Nasal cancer

Definition

Nasal cancer is any cancer that occurs within the nose, either in the nasal vestibule (the immediate interior of the nose, just beyond the nostrils), or the nasal cavity (the deep interior of the nose). Many different types of cancer can occur within the nose, and the type of treatment and the chance of cure will vary according the type of cancer that occurs.

Description

Nasal cancers are very rare, making up less than 2% of all tumors of the respiratory tract in the United States. Less than 50 cases a year are diagnosed in the United States. Although squamous cell carcinoma is the most common type of cancer that occurs within the nose, many other types can also occur, including adenocarcinoma , melanoma , different kinds of sarcomas , inverted papilloma, lymphoma , and esthesioneuroblastoma.

Squamous cell carcinomas arise from skin tissue. They are the most common type and are often the result of either cigarette smoking or occupational exposure to dusts or chemical fumes. Adenocarcinomas are malignancies that resemble glandular tissue. Nasal adenocarcinomas are also often associated with occupational exposure to dusts or chemical fumes. T-cell lymphomas (Non-Hodgkins) in the nasal area are strongly associated with a virus (Epstein-Barr virus , EBV). Although nasal T-cell lymphomas are fairly common in some parts of the world, they are very rare in the United States.

Inverted papillomas are associated with another virus (human papilloma virus , HPV) and arise from benign but locally invasive nasal polyps. They are rare, comprising only about 0.5% of all nasal tumors. Although a definite association with HPV has been shown, a tumor may require interaction of the virus with chemicals or other factors, which appear to cause transformation of the inverted papilloma into squamous cell carcinoma in the nose. Esthesioneuroblastoma is a very rare nasal tumor, with less than 200 cases reported in the last 25 years. They are tumors that arise in the nerves in the nose, and have occurred most commonly in teenagers and senior citizens.

Demographics

Although the overall risk of nasal cancer is quite low (since this type of cancer is very rare), relative risks for some specific groups are fairly high. For example, nasal T-cell lymphomas are virus-associated and occur in high incidence in Asia and South America. Nasal squamous cell carcinomas occur much more frequently in cigarette smokers and individuals who have occupational exposures to dusts or chemical fumes, especially in Europe. Consumption of salted and pickled foods creates an increased relative risk of nasal cancer in Asia. Nasal cancers are also more frequent in some African populations that use mahogany wood in cooking fires.

In the United States, nasal cancers are rare. There are no significant racial differences in incidence. Males experience all types of nasal cancer in significantly greater numbers than women, probably due to more occupational exposure to agents that can cause these types of cancer. Most nasal cancers occur in people over 40, although the rare esthesioneuroblastoma has occurred in relatively high percentages in adolescents.

Causes and symptoms

All cancers are caused when a genetic mutation is made in a gene that is involved in the control of cell division. This mistake can arise naturally, can be inherited, or it can be caused by a virus, by sunlight or other radiation, or by some chemical that a person is exposed to, usually through eating, drinking or breathing. For nasal cancers, all of these factors have been shown to play a part.

The use of tobacco products has been strongly associated with the occurrence of nasal adenocarcinomas and squamous cell carcinomas. Chronic occupational exposures to leather, wool, or wood dust or chemical mixtures, particularly nickel, dioxane, nitrosamine, chromium used in dye manufacturing, mustard gas, rubbing alcohol, or formaldehyde, have a demonstrated association with nasal adenocarcinomas and squamous cell carcinomas as well. Some rare nasal T-cell lymphomas have been shown to be very strongly associated with a virus (Epstein-Barr Virus, EBV). Some nasal malignancies (about 5%) begin as inverted papillomas, a locally aggressive tumor which does not usually metastasize but which may turn malignant. These are also thought to be caused by a virus, although a different one: Human papilloma virus (HPV). Some nasal cancers have a strong hereditary component: people with genetic alterations that cause hereditary retinoblastoma have a much higher incidence of nasal cancers than average, which indicates that the genetic change that caused their original disease may also contribute to nasal cancer.

People with nasal cancer may think that they have a cold or chronic sinus infections. They may experience a feeling of stuffiness or blockage in the nose, persistent nasal drainage, or frequent nose bleeds. Other symptoms can occur if the tumor has invaded other tissues around the nose, particularly the orbit of the eye or the base of the skull. Other symptoms which may occur include:

• double vision
• bulging of the eye
• a lump on the face or around the eye
• loose teeth
• frequent headaches

In advanced stages, patients with nasal cancers may suffer from fatigue , weight loss , lack of appetite (anorexia ), and fever .

