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5 Rare Cancers: What You Need To Know

July 27, 2021

Chances are if you have a sore throat, stuffy nose or ear pain, it’s nothing serious. But some of the rarest and little-known cancers involve the head, neck and throat, so it’s smart to be aware of the symptoms that can mean something more than a cold or allergies. 

A type of cancer is classified as rare if it affects fewer than 40,000 people in the United States each year. Oral cavity, pharynx, larynx, sinus/nasal and salivary gland cancers fall into this category. You may not have heard of these because they only make up 3 percent of cancer cases. 

Here’s a look at each of these five cancers, their risk factors, symptoms and treatment.

1. Oral Cavity Cancer

Oral cancer affects the mouth and lips, including the inner cheeks, the area under your tongue, the back of the tongue, gums around the bottom of your teeth and around the wisdom teeth and roof of the mouth. 

Symptoms of oral cancer may include: 

  • Difficulty breathing, chewing or swallowing

  • Feeling like something is stuck in your throat 

  • Lump on the cheek

  • Mouth sores

  • Mouth pain that doesn’t go away

  • Halitosis (chronic bad breath)

  • Red or white patches on the gums or lining of your mouth 

Early symptoms of oral cancer can be mistaken for something more benign, like tooth pain. If you have pain or symptoms that persist more than eight weeks, you should see your doctor. 

Alcohol and tobacco use are associated with nearly 75 percent of oral cancers. Other risk factors include human papillomavirus (HPV), sun exposure and poor diet.

Most oral cancers are treated with surgery. If detected in later stages, radiation and/or chemotherapy may also be part of the treatment regimen. 

2. Pharyngeal Cancers 

The pharynx is a tube-like structure that starts at the back of the nose and ends at the voice box. Cancer of the pharynx, or pharyngeal cancer, is classified into three different subtypes: 

  • Nasopharyngeal: in the back of nose

  • Oropharyngeal: in the back of mouth just below the nasopharynx

  • Hypopharyngeal: in the back of the larynx 

Nasopharyngeal Cancer 

Many people with this cancer may notice a lump or mass in the neck. These lumps are typically not painful or tender. Other symptoms include: 

  • Chronic sore throat

  • Nosebleeds

  • Recurrent ear infections

  • Ringing in the area or a feeling of fullness in the ear

  • Stuffy nose

  • Trouble breathing 

Risk factors for nasopharyngeal cancer include a previous infection of Epstein-Barr virus (commonly known as “mono”), tobacco and alcohol use, workplace exposure to chemicals and poor diet. 

Depending on the stage of cancer when found, treatment typically includes surgery, and potentially radiation and/or chemotherapy. 

Oropharyngeal Cancer 

Oropharyngeal includes the tonsils, back of the tongue, soft palate and the walls on the middle of the throat. Symptoms of oropharyngeal cancer include: 

  • Chronic sore throat

  • Coughing up blood

  • Lump in the neck

  • Trouble swallowing, moving the tongue or fully opening the mouth 

Heavy alcohol use, long-term tobacco use and being infected with HPV are all risk factors for oropharyngeal cancer.  

Treatments for oropharyngeal cancer include surgery, radiation, chemotherapy and in some cases, immunotherapy

Hypopharyngeal Cancer 

Hypopharyngeal cancer affects the area behind the larynx, which is also called the voice box. Signs and symptoms of hypopharyngeal cancer include: 

  • Change in voice

  • Ear pain

  • Difficulty/ pain swallowing

  • Lump in the neck

  • Sore throat that doesn’t go away

  • Coughing blood 

Alcohol and tobacco use, excess body weight, HPV, poor nutrition and workplace exposure to chemicals are all risk factors for developing this type of cancer. 

Depending on the stage of cancer, treatment options include surgery, radiation, chemotherapy and immunotherapy. 

Patients with any type of pharyngeal cancer are typically treated by a multidisciplinary team, including a medical oncologist, radiation oncologist, nutritionists, plastic surgeons, head and neck surgeons, psycho-social therapists and speech therapists. 

3.     Larynx Cancer

Cancer of the larynx (voice box) occurs when cancer cells form around the throat between the base of the tongue and trachea, including the vocal cords. Symptoms of laryngeal cancer include: 

  • Chronic sore throat and/or cough

  • Difficulty swallowing

  • Ear pain

  • Hoarseness in the voice

  • Lump in the neck or throat

  • Difficulty breathing 

Heavy alcohol and tobacco use and HPV are risk factors for developing laryngeal cancer. Standard treatment includes radiation, surgery, chemotherapy and immunotherapy. One or more of these treatments may be used, depending on the stage of the cancer. 

4. Nasal and Sinus Cancer

Nasal cancers affect the inside of the nose. Paranasal sinus cancers start in spaces in the skull and around or near the sinuses. 

Signs and symptoms of nasal and paranasal sinus cancers may include: 

  • Blockage in one side of the nose

  • Chronic nasal congestion

  • Headache

  • Hearing loss

  • Nosebleeds

  • Problems with sense of smell

  • Numbness in the face and/or teeth

  • Watery eyes and/or changes in vision 

Workplace exposure to chemicals and dusts, smoking tobacco and HPV are all risk factors for developing nasal and sinus cancers. Surgery, radiation therapy and chemotherapy are the three standard treatments for these types of cancer. One or more of these may be used, depending on the stage of cancer when detected. 

5. Salivary Gland Cancer

Salivary cancer occurs when cancerous cells form in the tissues of salivary glands. There are two types of salivary glands: 

Major salivary glands: Found on the cheek, chin and under the tongue.

Minor salivary glands: Found throughout the oral cavity, including the palate and upper digestive tract. 

Signs and symptoms of salivary gland cancer may include: 

  • Difficulty swallowing

  • Facial numbness/weakness

  • Fluid draining from the ear(s)

  • Lump in the cheek, jaw, lip or inside of the mouth

  • Ongoing face pain 

Risk factors for salivary gland cancer include exposure to radiation in the head and neck area, advanced age, and family history of head and neck cancers. 

Nearly 90 percent of salivary gland tumors are benign, and the masses are generally removed before they become cancerous. Treatment of salivary gland cancer depends on the stage of cancer, but typically involves surgery and/or radiation. Chemotherapy is used less frequently in treating this kind of cancer.

Should I See My Doctor?

If you are experiencing symptoms of any of these rare cancers or have a symptom that persists for eight weeks or more, it is important to see your doctor for a thorough examination. 

They will ask you about any symptoms you are experiencing, your medical and family history, and may run additional tests (like blood work or imaging) before determining your diagnosis. 

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