Adenoid Cystic Carcinoma – Symptoms, Diagnosis, Treatment and What to Expect

Adenoid Cystic Carcinoma Symptoms, Diagnosis and Treatment: Adenoid cystic carcinoma (ACC) is a rare form of cancer that develops in the salivary glands. Additionally, it may impact the tear or sweat glands, as well as the tongue, throat, or other areas of your body. Although ACC has a slow growth and high survival rate, it frequently recurs after a prolonged period of time.

Adenoid Cystic Carcinoma

Adenoid cystic carcinoma (ACC) is a very rare kind of cancer that usually originates in the salivary glands or other areas of the head and neck. ACC can develop in numerous different regions of the body, including the breast, skin, and cervix in women, the prostate gland in men, and various other places. ACC tumours have a distinct histological pattern of aberrant “nests” or cords of particular cells (epithelial cells) that surround and/or infiltrate ducts or glandular systems inside the affected organ.

Typically, these structures are filled with a mucous-like substance or have aberrant fibrous membranes within. ACC is classified as low-grade cancer with a history of a slow proliferation rate. Rarely, ACC can become aggressive and invade neighbouring lymph nodes as well as the “sheaths” or coats surrounding nerve fibres (perineural spaces). There may be a potential for this type of cancer to reoccur at the original spot in the future (local recurrence). Although ACC is commonly diagnosed in adults between the ages of 40 and 60, it can also be seen in children and teenagers.

Adenoid Cystic Carcinoma - Symptoms, Diagnosis, Treatment and What to Expect

Adenoid Cystic Carcinoma Symptoms

Adenoid cystic carcinoma can impact several organ systems and body components. Adenoid Cystic Carcinoma symptoms might vary greatly depending on the part of your body affected.

  • ACC of the salivary gland:
    Some common symptoms might be:
    • Facial discomfort.
    • Facial sagging
    • Numbness in the lips or other parts of the face.
  • ACC of the lacrimal gland:

Adenoid cystic carcinoma can occur in the gland that makes tears in certain persons. The example of potential symptoms are:

  • Vision abnormalities
  • Proptosis (bulging eye)
  • Pain and swelling
  • ACC of the skin:

Adenoid cystic carcinoma most impacts the scalp or the external ear canal when it originates in the epidermis. Among the symptoms, the most common ones are:

  • Pain
  • Pus
  • Bleeding
  • Heightened sensitivity
  • Loss of hair in the afflicted region
  • ACC of the lower respiratory tract:

ACC of the lower respiratory tract typically develops in the trachea’s mucous glands. Your windpipe may gradually get blocked as a result of this, leading to:

  • Troubled breathing
  • Stridor (a high-pitched, wheezing sound when breathing in) 
  • Hoarseness
  • ACC of the larynx:

ACC tumours of the larynx (voice box) can harm the region just below the glottis, the slit-like entrance between the vocal cords. Those who have this condition may experience a variety of symptoms, such as:

  • Breathing difficulty (dyspnea).
  • Respiratory problems when exerting oneself.
  • Hoarseness.
  • Voice variations
  • Sore throat (pharyngitis).
  • Swallowing issues
  • A lump in the neck.
  • Pain.
  • ACC of the oesophagus:

Although it’s uncommon, adenoid cystic carcinoma can also form in your oesophagus. If this happens, you could have:

  • Difficulty swallowing liquids, soft meals, and even saliva.
  • Food and liquid regurgitation
  • Loss of weight brought on by the inability to swallow food and beverages.
  • ACC of the cervix:

Adenoid cystic cervical cancer can occur in people, commonly following menopause. It is highly aggressive and is distinguished by a massive cervical tumour. The common signs and symptoms are:  

  • Vaginal discharge.
  • Uterine bleeding
  • Pain.
  • ACC in the prostate:

Prostate ACC leads to the following symptoms:

  • Frequent urination 
  • Sluggish urine flow

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Adenoid Cystic Carcinoma Diagnosis

Your doctor will probably perform a biopsy if they suspect you have adenoid cystic cancer. The tissue sample will be delivered to a pathology lab for further analysis. Additionally, your doctor could do one or more imaging tests, such as:

  • CT scan (Computerised tomography):  A CT scan captures several X-ray pictures and structures them together to create a comprehensive 3-D image.
  • MRI scan (Magnet resonance imaging):  This process takes precise pictures of the interior of your body using radio waves and strong magnets.
  • PET scan (positron emission tomography): This imaging test employs a nontoxic injectable substance (known as a radiotracer) to demonstrate how your organs work in real time.

Adenoid Cystic Carcinoma Treatment

Surgery, followed by radiation therapy, is the standard treatment procedure followed for adenoid cystic cancer.

Your surgeon will perform surgery to remove the tumour as well as some of the surrounding healthy tissue.

Unlike most cancers that spread through the lymph nodes, adenoid cystic carcinoma spreads along the nerves. Your doctor will inspect your nerves to ensure that cancer isn’t present in its vicinity and will make an effort to remove any abnormal tissue without causing any harm to the nerves.

To completely eradicate the tumour, a portion of a nerve may occasionally need to be removed. That may result in facial drooping or facial paralysis in some areas. To restore function, your doctor may attempt to connect the injured nerve to a portion of another nerve.

You might undergo radiation therapy if your doctor is not able to remove the entire tumour without damaging vital organs or if they are concerned cancer has advanced to some place undetected. There are three major categories:

  • External beam radiation therapy: This treatment procedure targets cancer cells with high-energy X-rays or protons in order to eliminate them.
  • Internal radiation therapy commonly known as brachytherapy:  Small radioactive “seeds” will be injected by your doctor into or around the tumour. After a few weeks, the radioactivity of the seeds decreases.
  • Neutron therapy: This treatment mechanism aims at attacking tiny cancer cells by heating the cells with 100 times the energy of traditional radiation therapy. That frequently eliminates cancer cells while allowing the healthy cells around them to regenerate.

Radiation therapy can cause certain side effects, therefore discuss it with your healthcare provider before undergoing any procedure. 

What to expect if you develop Adenoid Cystic Carcinoma

If you develop adenoid cystic tumours, you may observe the development of a lump inside the mouth, especially in the areas under your tongue or inside your cheek. These lumps often progress slowly and are painless. Your voice may sound hoarse or you may have problems swallowing.  You may experience some discomfort or numbness in your face since this form of cancer can travel along nerves.

Visit your doctor if you have any of these symptoms. It might be challenging to completely eradicate cancer. Tumours might reappear years later, either in the same location or, more often, somewhere else, frequently in your lungs. Adenoid cystic carcinoma patients often survive for at least five years following their diagnosis.

You’ll require routine checks following your treatment to monitor for any symptoms of developing malignancies. Depending on the results of your diagnosis, this may need X-rays, CT scans, or MRIs.

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