Diagnosis

When otherwise unexplainable symptoms lead a doctor to suspect that a patient may have nasal cancer, often he or she will arrange for endoscopic examination of the nasal cavity (and possibly the sinuses) in order to see if there is a tumor. Definite diagnosis requires a biopsy , in which a small piece of the tumor is cut out and examined to see what types of cells it contains. After a nasal cancer is diagnosed (depending on the type of cancer), many doctors will ask the patient to have an x ray , computed tomography scanning (CT scans), or magnetic resonance imaging (MRI). These techniques visualize the tumor and show the doctor how much the tumor has invaded surrounding tissues. Because treatment for nasal cancer, as well as paranasal sinus cancer , involves surgery in a small, complex space which requires the surgeon to set very precise surgical boundaries, and because most nasal cancers are advanced by the time a patient sees a doctor, it is very important that the doctor evaluate the tumor thoroughly before planning treatment. If the tumor appears to have invaded other tissues, often a doctor will schedule a surgical exploration of the tumor in order to better evaluate the cancer, with the goal of constructing the best possible treatment plan. Sometimes, in addition, surgical exploration is necessary to determine whether the position and invasion of the tumor into surrounding tissue makes surgical removal of the tumor impossible.

Treatment team

As the understanding of cancer grows and new treatment approaches are developed, the complexity of cancer treatment also increases. Today, a multidisciplinary approach to cancer treatment is considered necessary for effective patient care. People involved in the treatment of a nasal cancer will typically include the referring physician, an otolaryngologist, a medical oncologist, a pathologist, and a nurse. If radiation therapy is pursued, a radiation oncologist, radiation therapist, radiation nurse, radiation physicist, and a dosimetrist will also be involved. Treatment will also probably include a psychologist, nutritionist, social worker and chaplain. For nasal cancers, a reconstructive or plastic surgeon may be necessary for optimum cosmetic results after removal of a nasal tumor. If surgical removal of the eye is necessary, specialists in prosthetic eye replacement will be necessary as well.

Clinical staging, treatments, and prognosis

When a cancer develops, the original tumor can spread, usually through the blood or lymph system, to other parts of the body. Since the cancer spreads through the lymph system, often the lymph nodes in the area of the original tumor are the first other sites where cancerous cells can be found. Common other places that metastatic disease may appear are the lungs, the liver, and the bones.

One of the foremost goals of a doctor's assessment of a cancer patient is to determine how far the cancer has already spread and how likely it is to spread further, both of which are key factors in the likelihood that the patient will be cured. The assessment of the tumor's spread is termed staging, and the assessment of how aggressive the cancer cells are is termed grading.

Staging of nasal cancers is performed by visual inspection of tumors (maybe through endoscopy) or visualization of tumors by imaging techniques like x rays, MRIs, or CT scans. The doctor may also attempt to feel for tumors manually. This information will be used to create an official stage for the tumor that is a standardized expression of how much the tumor has already spread.

Because tumors of the nasal vestibule and cavity are rare, and because they are comprised of so many different types, no one staging system has been defined for use with these cancers. Cancers of the paranasal sinuses have a defined staging system based on the TNM system, and this system is often used for describing nasal cancers. The T in the TNM system represents the growth of the local tumor, N describes the spread of the tumor to the lymph nodes, and M describes the spread, or metastasis , of the cancer to distant body sites. The cancer is given various numbered ratings in each letter category, and these are used to create a standardized stage. Generally, tumors with no invasion of local tissues are described as Stage I, while tumors with minimal invasion of local tissues are identified as Stage II. Tumors that have extensive local invasion or that have spread to the lymph nodes but which have not metastasized are described as Stage III or early Stage IV (A and B). Stage IVC tumors are >any tumors which have metastasized.

Most nasal cancers (up to 80%) have already spread to other body sites by the time the symptoms prompt a patient to see their doctor. This fact, combined with the fact that the area is anatomically complex and tightly constructed, makes it very important that the first attempt at treatment is well-planned, with input from a multidisciplinary team and thorough evaluation of the cancer before treatment is begun.

Since cancers of the nasal cavity and vestibule include many different types of cancers, treatment will vary depending on the type of cancer involved, where it is located, and the extent to which it has already spread. Because of this, and because of the complexity of the anatomy in the area and the multitude of other important structures that may be involved in later stages, treatment of nasal cancers is highly individualized, with no firm standard practice guidelines.

