Adenoid cystic carcinoma
Encyclopedia
Adenoid cystic cancer is a rare type of cancer
that can exist in many different body sites. It most often occurs in the areas of the head and neck, in particular the salivary gland
s; but has also been reported in the breast
, lacrimal gland
of the eye
, lung
, brain
, bartholin gland, trachea
, and the paranasal sinuses. It is sometimes referred to as adenocyst, malignant cylindroma, adenocystic, adenoidcystic, ACC, AdCC.
It is the second most common malignant salivary gland tumor overall (after mucoepidermoid carcinoma
). It represents 28% of malignant submandibular gland tumors, making it the single most common malignant salivary gland tumor in this region. Patients may survive for years with metastases because this tumor is generally well-differentiated and slow growing. In a 1999 study of a cohort of 160 ACC patients, disease specific survival was 89% at 5 years but only 40% at 15 years, reflecting deaths from late-occurring metastatic disease.
or palliative radiotherapy is commonly given following surgery. In some cases surgery is not a reasonable option, however radiation used alone may provide an effective treatment.
Chemotherapy
is used for metastatic disease. Chemotherapy is considered on a case by case basis, as there is limited trial data on the positive effects of chemotherapy. Clinical studies are ongoing, however.
Cancer
Cancer , known medically as a malignant neoplasm, is a large group of different diseases, all involving unregulated cell growth. In cancer, cells divide and grow uncontrollably, forming malignant tumors, and invade nearby parts of the body. The cancer may also spread to more distant parts of the...
that can exist in many different body sites. It most often occurs in the areas of the head and neck, in particular the salivary gland
Salivary gland
The salivary glands in mammals are exocrine glands, glands with ducts, that produce saliva. They also secrete amylase, an enzyme that breaks down starch into maltose...
s; but has also been reported in the breast
Breast
The breast is the upper ventral region of the torso of a primate, in left and right sides, which in a female contains the mammary gland that secretes milk used to feed infants.Both men and women develop breasts from the same embryological tissues...
, lacrimal gland
Lacrimal gland
The lacrimal glands are paired almond-shaped glands, one for each eye, that secrete the aqueous layer of the tear film. They are situated in the upper, outer portion of each orbit, in the lacrimal fossa of the orbit formed by the frontal bone. Inflammation of the lacrimal glands is called...
of the eye
Human eye
The human eye is an organ which reacts to light for several purposes. As a conscious sense organ, the eye allows vision. Rod and cone cells in the retina allow conscious light perception and vision including color differentiation and the perception of depth...
, lung
Human lung
The human lungs are the organs of respiration in humans. Humans have two lungs, with the left being divided into two lobes and the right into three lobes. Together, the lungs contain approximately of airways and 300 to 500 million alveoli, having a total surface area of about in...
, brain
Human brain
The human brain has the same general structure as the brains of other mammals, but is over three times larger than the brain of a typical mammal with an equivalent body size. Estimates for the number of neurons in the human brain range from 80 to 120 billion...
, bartholin gland, trachea
Vertebrate trachea
In tetrapod anatomy the trachea, or windpipe, is a tube that connects the pharynx or larynx to the lungs, allowing the passage of air. It is lined with pseudostratified ciliated columnar epithelium cells with goblet cells that produce mucus...
, and the paranasal sinuses. It is sometimes referred to as adenocyst, malignant cylindroma, adenocystic, adenoidcystic, ACC, AdCC.
It is the second most common malignant salivary gland tumor overall (after mucoepidermoid carcinoma
Mucoepidermoid carcinoma
Mucoepidermoid carcinoma is the most common type of salivary gland malignancy in children. Mucoepidermoid carcinoma can also be found in other organs, as bronchi, lacrimal sac and thyroid.Mucicarmine staining is one stain used by pathologist for detection....
). It represents 28% of malignant submandibular gland tumors, making it the single most common malignant salivary gland tumor in this region. Patients may survive for years with metastases because this tumor is generally well-differentiated and slow growing. In a 1999 study of a cohort of 160 ACC patients, disease specific survival was 89% at 5 years but only 40% at 15 years, reflecting deaths from late-occurring metastatic disease.
Treatment
Primary treatment for this cancer, regardless of body site, is surgical removal with clean margins. This surgery can prove challenging in the head and neck region due to this tumour's tendency spread along nerve tracts. AdjuvantAdjuvant
An adjuvant is a pharmacological or immunological agent that modifies the effect of other agents, such as a drug or vaccine, while having few if any direct effects when given by itself...
or palliative radiotherapy is commonly given following surgery. In some cases surgery is not a reasonable option, however radiation used alone may provide an effective treatment.
Chemotherapy
Chemotherapy
Chemotherapy is the treatment of cancer with an antineoplastic drug or with a combination of such drugs into a standardized treatment regimen....
is used for metastatic disease. Chemotherapy is considered on a case by case basis, as there is limited trial data on the positive effects of chemotherapy. Clinical studies are ongoing, however.
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External links
- Adenoid Cystic Carcinoma Research Foundation
- FAQ for patients at Adenoid Cystic Carcinoma Organization International
- Adenoid Cystic Carcinoma Information
- Cancer.Net: Adenoid Cystic Carcinoma
- Adenoid Cystic Carcinoma at Oral Cancer Foundation