Basal Cell Adenocarcinoma of Salivary Gland

Basal Cell Adenocarcinoma 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)

  • Basal Cell Carcinoma of Salivary Gland
  • Basaloid Salivary Carcinoma
  • Malignant Basal Cell Tumour of Salivary Gland

What is Basal Cell Adenocarcinoma of Salivary Gland? (Definition/Background Information)

  • Basal Cell Adenocarcinoma of Salivary Gland is a type of malignant epithelial tumor, commonly affecting the major salivary (parotid) glands. The tumor is mostly seen in elderly adults
  • The tissue biopsy of the tumor may resemble a benign basal cell adenoma; however, Salivary Gland Basal Cell Adenocarcinoma is a malignant tumor
  • 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 known to be influenced by factors such as exposure to radiation sources and occupational exposure to hazardous chemicals/products
  • The signs and symptoms of Basal Cell Adenocarcinoma of Salivary Gland may include a tumor growth forming a lump, associated with pain in some cases, eating/chewing difficulties, and infiltration to the jawbone
  • The treatment for Basal Cell Adenocarcinoma of 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 is generally good

Who gets Basal Cell Adenocarcinoma of Salivary Gland? (Age and Sex Distribution)

  • Basal Cell Adenocarcinoma of Salivary Gland has only been recorded in adults, with an average age of diagnosis being 60 years
  • Both male and female genders are affected and no ethnic or racial preference has been observed

What are the Risk Factors for Basal Cell Adenocarcinoma of Salivary Gland? (Predisposing Factors)

The following risk factors may be noted for Basal Cell Adenocarcinoma of Salivary Gland:

  • Presence of basal cell adenoma (benign tumor)
  • Family history of basal cell carcinoma (in rare cases)

It is important to note that most basal cell adenocarcinoma tumors arise sporadically (de novo), without a preceding family history.

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 Basal Cell Adenocarcinoma of Salivary Gland? (Etiology)

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

  • Genetic study reports have revealed some of the following abnormalities:
    • Loss of chromosome at 2q24.2 and 4q25-q27
    • Gains of chromosome at 9p21.1-pter, 18q21.1-q22.3, and 22q11.23-q13.31
    • Loss of heterozygosity (LOH) has been observed in 80% of the cases (both familial and sporadic tumors) involving chromosome 16 (specifically at 16q12-13 region). The CYLD gene has been identified in such cases
  • Transformation of a benign basal cell adenoma (BSA) tumor to a basal cell carcinoma (malignancy) is known to take place very infrequently

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 Basal Cell Adenocarcinoma of Salivary Gland?

Basal Cell Adenocarcinoma of Salivary Gland signs and symptoms may include the following:

  • The tumor mass may grow at a slow rate and present no pain. Pain is felt only in rare cases
  • The tumors may be present for weeks, months, or even several years, prior to diagnosis
  • A mass or lump on the side of the face may be observed, since mostly the parotid gland is affected
  • Most tumors are locally infiltrative, but some are well-defined
  • Some individuals with basal cell adenocarcinomas may have other unrelated skin tumors, such as adnexal tumors of skin
  • Most tumors are asymptomatic and no significant signs and symptoms are observed
  • Neurological signs and symptoms, such as facial muscle weakness and pain, due to facial nerve involvement may be seen (in 1 in 4 cases)
  • Pain while eating/chewing
  • Persistent facial pain at the site of swelling of the tumor; this requires an immediate checkup by a healthcare provider
  • Tumor infiltration into the bone
  • Involvement of the lymphatic system may be seen in 25% of the cases

Location of the malignant tumor:

  • Minor salivary glands: The tumors are very rarely observed in the mouth (oral cavity)
  • Major salivary glands: Involvement of the parotid glands is observed in 90% of the cases

How is Basal Cell Adenocarcinoma of Salivary Gland Diagnosed?

A diagnosis of Basal Cell Adenocarcinoma 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

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 Basal Cell Adenocarcinoma of Salivary Gland?

Complications of Basal Cell Adenocarcinoma 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)
  • A high incidence of recurrence of the tumor after surgery is noted
  • Bones may be affected and undergo demineralization
  • Rarely, tumor metastasis to local and distant sites may be noted
  • 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 Basal Cell Adenocarcinoma of Salivary Gland Treated?

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

The treatment measures for Salivary Gland Basal Cell Adenocarcinoma may involve:

  • Wide surgical excision with removal of the entire lesion is the treatment of choice
  • 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 Basal Cell Adenocarcinoma of Salivary Gland be Prevented?

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

What is the Prognosis of Basal Cell Adenocarcinoma of Salivary Gland? (Outcomes/Resolutions)

  • The prognosis of Basal Cell Adenocarcinoma of Salivary Gland is generally good with early diagnosis and prompt treatment. The rate of metastasis is low; also, metastasis to distant sites is very uncommon. Very few deaths due to this malignancy have been reported
  • 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 Basal Cell Adenocarcinoma 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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