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

• Carcinoma Ex Lymphoepithelial Lesion of Salivary Gland
• Lymphoepithelioma-Like Carcinoma of Salivary Gland
• Undifferentiated Carcinoma with Lymphoid Stroma of Salivary Gland

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

• Lymphoepithelial Carcinoma (LEC) of Salivary Gland is a rare type of an undifferentiated carcinoma. It is mostly present in the major salivary (parotid) glands and affects middle-aged and older men and women
• A strong predilection for certain ethnic groups, notably the Inuit tribes of the Arctic region, is reported. In these endemic groups, the tumor is almost always associated with Epstein-Barr virus infection
• The cause of formation of this salivary gland malignancy is generally unknown, but it may be due to a complex mix of numerous factors, such as geographical location, viral infections, and ethnicity
• The signs and symptoms of Lymphoepithelial Carcinoma of Salivary Gland may include a tumor growth forming a lump, associated with pain in some cases, facial paralysis, and eating/chewing difficulties. The tumors exhibit local destruction in many cases
• The treatment for Lymphoepithelial Carcinoma of Salivary Gland involves a combination of surgery, chemotherapy, and radiation therapy, in most cases. The prognosis of the individual depends upon a set of several factors including the tumor stage and overall health of the individual

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

• Lymphoepithelial Carcinoma of Salivary Gland has been recorded in both children and adults
• The age range of presentation is between 1-90 years (peak period is between 40-50 years)
• Both male and female genders are affected, but a slight male preference is noted
• LEC of Salivary Gland shows a strong predominance for certain racial and ethnic groups such as the following:
  • The indigenous tribe of Inuits (Eskimo population) inhabiting the Arctic regions including countries such as Canada, Greenland, and US (Alaska)
  • Certain Japanese population
  • People of south-eastern China
• It is estimated that only about 1% of salivary gland malignancies are lymphoepithelial tumors

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

The following risk factors may be specifically noted for Lymphoepithelial Carcinoma of Salivary Gland:

• Certain racial and ethnic groups in the geographical (endemic) regions of the Arctic (the Inuits), China, and Japan, are more prone to this carcinoma type
• Infection with Epstein-Barr virus (EBV): In the indigenous populations that are affected by LEC, a 100% association with EBV is observed

Note: In individuals, who do not belong to these (above) regions, the presence of EBV is not observed in almost all of the cases.

• Presence of lymphoepithelial sialadenitis (chronic infection of the salivary glands)

It is important to note that an association of the tumor with Sjögren's syndrome is not usually observed; meaning, that the syndrome is not a risk factor for LEC of Salivary Gland.

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 Lymphoepithelial Carcinoma of Salivary Gland? (Etiology)

The exact cause of Lymphoepithelial Carcinoma of Salivary Gland formation is unknown.

• Scientists believe that the carcinoma may develop from an interplay of a set of complex factors that include the environment, one’s geographical location, ethnicity, and influences from viral infection
• Even though, an absence of the Epstein-Barr virus is observed when individuals outside the endemic locations develop LEC, it is thought that the role of EBV is very important for tumor development

Most LEC of Salivary Gland tumors are known to arise spontaneously (de novo). But, it is also suggested that genetic factors may have a role in their formation.

• Some study case reports reveal that many members within the same family have been affected
• In a particular study, it was noted that the inherited form of trichoepithelioma may increase the risk for development of Salivary Gland LEC, which indicates that there may be the involvement of certain genetic factors

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 Lymphoepithelial Carcinoma of Salivary Gland?

Lymphoepithelial Carcinoma of Salivary Gland signs and symptoms may include the following:

• Most tumors are initially present as a slow-growing tumor (over a long duration). It presents no pain, in some cases
• A sudden burst of tumor growth may be observed over a few weeks or months
• The tumors may show deep infiltration into the surrounding tissue structures
• The tumors are generally single, fleshy masses with infiltrative borders; they may be well-defined
• The size of the tumor can vary from 1 to 10 cm (average size 2-3 cm); some grow to large sizes
• Presence of visible swelling that is slowly increasing in size, typically on the side of the face, if the parotid gland is involved
• Tumors in the oral mucosa may ulcerate and bleed
• Pain while eating/chewing
• Neurological signs and symptoms, such as facial muscle weakness and pain, due to facial nerve involvement are noted in 1 in 5 cases
• The tumor is known to spread to the neck lymph nodes (vascular invasion is often observed) in about 10-40% of the cases. Extensive involvement of the cervical (neck) lymph nodes may be noted
• Advanced stage tumors may involve the tissues and overlying skin
• The malignancies are known to erode the bone and present pain
• 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: About 80% of the cases involve the parotid glands; the submandibular glands are also known to be affected to a much lesser extent
• Minor salivary glands: Very few cases are observed in the oral mucosa (inside the mouth), including within the pharyngeal region

How is Lymphoepithelial Carcinoma of Salivary Gland Diagnosed?

A diagnosis of Lymphoepithelial Carcinoma of Salivary Gland is made using the following tools:

• Complete evaluation of family (medical) history, along with a thorough physical examination
• Testing for Epstein-Barr virus: Blood tests for detecting antibodies against a viral etiology/cause
• 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

A differential diagnosis is essential to eliminate the following tumor types (or conditions) prior to arriving at a definite diagnosis:

• Metastatic nasopharyngeal carcinoma: Many cases of cancer spread from the nasopharynx to the salivary glands have been observed. And hence, prior to identifying LEC of Salivary Gland as a primary tumor (i.e., origin site is within the salivary gland itself), metastasis of nasopharyngeal carcinoma must be ruled out
• Large cell and metastatic undifferentiated carcinoma
• Lymphadenoma
• Lymphoepithelial sialadenitis
• Malignant lymphoma

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 Lymphoepithelial Carcinoma of Salivary Gland?

Complications of Lymphoepithelial Carcinoma 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 after surgery is observed
• Bones may be affected and undergo demineralization
• Tumor metastasis to local and distant sites is known to take place in about 1 in 5 cases. The organs involved include the lung, bone, liver, and brain
• 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 Lymphoepithelial Carcinoma of Salivary Gland Treated?

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

The treatment measures may involve:

• Wide surgical excision with removal of the entire lesion including neck lymph node dissection is the generally undertaken
• 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 Lymphoepithelial Carcinoma of Salivary Gland be Prevented?

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

• The prognosis of Lymphoepithelial Carcinoma of Salivary Gland may be assessed on a case-by-case basis. The 5-year survival rate is between 75-86%
• With early diagnosis and effective treatment, which includes surgical excision of the tumor and dissection of the affected cervical lymph node, the prognosis is generally good. However, the strongest indicator of an adverse outcome is the advanced stage of the tumor
• 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 Lymphoepithelial Carcinoma 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/