For most nasal cancers, treatment will involve surgical removal of the tumor followed by four to five weeks of radiation therapy. In advanced cancers, preoperative radiation therapy may also be employed. However, since radiation therapy has proven very effective for nasal cancers and because radiation has better cosmetic results than surgical removal of a tumor, for many nasal cancers (especially T-cell lymphomas and esthesioneuroblastomas), radiation will be the initial treatment option. If the doctor decides to remove as much of the tumor as possible surgically, radiation therapy (external) will usually be used for four to five weeks after surgery in order to destroy any remaining cancerous tissue. One exception is the case of inverted papillomas, for which surgical excision alone is usually employed. Surgery, because of the tight anatomical area in which a surgeon must work, may also involve more recent techniques like cryosurgery (freezing tissue) or laser surgery.

Tumors initially treated by either radiation or surgery alone may, if they come back, be treated by the untried option or by employing both. External radiation may be supplemented, especially in advanced nasal vestibule cancer, by internal radioactive implants. In addition, advanced stage or recurrent nasal cancer may be treated by chemotherapy , usually involving a combination of drugs. Drugs are used in combination in most chemotherapy because combinations of different drugs (with different side effects) deliver the highest cancer-destroying effect, while minimizing the chance for a serious adverse reaction to the therapy. The drug combinations used in nasal cancer vary on the type of cancer, and may include one or all of the drugs cisplatin , fluorouracil , bleomycin , or methotrexate . In addition, nasal cancers described as Stage III or IV will probably be treated with preventative radiation therapy of the neck area, in order to destroy cancerous cells which may have traveled to the lymph nodes.

Although nasal cancers are made up of many different types of cancer, all types of nasal cancers are considered aggressive. The majority of nasal cancers, because symptoms mimic upper respiratory illnesses and because symptoms often do not occur until the cancer has already filled up the nasal cavity and has invaded surrounding tissues, are already in advanced stages when a patient seeks medical help. For this reason, and because treatment is difficult because of the complexity of the anatomical area, fewer than half of nasal cancer patients survive. If the first treatment attempt is successful, and a patient is cancer-free at two years, however, chances improve greatly.

Nasal cancer is unusual in that, although many patients have metastasis to the lymph nodes or beyond (usually to the lungs), metastasis is not usually the reason for a patient's death. Most nasal cancer patients who succumb to the disease die from invasion of the tumor into vital areas of the brain.

Alternative and complementary therapies

Alternative and complementary therapies are treatments that are not traditional, first-line therapies like surgery, chemotherapy and radiation. Complementary therapies are those that are meant to supplement traditional therapies and usually have the objective of relieving symptoms or helping cancer patients cope with the disease or traditional treatments. Alternative therapies are nontraditional treatments that are chosen instead of traditional treatments in an attempt to cure the disease. Alternative therapies have typically not been proven to be effective in the same way that traditional drugs are evaluated, in clinical trials .

Common complementary therapies that may be employed by patients with nasal cancer are art therapy, massage, meditation, visualization, music therapy, prayer, t'ai chi, and yoga or other forms of exercise, which reduce anxiety and can increase a patient's feeling of well-being.

Numerous alternative therapies exist in cancer treatment, but none has been proven in clinical trials to be effective. Laetril, a product of apricot seeds, is probably one of the most well known. Laetril contains a form of cyanide that may be released by tumor enzymes and may then act to kill cancerous cells. Laetril is not approved for use in the United States, although it is available in Mexico. The National Cancer Institute (NCI) sponsored two trials of Laetril in the late 1970s and early 1980s, but found Laetril to be ineffective and concluded that no further study of the substance was necessary. Vitamins and other nutritional elements like vitamins A, C, and E, and selenium are thought to act as antioxidants . Vitamin E, melatonin, aloe vera, and a compound called beta-1, 3-glucan are reported to stimulate the immune system. Natural substances like garlic, ginger, and shark cartilage are also commonly held to shrink tumors, with less defined modes of action.

Antineoplastons are believed by some to be another alternative approach to a cancer cure. Antineoplastons are small proteins which may act as molecular messengers and which may be absent from the urine and blood of many cancer patients. Proponents believe that replacing these proteins may have beneficial effects. The NCI has been unable to draw definitive conclusions about the usefulness of antineoplastons as a therapy because no large-scale clinical trials of the therapy have been completed.

Coping with cancer treatment

Treatment of nasal cancers commonly includes surgery, radiation therapy and chemotherapy. Although the use of chemotherapy and radiation therapy in addition to surgery has improved the chance of survival for nasal cancer patients, both of these treatments unavoidably result in damage to some healthy tissues and other undesirable side effects.

