Q&A: Acinic cell carcinoma of the parotid

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Acinic cell carcinoma Tsimberidou_Doctor.JPGAcinic cell carcinoma (ACC) of the parotid is a rare tumor and therefore information compared to other tumor types is relatively limited. 

Answering questions about ACC is Apostolia Tsimberidou, M.D., Ph.D., associate professor in the Department of Investigational Cancer Therapeutics at MD Anderson.

What is acinic cell carcinoma?

Acinic (or acinar) cell carcinoma of the parotid gland is a rare tumor that represents approximately 10% of salivary gland tumors. This tumor is usually a low-grade, highly differentiated carcinoma. Women are affected with salivary gland tumors more than men, and the average age of diagnosis of all salivary gland tumors is approximately 44 years old.

What causes ACC?
While the cause of salivary gland tumors is unknown, risk factors include: cigarette smoking, genetic predisposition, viral infections, rubber manufacturing, plumbing equipment and some types of woodworking, as well as asbestos mining and exposure to nickel compounds. The only well-established risk factor is ionizing radiation.

Workplace exposure to certain radioactive substances may also increase the risk of salivary gland cancer. In rare instances, members of some families seem to have a higher than usual risk of developing this type of cancer.

Is ACC preventable?
The clear causes are unknown. As with every type of cancer, avoidance of potential risk factors might help prevent this disease.

What are common symptoms of ACC?
Clinically, patients typically present with a slowly enlarging mass in the parotid region. Pain is a symptom in more than 33% of patients.

How is ACC treated?
Surgical excision is the most common treatment for ACC, but radiation therapy may be used in some cases. The facial nerve is in the salivary gland and should be preserved if not involved with the tumor.

Most deep parotid tumors require postoperative radiation therapy because of the limitations in resection of these tumors, or radiation therapy is given as additional therapy for tumors that are intermediate or high grade.

Various chemotherapeutic agents may be used to treat advanced ACC.

What are the survival rates and statistics for those diagnosed with ACC?

Staging is likely a better predictor of outcome than histological grading. According to data from the Atlas of Genetics and Cytogenetics in Oncology and Haematology, "Acinic cell carcinoma tends to recur in 35% of cases, and the rate of disease-associated death is about 16%."

Are there clinical trials available for ACC patients?

Patients with ACC are usually candidates for the same treatment as patients with other tumors of the salivary gland.

To enable the selection of optimal therapy, patients can also enroll in tumor molecular profiling, identification of molecular aberrations or new clinical trials as they become available.

What advice would you give to newly diagnosed or current patients with ACC?
To new patients, I recommend that they be treated by a group that can obtain multidisciplinary management including oncologists, radiation therapists, pathologists, and radiologists.

The use of molecular profiling to identify genetic markers that drive tumor pathogenesis, progression and participation in clinical trials with targeted agents using the "personalized medicine" approach (treating a patient with a drug that inhibits the function of the specific molecular aberration that the patient may have) is expected to improve the clinical outcomes of patients with this disease, as with other types of cancer.

Current patients should work with their physician to closely monitor their disease.

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My husband has been diagnosed with metastatic acinic cell carcinoma. The cancer has moved to his lung and spine. We've been told this is very rare. Do you have any research on this?

Hi Tanya, I'm sorry to hear about your husband. We do have a blog post with some information on acinic cell carcinoma: If you have more questions, please contact Ask MD Anderson at 1-877-632-6789. Your husband is in our thoughts and prayers.

Hello Tanya, my husband was diagnosed with metastatic acinic cell carcinoma as well. The cancer has moved to his lungs; we're in the midst of trying to treat this very rare cancer. We did do some genetic testing and found only one trial. Have you done the same? Where are you with his treatments?

My friend was diagnosed with acinic cell carcinoma in 2008 and had surgery in 2008, 2009, and neutron radiation in August of 2009. PET scans were negative in 5/2012, 11/2012, and 4/2013. PET scan in 3/2014 showed hypermetabolic focus contacting the temporal bone. Followed up with MRI. Local recommendation is surgery with removal of the rest of the parotid, nerve, and some temporal bone. We have been told that he will lose hearing in his left ear and will have left facial paralysis. They will transplant a nerve from his calf and hopefully he will regain some facial movement after some months. They also plan on doing a free flap transplant from his forearm or chest. This all sounds very drastic and life changing. Does this sound like the best course of treatment or does MD Anderson have any other suggested protocols?

Unfortunately, we cannot give medical advice online. It's best if your friend talks to a doctor. They can call 1-877-632-6789 to speak to one of our health information specialists or schedule an appointment. Your friend is in our thoughts and prayers.

Hi I was diagnosed last May (2013) and had it removed 8/2013. They believe they got it all, it was all inside the gland. I wonder since all they are doing is followup in 1 year with another MRI. Do you think I am being to relaxed about this or should I be more aggressive? My sister died of Triple X Breast cancer so there is family history.... anyone with any suggestions?? Those that have had it matastize .. how early was it caught? I am one of those "rare" people that if it can happen.. it usually does :( thanks!

Hi Kara, every case is different, so it's best to talk to your doctor.

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