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​Adenoid Cystic Carcinoma

What is Adenoid Cystic Carcinoma?

Adenoid cystic carcinoma is a rare form of adenocarcinoma, which is a type of cancer.

It can exist in many different body sites and most often occurs in the areas of the head and neck — in particular the salivary glands. Adenoid cystic carcinoma has also been reported in the breast, lacrimal gland of the eye, lung, brain, bartholin gland, and trachea.

Compared with most other carcinomas, adenoid cystic carcinoma has a slow course with a survival rate of 89 percent at five years. Tumors progress persistently and often recur, resulting in a survival rate of only 40 percent after 15 years.

Doctors treat adenoid cystic carcinoma as a chronic condition.

Remote metastases can occur a decade or more after initial treatment of the primary tumor, with the most common metastatic locations being the lung, bone, and viscera.

The preferred treatment at UPMC for adenoid cystic carcinoma of the sinuses and skull base is the Endoscopic Endonasal Approach (EEA). This innovative, minimally invasive surgical technique uses the nose and nasal cavities as natural corridors to access hard-to-reach or previously inoperable tumors.

The benefits of EEA treatment include:

  • No incisions to heal
  • No disfigurement
  • Faster recovery time

Diagnosing Adenoid Cystic Carcinoma

Adenoid cystic carcinoma tumors have a distinctive pattern in which abnormal bundles of epithelial cells surround or infiltrate ducts or glandular structures within the affected organ.

To diagnose adenoid cystic carcinoma, your doctor will perform a physical exam, ask you about your symptoms, and order diagnostic tests.

Adenoid cystic carcinoma symptoms

Symptoms vary depending on the site and origin of the tumor.

Tumor Site ​Symptoms
Salivary glands ​Painless, slow-growing masses in the mouth or face
Lacrimal glands ​Bulging of the eyes and changes in vision
Tracheobronchial tree ​Respiratory symptoms, such as shortness of breath (dyspnea)
Larynx Changes in speech

Pain, numbness, and nerve paralysis may accompany advanced tumors because they can infiltrate and spread along nerves.

Tests for diagnosing adenoid cystic carcinoma

To confirm a diagnosis of adenoid cystic carcinoma, your doctor will order a biopsy. A pathologist will evaluate the tumor cells microscopically.

Imaging tests, such as MRI and CT scans, usually can identify recurrences of tumors.

Adenoid Cystic Carcinoma Treatments

Surgery

The standard treatment for adenoid cystic carcinoma is surgical removal of the cancerous (malignant) tissue. The surgeon will remove the tumor and an area of tissue surrounding it.

The presence of clean margins, meaning a minimum of 2 mm of tissue is cancer-free surrounding the tumor, signals total tumor removal and gives the best chance that cancer will not recur at that site.

Endoscopic Endonasal Approach (EEA)

Adenoid cystic carcinomas of the sinuses and skull base can be approached directly using the Endoscopic Endonasal Approach (EEA). This state-of-the-art, minimally invasive approach allows surgeons to access the tumor through the natural corridor of the nose, without making an open incision. Surgeons then remove the adenoid cystic carcinoma through the nose and nasal cavities.

EEA offers the benefits of no incisions to heal, no disfigurement, and a faster recovery time.

If you need complementary treatments, such as radiation, those therapies can begin soon after EEA surgery.

Radiation therapy

Surgery usually is followed by radiation therapy.

Doctors may recommend neutron radiation therapy to treat adenoid cystic carcinoma. Compared with conventional radiation therapy, neutrons can deliver 20 to 100 times more energy along their path length, causing much greater damage to the cancer cells.

Neutron radiation therapy has been tested on many different types of tumors, with salivary gland tumors showing the greatest benefit. In certain instances, it may be the treatment of choice for adenoid cystic carcinoma, particularly in areas of the body where it is difficult to perform surgery.

Neutron therapy carries more severe side effects than conventional radiation therapy, such as severe sore mouth and/or throat and difficulty swallowing. Therefore, neutron therapy is used more often in cases where the disease is inoperable or recurrent.


Endoscopic Endonasal Approach (EEA)
Pituitary Tumor Removal Using the Endoscopic Endonasal Approach (EEA) at UPMC

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