Adenoid cystic carcinoma (ACC) is a type of cancer that usually affects surrounding areas such as the salivary glands and 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 compared to other types of cancer. And only 1 percent of them originate from exocrine glands such as salivary glands and ear canals and affect the head and neck. The tumour may be solid, hollow, round, or perforated. Women are more susceptible to this cancer than men and often appear in groups between the ages of 40 and 60.
What are the signs and symptoms?
This cancer affects many parts of the body, so symptoms depend on which part of the body is affected. Salivary gland ACC can cause facial pain, sagging, and numbness in the lips and surroundings. When ACC affects the lacrimal duct, it can cause blurred vision, swelling of the eyes, pain, and swelling near the lacrimal duct. ACC, which affects the skin, can cause pain, bleeding, pus accumulation, hair loss, and increased sensation of pain in the affected area. The joints near the areola usually develop when they affect the breast. In the case of the cervix, there may be vaginal discharge and bleeding as well as pain. ACC in the prostate can lead to frequent urination and reduced urinary flow.
What is the cause?
It turns out that certain genes are involved in this type of cancer. Several genes, NFIB, MYB, MYBL1, and SPEN, can play a role in the development of the disease. Abnormalities in these genes can increase the risk of this cancer. Mutations in these genes can alter certain biological signalling pathways, resulting in the formation of cancer cells that grow and become aggressive even during treatment. In addition, certain lifestyle choices can contribute to an increased risk of illness. One such factor is that smoking and drinking can have a significant impact on a patient’s response to the prescribed treatment. BMI or body mass index is a potential contributor to this cancer. Studies show that diet and nutrition can adversely affect treatment, promote healing, or play no role at all. Therefore, diet and nutrition supplements are essential for faster recovery and potentially improved quality of life.
Recent whole-genome and exome sequencing has dramatically improved our understanding of the aetiology of ACC. Balanced translocations leading to the MYBNFIB fusion gene appear to be a fundamental feature of ACC. In addition, sequencing has identified many other driver genes that are mutated in downstream signalling pathways common to other well-studied cancers. Overexpression of carcinogenic proteins involved in cell proliferation, adhesion, cell cycle regulation, and angiogenesis is also present in ACC. Overall, studies have identified genes and proteins for targeted mechanism-based therapies based on tumour phenotype, as opposed to non-specific cytotoxic drugs. In addition, although few studies in ACC currently exist, immunotherapy may also hold promise. Better genetic understanding will enable treatment with novel targeted agents and initial exploration of immune-based therapies with the goal of improving outcomes for patients with ACC.
Let’s discuss a few of them:
Targeted Therapies: Targeted therapies have been extensively studied and tested in multiple clinical trials to develop practical therapeutic approaches for managing ACC. The study is investigating the effectiveness and usefulness of anti-angiogenic therapy. The study also focuses on understanding the molecular mechanisms that regulate ACC transmission along nerves by paying clear attention to mutations in the NOTCH1 gene. Understanding these genetic mutations can give doctors clues as to how tumours respond to targeted therapies.
Immunotherapy: It is a relatively newer approach but holds many promises for treatment with minimum side effects and effectiveness even in the advanced stages. Cancer cells can be eliminated by the immune system effectively. Clinical trials are being carried out to find an effective way to draw the maximum advantage of this treatment.
Anti-angiogenesis / TKI: Anti-angiogenesis therapy is a special form of targeted therapy. Today, it is an active area of research and research for the treatment of ACC cancer. Treatment focuses on stopping the formation of new blood vessels or angiogenesis. The purpose of anti-angiogenesis is to deprive the tumour of all nutrients. Tumours nourish, grow, and spread by absorbing nutrients supplied by blood vessels. Therefore, stopping blood vessels causes the tumour to starve. Studies are analysing how this treatment, often given in the form of specific angiogenesis inhibitors called TKI/tyrosine kinase inhibitors, can help treat ACC patients. Drugs such as axitinib (Inlyta) and lenvatinib (Lenvima) are tyrosine kinase inhibitors that have been studied and evaluated.
Supportive Care: Supportive care is essential for the treatment and cure of cancer. Many types of research and clinical trials are underway to develop better ways to treat and alleviate the symptoms and side effects of ACC treatment. It focuses on providing services to improve the quality of life and comfort of patients.
There are many ways of treating ACC but we need to find a way that can prevent the recurrence and stop this disease from spreading or metastasis. It is said that a combination of treatments is better than a single one carried out alone. For example, chemotherapy plus radiation gives better results than those carried out alone. Some research suggests that surgery and radiation together give better results and even give a higher survival rate for the patients. Targeted therapy is in early development and needs to be explored further. The same goes for therapies using the genomic sequences as well. A lot of work needs to be done before we can delve further and find a methodological approach to treat this disease to improve survival rate and quality of life.