Fatigue is a very common side effect of both radiation therapy and chemotherapy. Side effects of the actual treatments combine with the natural depletion of the body's resources as it fights off the disease and normal psychological consequences of the disease such asdepression to make coping with fatigue a very significant aspect of dealing with cancer treatment. The best way to deal with these symptoms is to cut back on stressful activities and take plenty of time to allow the body to heal. It is also important to try to maintain a well-balanced, nutritious diet, and to exercise. Patients should avoid as much extra stress as possible and should limit visitors, if needed, to avoid being overtired. At the same time, it is also important for psychological health for the patient to pursue their interests as much as possible and to avoid becoming isolated.

The biggest problem for those undergoing radiation therapy is the development of dry, sore, "burned" skin in the area being treated. (Radiation does not hurt during treatment and does not make the person radioactive.) Skin in the treatment area will become red, get itchy and sore, and may blister and peel, becoming painful. Patients with fair skin or those who have undergone previous chemotherapy have a greater risk of more serious reactions. Dry, itchy or sore skin is temporary, but affected skin may be more sensitive to sun exposure for the rest of the patient's lifetime, so a good sunscreen and a hat should be used whenever affected skin is exposed to sunlight.

Other effects, specific to the nasal area, may also occur. Sometimes very thick mucus is produced that may be difficult to cough up. Some patients become hoarse and find it difficult to eat. It is important for patients to keep well-hydrated by drinking plenty of fluids and to eat as much protein as possible. If patients cannot eat enough to maintain a high-protein diet, liquid high-protein drinks should be consumed. Patients may be more susceptible to upper respiratory infections after treatment, so some physicians will prescribe preventative antibiotics . If eating is extremely painful, tylenol can be consumed in milk about thirty minutes before a meal for pain relief. Patients should be prepared for the fact that symptoms of radiation treatment can persist for up to a month after the last treatment.

Some of the more common side effects of chemotherapy include hair loss, and nausea and vomiting . Hair loss (alopecia ) is a difficult part of dealing with cancer treatment for most patients, especially women. Hair may thin out gradually, or it may fall out in big clumps. To slow down the rate of hair loss, avoid any unnecessary sources of damage to the hair, like curling, blow-drying, or chemical treatments.

Different patients choose different ways of coping with the loss of their hair. Some patients may find they are more comfortable hiding hair loss with a wig; it is a good idea to cut off a lock of hair before hair loss begins in case a wig is later desired. Some patients may choose to remain bald, or may want to choose hats or scarves instead of wigs. In any case, it is important to remember that the loss of hair is a sign that the medication is doing its job, and that hair loss is temporary. Hair usually begins regrowth within a few months of the end of intensive chemotherapy, although it may come in a different color or texture than the original hair.

Nausea and vomiting are other fairly common side effects of many chemotherapy drugs. (Radiation to the brain or the GI tract can also cause nausea and vomiting.) After a few courses of chemotherapy drugs, some patients will become nauseated just from thinking about an upcoming treatment or from smelling certain odors. Drugs that combat nausea and vomiting can be prescribed, but are often not effective for anticipatory nausea. If nausea and vomiting are a problem, heavy, regular meals should be avoided in favor of small, frequent snacks made up of light but nourishing foods like soup. Avoiding food smells and other strong odors may help.

Desensitization, hypnosis, guided imagery, and relaxation techniques may be used if nausea and vomiting are severe. These techniques help to identify the triggers for the nausea and vomiting, decrease patient anxiety, and distract the patient from thinking about getting sick. Acupressure bands, commonly used for seasickness, and acupuncture, may also provide some relief for some patients.

Both radiation therapy and chemotherapy treatments require a substantial level of commitment from the patient in terms of time and emotional energy. Fear and anxiety are major factors in coping with cancer in general and these cancer treatments. The feelings are completely normal. Some patients find that concentrating on restful, pleasurable activities like hobbies, prayer, or meditation is helpful in decreasing negative emotions. It is also very important that patients have people to whom they can express their fears and other negative emotions. Support groups may help to provide an environment where fears can be freely expressed and understood.

Clinical trials

Clinical trials are studies in which new treatments for disease are evaluated in human patients. Current clinical trials for nasal cancer patients are concentrating on the addition of chemotherapy to the more common treatments of surgery and radiation therapy, either before or after those treatments, in order to improve cure rates or to lessen the side effects of radiation.

Nasal cancer patients are also being recruited for a clinical trial evaluating an alternative therapy known as antineoplaston therapy.

