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Management of Adenoid Cystic Carcinoma of the Head and Neck

Adenoid cystic carcinoma (ACC) is a type of cancer that affects surrounding areas like the salivary glands, head and neck. However, it can also occur in other parts of the body, such as breast tissue, skin, prostate, and cervix. 

This type of cancer is relatively rare in comparison to other types of cancer. And it arises from the exocrine glands such as salivary glands or the external auditory canal. Only one percent of them affect the head and neck. The tumor may be solid, hollow, round, or perforated. Women are more susceptible to this cancer than men and are common in groups aged 40 to 60 years. 

What are the signs and symptoms?  

This cancer affects many parts of the body, so symptoms depend on which part of the body it affects. ACC in the salivary glands can cause facial pain, sagging, or numbness of the lips and surroundings. When ACC affects the tear duct, it can cause vision problems, eye swelling, pain, and swelling in the area near the tear duct. ACC, which affects the skin, can cause pain, bleeding, accumulation of pus, hair loss, and increased pain sensation in the affected area. The joints near the areola usually develop when they affect your breast. In the case of the cervix, there may be vaginal discharge and bleeding as well as pain. ACC of the prostate can lead to frequent urination and poor urine flow. 

What could possibly cause it?

We found the involvement of certain genes in this type of cancer. Several genes NFIB, MYB, MYBL1, and SPEN may play a role in the onset of the disease. Any abnormality in these genes can lead to an increased risk of this cancer. If there is a mutation in these genes, it can lead to changes in specific biological pathways that lead to the formation of cancer cells that grow and become aggressive even during treatment.

In addition to these, certain lifestyle choices can contribute to an increased risk of the disease. One of these factors is that smoking and alcohol consumption can have a significant impact on patient’s response to treatments. BMI or body mass index is a factor that can contribute to this cancer. Research shows that nutrition and diet can negatively affect treatment, may promote healing, or play no role at all. Therefore, diet planning and nutrition focus are essential for faster recovery and possibly improved quality of life.

Types of treatment for ACC

Several treatments are available for ACC out of which radiation therapy and surgery are the most common. Management in such a situation depends on many factors, some of which include cell type and degree of differentiation and the condition of the lymph nodes, and the presence of bones. Therapeutic management should also consider preserving speech and swallowing function and also deal with the complications that may arise in the course of treatment. The treatment of ACC is becoming frenzied due to its unreliable occurrence and nature, repeated and late recurrence, and sometimes longevity compounded by recurrent and metastatic nature. However, a review of the studies shows that the best survival outcomes are the combination of surgery and radiation. 

Surgery may be the mainstay of treatment or is in combination with radiation therapy. Typical surgical indications include tumors involving bone, tumors unresponsive to radiation, tumor recurrence at the initial radiation site, and when side effects are less desirable than with radiation therapy. Often, surgery is necessary to reduce the volume of the tumor, for example by facilitating drainage. Surgical failure is often attributed to incomplete resection, failure to obtain clear margins, nucleation of the tumor, undetected lymphatic or hematogenous spread, nerve invasion, or perivascular spread. In cases of ACC, both initial surgical treatment and repeated surgical resection of resectable relapsed lesions, including both local and lung metastases, resulted in longer survival.

Radiation therapy

Radiation therapy is rarely in use for the treatment of ACC. One must not forget, ACC though radiosensitive, are not radio-curative. Though the initial response is encouraging, these tumors did recur. Doses of 60 Gy or more were of benefit when the minimal residual microscopic disease was evident. Though the best results are with the combination of radical surgery and radiation, there are no trials that prove the value of adjunctive radiation therapy.


In a follow-up study by Sloan Kettering, among patients receiving radiation alone, 96% had tumor regression but 93% relapsed, and half of them relapsed in 18 months. As a part of palliative care, radiation may provide symptomatic relief from pain, ulceration, bleeding, and pharyngeal obstruction. For advanced tumors, radiation therapy is rarely curative. In another follow-up study, recommended neutron radiotherapy was used for tumors that were unresectable, associated with high surgical morbidity and postoperative tumor burden after surgery.

Chemotherapy use for ACC is controversial as it has shown a limited and poorly defined role. It is often administered for palliation. Few studies have shown cisplatin, fluorouracil, doxorubicin, and cyclophosphamide to have some activity, either as single agents or in combination. An interesting alternative was involving a super-selective intracranial application of cisplatin. This resulted in complete local remission and also in pulmonary dissemination of the tumor. Some research suggests chemotherapy is ineffective while others recommend it as palliative treatment in advanced cases of ACC.

Summing up

ACC is a rare but malignant cancer and head and neck cancer only account for one percent of the cases of the ACC. Still, a lot of research has to be put into bolstering the diagnosis and treatment methods for the head and neck ACC. This will help to improve the quality of life of the patients and also their survival rate.


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