Adenoid cystic carcinoma (sometimes referred to as adenocyst, malignant cylindroma, adenocystic, adenoidcystic, ACC or AdCC.) is a rare type of cancer that can exist in many different body sites. This tumor most often occurs in the salivary glands, but it can also be found in many anatomic sites, including the breast, lacrimal gland, lung, brain, bartholin gland, trachea, and the paranasal sinuses.
It is the third most common malignant salivary gland tumor overall (after mucoepidermoid carcinoma and polymorphous low grade adenocarcinoma). 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.
Video Adenoid cystic carcinoma
Cause
Maps Adenoid cystic carcinoma
Diagnosis
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 to spread along nerve tracts. Adjuvant or palliative radiotherapy is commonly given following surgery. For advanced major and minor salivary gland tumors that are inoperable, recurrent, or exhibit gross residual disease after surgery, fast neutron therapy is widely regarded as the most effective form of 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.
Images
See also
- Primary cutaneous adenoid cystic carcinoma
- Collagenous spherulosis
References
Further reading
- Neville, Damm, Allen, Bouquot. Oral and Maxillofacial Pathology. 2nd edition.
External links
- SinusTumor.org - an educational site that provides information about sinus tumors
- 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
- [2] Search for clinical trials at ClinicalTrials.gov
- [3] Search for research literature at PubMed.gov
- [4] Search for consumer health information at Medlineplus.gov
- Cancer.Net: Lacrimal Gland Tumor
Source of article : Wikipedia
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