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

• CAMSG (Cribriform Adenocarcinoma of Minor Salivary Gland)
• CATMSG (Cribriform Adenocarcinoma of the Tongue and Minor Salivary Gland)
• Cribriform Adenocarcinoma of Minor Salivary Gland (CAMSG)

What is Cribriform Adenocarcinoma of the Tongue and Minor Salivary Gland? (Definition/Background Information)

• Cribriform Adenocarcinoma of the Tongue and Minor Salivary Gland (CATMSG) is an uncommon tumor affecting the tongue or minor salivary glands. The tumor is observed in young and old adults
• Some reports indicate that it may be a variant of, or a different growth pattern type of polymorphous low-grade adenocarcinoma (PLGA). PLGA, as the name indicates, is a low-grade malignancy with some metastatic potential, but showing an infiltrative growth pattern
• 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, though salivary gland cancers are generally known to be influenced by factors such as exposure to radiation sources and occupational exposure to hazardous chemicals/products
• The signs and symptoms of Cribriform Adenocarcinoma of the Tongue and Minor Salivary Gland may include the formation of a tumor lump at the base of the tongue or in the minor salivary glands. It may be associated with pain in some cases, eating/chewing difficulties, and infiltration into the surrounding tissue structures
• The treatment for Cribriform Adenocarcinoma of the Tongue and Minor Salivary Gland involves surgery. Other measures, such as chemotherapy and radiation therapy, may be considered on a case-by-case basis. With prompt diagnosis and adequate treatment, the prognosis of CATMSG can be good

Who gets Cribriform Adenocarcinoma of the Tongue and Minor Salivary Gland? (Age and Sex Distribution)

• Cribriform Adenocarcinoma of the Tongue and Minor Salivary Gland has been recorded in young, middle-aged, and elderly adults; age range 25-70 years (average age of diagnosis is 50 years)
• Both genders are affected and no specific predominance is noted
• CATMSG is a very rare tumor type. However, no ethnic or racial preference has been observed

What are the Risk Factors for Cribriform Adenocarcinoma of the Tongue and Minor Salivary Gland? (Predisposing Factors)

No risks are evident for Cribriform Adenocarcinoma of the Tongue and Minor Salivary Gland. Nevertheless, 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 Cribriform Adenocarcinoma of the Tongue and Minor Salivary Gland? (Etiology)

The exact cause of Cribriform Adenocarcinoma of the Tongue and Minor Salivary Gland formation is unknown. It is suggested that genetic factors may have a role in their formation. However, no specific genetic abnormality is seen.

• 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 Cribriform Adenocarcinoma of the Tongue and Minor Salivary Gland?

Cribriform Adenocarcinoma of the Tongue and Minor Salivary Gland signs and symptoms may include the following:

• CATMSG is observed at the base of the tongue or within the mouth (in the minor salivary glands)
• In the mouth, the tumor may be seen in the palate, lips, behind the jaw (at the back of the mouth), and in the tonsils
• The tumors are generally single and firm with clearly-defined boundaries
• The tumor mass is known to be infiltrative
• Tumor necrosis or hemorrhage is uncommon
• Bleeding and skin ulceration may be observed
• Neurological signs and symptoms, such as facial muscle weakness and pain, due to facial nerve involvement may be seen
• Pain while eating/chewing
• Persistent facial pain at the site of swelling of the tumor; this requires an immediate checkup by a healthcare provider

How is Cribriform Adenocarcinoma of the Tongue and Minor Salivary Gland Diagnosed?

A diagnosis of Cribriform Adenocarcinoma of the Tongue and Minor 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

A differential diagnosis may be necessary to eliminate tumors presenting similar signs and symptoms and for a definitive diagnosis of CATMSG:

• Polymorphous low-grade adenocarcinoma of salivary gland
• Papillary thyroid carcinoma 

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 Cribriform Adenocarcinoma of the Tongue and Minor Salivary Gland?

Complications of Cribriform Adenocarcinoma of the Tongue and Minor 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
• Metastasis of the tumor to the lymph nodes of the neck. Local metastasis is usually present at the time of diagnosis, but no regional spread is seen
• 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 Cribriform Adenocarcinoma of the Tongue and Minor Salivary Gland Treated?

A combination of surgery, chemotherapy, and radiation therapy are used to treat Cribriform Adenocarcinoma of the Tongue and Minor Salivary Gland. The treatment may also depend upon the stage, overall health, age, and pattern/configuration of the tumor.

The treatment measures for CATMSG may involve:

• Wide surgical excision with removal of the entire lesion is the treatment of choice. In case the neck lymph nodes are affected, then a lymph node dissection may be 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 Cribriform Adenocarcinoma of the Tongue and Minor Salivary Gland be Prevented?

• Current medical research has not established a method of preventing the formation of Cribriform Adenocarcinoma of the Tongue and Minor 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 metastasizing potential and chances of recurrence, often several years of active follow-up and vigilance is recommended

What is the Prognosis of Cribriform Adenocarcinoma of the Tongue and Minor Salivary Gland? (Outcomes/Resolutions)

• The prognosis of Cribriform Adenocarcinoma of the Tongue and Minor Salivary Gland is generally good with early diagnosis and prompt treatment
• 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 Cribriform Adenocarcinoma of the Tongue and Minor 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/