Sinonasal Carcinoma

Sinonasal carcinomas are a rare group of malignancies that develop in the nasal cavity, nasopharynx (located at the uppermost portion of the throat, behind the nose and above the soft palate), or paranasal sinuses (air-filled spaces behind the forehead, nasal bones, cheeks and eyes).

Sinonasal carcinoma is more common in men, and is generally diagnosed between the ages of 45-85. However, anyone can develop this disease.

Types of Sinonasal Carcinoma

There are several different types of sinonasal carcinoma, which are categorised by the types of cells they develop from.

Sinonasal Squamous Cell Carcinoma

Sinonasal squamous cell carcinomas are the most common type of sinonasal carcinoma, and develops from the squamous cells that line the nasal cavity and paranasal sinuses. This type of tumour is often aggressive, can have a high recurrence rate, and may not have as good of a prognosis as other types of sinonasal cancer.

Sinonasal Adenocarcinomas

Sinonasal adenocarcinomas are the second most common type of sinonasal carcinoma, and develops from the mucus-producing cells in the nasal cavity and paranasal sinuses. They can be further categorised into:

  • Salivary type (develop from seromucinous glands and surface epithelium and are similar to those found in salivary gland cancers).
  • Non-salivary type (develop from surface epithelium alone), which is further subdivided into:
    • Intestinal type (cancer cells resemble carcinomas or adenomas of the intestines).
    • Non-intestinal type (cancer cells don’t resemble salivary type adenocarcinomas or intestinal type adenocarcinomas).

This type of cancer is often aggressive and can have a high recurrence rate, however they can have an improved prognosis when caught early.

Rare Types of Sinonasal Carcinomas

Rare types of sinonasal carcinomas include:

  • Sinonasal adenoid cystic carcinoma.
  • Sinonasal undifferentiated carcinoma.
  • NUT carcinoma / NUT midline carcinoma of sinonasal cavity.
  • SWI/SNF complex deficient sinonasal carcinoma.
  • Teratocarcinosarcoma.
  • HPV related multiphenotypic sinonasal carcinoma.
  • Sinonasal lymphoepithelial carcinoma.
  • Sinonasal neuroendocrine carcinoma.

Treatment

If a sinonasal carcinoma is detected, it will be staged and graded based on size, metastasis, and how the cancer cells look under the microscope. Staging and grading helps your doctors determine the best treatment for you.

Cancers can be staged using the TNM staging system:

  • T (tumour) indicates the size and depth of the tumour.
  • N (node) indicates whether the cancer has spread to nearby lymph nodes.
  • M (metastasis) indicates whether the cancer has spread to other parts of the body.

This system can also be used in combination with a numerical value, from stage 0-IV:

  • Stage 0: this stage describes cancer cells in the place of origin (or ‘in situ’) that have not spread to nearby tissue.
  • Stage I: cancer cells have begun to spread to nearby tissue. It is not deeply embedded into nearby tissue and had not spread to lymph nodes. This stage is also known as early-stage cancer.
  • Stage II: cancer cells have grown deeper into nearby tissue. Lymph nodes may or may not be affected. This is also known as localised cancer.
  • Stage III: the cancer has become larger and has grown deeper into nearby tissue. Lymph nodes are generally affected at this stage. This is also known as localised cancer.
  • Stage IV: the cancer has spread to other tissues and organs in the body. This is also known as advanced or metastatic cancer.

Cancers can also be graded based on the rate of growth and how likely they are to spread:

  • Grade I: cancer cells present as slightly abnormal and are usually slow growing. This is also known as a low-grade tumour.
  • Grade II: cancer cells present as abnormal and grow faster than grade-I tumours. This is also known as an intermediate-grade tumour.
  • Grade III: cancer cells present as very abnormal and grow quickly. This is also known as a high-grade tumour.

Once your tumour has been staged and graded, your doctor may recommend genetic testing, which analyses your tumour DNA and can help determine which treatment has the greatest chance of success. They will then discuss the most appropriate treatment option for you.

Treatment is dependent on several factors, including location, stage of disease and overall health.

Treatment options for sinonasal carcinoma may include:

  • Surgery to remove as much of the tumour as possible.
  • Radiation therapy.
  • Chemotherapy.
  • Clinical trials.
  • Palliative care.

Risk factors

While the cause of sinonasal carcinoma remains unknown, the following factors may increase the likelihood of developing the disease:

  • Being exposed to certain workplace substances and chemicals, potentially including:
    • Leather dust.
    • Glues.
    • Formaldehyde.
    • Chrome.
    • Nickel.
    • Flour.
    • Textiles.
    • Organic solvents.
  • Having a history of smoking.
  • Being infected with the human papillomavirus (HPV).
  • Having nasal polyposis.
  • Having an inverted sinonasal papilloma.
  • Having chronic sinusitis.
  • Having previous radiation therapy for the treatment of retinoblastoma.

Not everyone with these risk factors will develop the disease, and some people who have the disease may have none of these risk factors. See your general practitioner (GP) if you are concerned.

Symptoms

Symptoms of sinonasal carcinomas may include:

  • Persistent blocked sinuses.
  • Sinus pressure.
  • Headaches.
  • Pain in the sinus area.
  • Runny nose.
  • Nosebleeds.
  • A lump or sore inside of the nose that does not heal.
  • A lump on the face or roof of the mouth.
  • Numbness and/or tingling in the face.
  • Swelling or difficulties with the eyes, potentially including:
    • Diplopia.
    • Blurriness.
    • Eyes pointing in different directions.
  • Pain in the upper teeth and/or loose teeth.
  • Pain and/or pressure in the ear.

Not everyone with the symptoms above will have cancer, but see your general practitioner (GP) if you are concerned.

Some of the information regarding symptoms was obtained from the Paranasal Sinus and Nasal Cavity Cancer Treatment (Adult) (PDQ) page published by the National Cancer Insitute.

Diagnosis

If your doctor suspects you have a sinonasal carcinoma, they may order the following tests to confirm the diagnosis and refer you to a specialist for treatment:

  • Physical examination.
  • Imaging tests, potentially including:
    • CT (computed tomography) scan.
    • MRI (magnetic resonance imaging).
    • X-ray of the head and neck.
  • Blood tests.
  • Nasoendoscopy.
  • Biopsy.

References

Keep up with Rare Cancers Australia

Inside Rare is a monthly newsletter that shares the latest news, events and stories connecting the rare community.