Adenoid cystic carcinoma

Infobox_Disease
Name = Adenoid cystic carcinoma


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ICDO = ICDO|8200|3
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MeshID = D003528

Adenoid cystic cancer (AdCC) is an uncommon 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 glands; 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. There is a 70% chance of surviving five years, which drops to 20% for 20 years,Fact|date=May 2008 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. Adjuvant or palliative radiotherapy is commonly given following surgery. 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.

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References

* Neville, Damm, Allen, Bouquot. Oral and Maxillofacial Pathology. 2nd edition.

External links

* [http://www.accrf.org Adenoid Cystic Carcinoma Research Foundation]
* [http://www.accoi.org/FAQSacc.htm FAQ for patients] at Adenoid Cystic Carcinoma Organization International
* [http://www.rare-cancer.org/adenoid-cystic-carcinoma/ Adenoid Cystic Carcinoma Information]
* [http://www.oralcancerfoundation.org/facts/rare/ac/index.htm Adenoid Cystic Carcinoma] at Oral Cancer Foundation