Prevention

Although mutations in genetic material happen frequently, most of these do not result in cancer. This is because a healthy body repairs most mistakes before a cancer develops and because, if a cancer does develop, the immune system of a healthy body will usually destroy it. In general, therefore, a healthy lifestyle that includes exercise, plenty of sleep, a diet rich in fruits and vegetables, regular health screenings and the avoidance of stress, excessive sun exposure, tobacco use or excessive alcohol consumption will help to prevent most cancers.

Since nasal cancers, in particular, are often caused by chemical exposures, many of these cancers are preventable by avoiding excessive inhalation of wood dust or chemical mixtures and by avoiding use of all tobacco products. (Nasal cancers resulting from wood dust appear to require high-dose, long-term exposure, especially to hardwoods.)

One type of nasal cancer appears to be virus-associated and is more prevalent in people with a history of nasal polyps. People who are diagnosed with nasal polyps should discuss their removal with their physician and have existing polyps checked regularly in order to detect a malignant polyp as quickly as possible.

Patients with nasal cancer can increase their chances of a cure by making sure that they see their doctor for all scheduled follow-up appointments. This is especially important for the first two years (when most recurrences of nasal cancer occur), but it is also important to maintain follow-up beyond that. Many nasal cancer patients experience a second tumor somewhere else in the upper respiratory tract.

Special concerns

One of the unique aspects of dealing with nasal cancer is the fact that surgical removal of a nasal tumor can result in substantial facial disfigurement. Patients who are dealing with this aspect of nasal cancer are forced to cope with the substantial emotional burden of disfigurement in addition to the other emotional ramifications of their disease.

People with facial disfigurement may be forced to cope with negative reactions from other people in public places, including staring, whispering, rude remarks or averted eyes and other avoidance of interpersonal interaction.

In addition, the loss of the accustomed appearance will be experienced much like a bereavement. Patients will probably initially feel numb, then experience intense, overwhelming feelings of sadness, fear, and anger. The period characterized by intense, almost unbearable emotions is usually followed by a period of time when the patient feels completely empty, fatigued, and apathetic. Given time, most patients will come to an acceptance of their new reality and begin to enjoy old friends and activities again. It is important not to expect patients in such circumstances to immediately accept their situation or to suppress the natural emotions that accompany the change in their appearance. Patients can ease the process by trying to focus on one day at a time and by finding people who can help them work through the process by listening and accepting their emotions. It is very important that a patient dealing with these changes have friends or family members to whom they can express their feelings of grief and anger. A support group might also be helpful.

Resources

BOOKS

Buckman, R. What You Really Need to Know About Cancer. Baltimore: Johns Hopkins University Press, 1999.

Cook, Allan R., ed. The New Cancer Sourcebook. New York:Omnigraphics, Inc., 1996.

ORGANIZATIONS

American Cancer Society, 1599 Clifton Road, NE, Atlanta, GA 30329-4251. (800)586-4872 <http://www.cancer.org>.

National Cancer Institute, 9000 Rockville Pike, Bethesda, Mary land, 20892. (800)422-6237. <http:www.nci.nih.gov>.

The Wellness Community, 10921 Reed Harman Highway, Cincinnati, Ohio, 45242 (888)793-9355. <http://www.wellness-community.org>.

See Also Cryotherapy; Tumor grading; Tumor staging

Wendy Wippel, M.Sc.

KEY TERMS

Carcinoma

—Any malignant tumor.

Endoscopy

—A diagnostic procedure in which a miniature videocamera on the end of a flexible tube is inserted into internal body cavities so that the physician can view the internal structures.

Lymphoma

—A type of cancer that arises in the lymph nodes.

Nasal polyp

—A non-cancerous mass that grows out from the inner lining of the nasal cavity.

Sarcoma

—A tumor that arises from bone or connective tissue.

Squamous cell carcinoma

—A malignancy that arises from outer skin cells.

QUESTIONS TO ASK THE DOCTOR

• Can you explain what kind of cancer I have?
• Can you explain the grade and stage of my cancer? What are the chances that it will come back?
• How was this cancer diagnosed?
• What is my prognosis?
• How much will the surgery alter my facial appearance?
• What treatments are we going to pursue? What happens if these don't work?
• Do you have experience in treating this type of cancer?
• Is there anything I can do to optimize treatment? Are there any particular side effects I should expect?
• Are there complementary therapies that you would recommend? Any other things that would help me cope with the diagnosis or treatment?
• How often will I need further check-ups? Is there anything I can do to keep this cancer from coming back?