Sandbox adenoid cystic
| Adenoid cystic carcinoma | |
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| Micrograph of an adenoid cystic carcinoma of a salivary gland (right of image). Normal serous glands, typical of the parotid gland, are also seen (left of image). H&E stain. |
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [2]Associate Editor(s)-in-Chief: Shivali Marketkar, M.B.B.S. [3]
Overview
Adenoid cystic cancer (AdCC) is a very 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 glands; but has also been reported in the breast, lacrimal gland of the eye, lung, brain, bartholin gland, and the trachea. It is sometimes referred to as adenocyst, malignant cylindroma, adenocystic, adenoidcystic, ACC, AdCC.
Pathophysiology
- 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. Despite this tumor most often occurs in the salivary glands, it can also be found in many anatomic sites, including the breast,[1][2] 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[3] of a cohort of 160 ACC patients, disease specific survival was 89% at 5 years but only 40% at 15 years,[4] reflecting deaths from late-occurring metastatic disease.
Microscopic Pathology
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Video
Shown below is a video describing the microscopic features of Adenoid cystic carcinoma {{#ev:youtube|QUDIkWIPYdQ}}
Treatment
Surgery
- Primary treatment for this cancer, regardless of body site, is surgical removal with clean margins. This can be challenging in the head and neck region due to this tumour's tendency spread along nerve tracts.
Medical Therapy
- 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 little data on the positive effects of chemotherapy. Clinical studies are ongoing, however.
- Recently, one of the women with a patient journal was treated with radiofrequency ablation for metastatic tumors in the lung.
- Radiofrequency ablation is a relatively new technique in the treatment of cancers, generally soft-tissue areas such as the liver and lungs, especially ones that make normal surgery not an option.
Case Study
Case #1
In this patient's case, she had 6 small (largest had grown to 2 cm when she went in for surgery) metastatic tumors in both of her lungs. Since surgical removal of both lungs is (obviously) not an option, pinpoint targeting of tumors with a microwave to "cook" the cancerous tumor was a much better option. As far as her doctor could tell, this is the first attempt at using this procedure on AdCC. There has also been some work in the lungs, albeit with other forms of cancer, with cryoablation which freezes rather than cooks the tumor.
References
- ↑ Marchiò C, Weigelt B, Reis-Filho JS (Mar 2010). "Adenoid cystic carcinomas of the breast and salivary glands (or 'The strange case of Dr Jekyll and Mr Hyde' of exocrine gland carcinomas)". J Clin Pathol. 63 (3): 220–8. doi:10.1136/jcp.2009.073908. PMID 20203221.
- ↑ Fusco N, Guerini-Rocco E, Schultheis AM, Badve SS, Reis-Filho JS, Weigelt B (Feb 2015). "The birth of an adenoid cystic carcinoma". Int J Surg Pathol. 23 (1): 26–7. doi:10.1177/1066896914548795. PMID 25185745.
- ↑ Fordice, Jim; Kershaw, Corey; El-Naggar, Adel; Goepfert, Helmuth (February 1999). "Adenoid Cystic Carcinoma of the Head and Neck: Predictors of Morbidity and Mortality". Archives of Otolaryngology--Head & Neck Surgery. 125 (2): 149–52. doi:10.1001/archotol.125.2.149. PMID 10037280.
- ↑ Christopher Moskaluk, MD, PhD, and Henry F. Frierson, Jr., MD. "Adenoid Cystic Carcinoma." [1][dead link][self-published source?]
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