoma) in numerous cases

The following risk factors are generally 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 Carcinosarcoma of Salivary Gland? (Etiology)

The exact cause of Carcinosarcoma of Salivary Gland formation is unknown. It is suggested that genetic factors may have a role in their formation.

  • The following genetic abnormalities have been observed:
    • TP53 gene mutations in some cases, affecting the chromosomal region 17p13
    • A solitary case of loss of heterozygosity (LOH) at the following locations - 17p13.1, 17q21.3, and 18q21.3 have been recorded
  • 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 Carcinosarcoma of Salivary Gland?

Carcinosarcoma of Salivary Gland signs and symptoms may include the following:

  • The tumor mass usually presents no pain; pain is present only in some cases
  • Tumors are highly-infiltrative and known to destroy the adjoining tissue structures
  • Malignant tumors may cause neurological signs and symptoms, such as facial muscle weakness, paralysis, and pain, due to facial nerve involvement
  • Pain while eating/chewing
  • Most tumors are firm and poorly-defined (some are well-defined though)
  • Presence of visible swelling, typically on the side of the face, or sometimes inside the mouth
  • 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
  • Persistent facial pain at the site of swelling of the tumor; this requires an immediate checkup by a healthcare provider

Location of the malignant tumor:

  • Major salivary glands: Involvement of the parotid gland is observed in about 65% of the cases; nearly 20% of the cases, involve the submandibular glands
  • Minor salivary glands: Nearly 15% of the cases have been reported in the mouth, affecting the hard/soft palate. Very few cases at other locations include the tongue and base of the tongue (supraglottic space)

How is Carcinosarcoma of Salivary Gland Diagnosed?

A diagnosis of Carcinosarcoma 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 tumor 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

When examined by a pathologist under the microscope, the salivary gland tumor consists of elements that are both carcinomatous and sarcomatous.

  • The carcinomatous elements may be ductal carcinoma (moderate or poorly-defined) or undifferentiated carcinoma
  • The sarcomatous elements may be osteosarcoma, chondrosarcoma, or undifferentiated sarcoma

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 Carcinosarcoma of Salivary Gland?

Complications of Carcinosarcoma 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 the tumor locally, after surgery, is noted to be generally high
  • Tumor metastasis to local and distant sites: Metastasis is often seen to involve the central nervous system (CNS), lungs, and bones
  • 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 Carcinosarcoma of Salivary Gland Treated?

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

The treatment measures for Salivary Gland Carcinosarcoma may involve:

  • Wide surgical excision with removal of the entire lesion is the standard treatment mode. This is usually followed by radiation therapy
  • High-dose radiation therapy may be used after surgery, to destroy the remaining tumor cells
  • 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 Carcinosarcoma of Salivary Gland be Prevented?

  • Current medical research has not established a method of preventing the formation of Carcinosarcoma 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 Carcinosarcoma of Salivary Gland? (Outcomes/Resolutions)

  • The prognosis of Carcinosarcoma of Salivary Gland is generally poor, since it is an aggressive tumor. The death rate is about 60% and most deaths occur in about 2.5 years following its diagnosis. Most deaths are known to occur due to local recurrences and/or tumor metastasis
  • 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 resectability 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

Additional and Relevant Useful Information for Carcinosarcoma 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/

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On the Article

Maulik P. Purohit MD MPH picture
Approved by

Maulik P. Purohit MD MPH

Assistant Medical Director, Medical Editorial Board, DoveMed Team

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