UPDATED June 2015
1. GENERAL INFORMATION
1.1 What is the disease and how is it treated?
What are nasal cavity and paranasal sinus cancers?
The nasal cavity is the space nostrils open into. It runs back from the nostrils, above the roof of the mouth and curves down to connect with the mouth at the back of the throat. Cancer can develop in this space.
Paranasal sinuses are small air-filled spaces within the bones of the face, above and behind the nose, and behind the cheekbones. There are several pairs of sinuses and cancer can develop in any of them.
What are the causes of nasal cavity and paranasal sinus cancers?
Although the exact causes of these cancers are unknown, it is well established that some jobs (i.e., those exposing workers to wood or leather dust), smoking and snuffing tobacco can increase the risk of getting a sinonasal cancer.
Are they frequent types of cancer?
No, they are not. Cancers of the nasal cavities and sinuses are very rare: about 1 case every 100,000 people. Their occurrence is higher (about double) in males than in females, and higher in Eastern Europe than in the UK and Ireland.
How are they treated?
These cancers can be treated with surgery, radiotherapy, and chemotherapy, used alone or in combination. The treatment is chosen depending on the type of cancer, its stage (whether it has spread or not), its grade (how the cells look like), and the patient’s general health.
2. NASAL CAVITY AND PARANASAL SINUS CANCERS: WHAT ARE THEY, HOW DO THEY OCCUR, HOW ARE THEY DIAGNOSED?
2.1 What is sinonasal cancer?
Nasal cavities and paranasal sinuses are lined by several layers of tissue, each of them containing many different types of cells. The type of cancer is determined by the type of cells which grow out of control and start to multiply abnormally.
The most common type (more than 6 out of 10 nasal cavity and paranasal sinus cancers) is squamous cell carcinoma (SCC), a type of cancer (“carcinoma” means cancer) that starts in squamous cells. Squamous cells are the cells that line the walls of the mouth, nose, larynx, and throat.
About 1 out of 10 people diagnosed with a cancer in the nose and paranasal sinuses have an adenocarcinoma. This cancer develops in the gland cells that produce mucus in the nose. Another kind of gland cell cancer is adenoid cystic cancer: it normally develops in the salivary glands, but in very rare cases it can grow in the nose or nasal sinuses.
Other types of nasal cavity and paranasal sinus cancers are: lymphoma (starting in lymph nodes), plasmacytoma (developing in plasma cells), melanoma (developing from the pigment producing cells that give the skin its colour), olfactory neuroblastoma and neuroendocrine carcinomas (very rare tumours of the nasal cavity), sarcoma (starting in the cells of soft tissues).
2.1.1 Disease stats
The following figures refer to tumours diagnosed in Europe in 2000-2007.
Frequency. These are very rare cancers: each year, about 1 person out of 100,000 is diagnosed with a sinonasal cancer (incidence). The average age at onset is between 50 and 60 years.
Gender. It occurs twice as frequently in males as in females.
Geographical distribution. The occurrence of the disease is higher in Eastern Europe and lower in the UK and Ireland.
Prevalence. In Europe, about 14,500 people are living with a diagnosis of an epithelial tumour of the nasal cavities and/or paranasal sinuses (prevalence). This figure includes people who are considered cured, under treatment, or in clinical follow-up (15 years or more after diagnosis). Cured patients are estimated to comprise about 22% of the total, while patients in treatment or in clinical follow-up represent 21%.
Survival. Among European adults diagnosed during 2000-2007, 47 out of 100 were still alive 5 years after diagnosis (47%). This percentage varies with age: among people who were younger than 65 years at the moment of diagnosis, 51 out of 100 (51%) were alive after 5 years; patients still alive after 5 years were 42 out of 100 (42%) if their age at diagnosis was over 65.
2.2 Risk factors
Some people are at higher risk than others of developing certain diseases. This can be due to different risk factors.
In the genesis of nasal cavity and nasal sinus cancers, the role of several risk factors has been established.
According to the International Agency for Research on Cancer (IARC) and to a number of epidemiological studies, working in some jobs increases the risk of developing cancers in the nasal cavity and paranasal sinuses. The reason is that workers can be exposed to harmful chemicals. The agents for which there is evidence and which contribute to carcinogenesis are:
- wood dust (carpentry, any wood-related industry) for intestinal-type adenocarcinoma;
- leather dust (shoe making) for intestinal-type adenocarcinoma;
- nickel compounds (stainless steel production);
- isopropyl alcohol (manufacture using strong acids);
- chromium compounds (stainless steel, textiles, plastics, leather production; chromium use is now restricted in Europe);
- formaldehyde (an industrial chemical used to make other chemicals, building materials, household products);
- cloth fibres (textile manufacturing).
Studies published by IARC confirm that smoking increases the risk of nasal cavity cancer. This is because cigarettes contain nitrosamines and other chemicals that cause cancer. Before reaching the lungs, these substances pass through the nasal cavities.
There is increasing evidence that the Human papillomavirus (HPV) is associated with a subset of sinonasal carcinomas (i.e., non-keratinizing carcinoma). HPV has been detected in about 30% of sinonasal carcinomas; among the different types of HPV, type 16 is the most frequent. However, its role as an aetiological factor has not been recognised yet.
HPV-positive cancers have been detected more in the nasal cavities than in the paranasal sinuses.
The identification of HPV in sinonasal carcinomas has important clinical implications, because the presence of HPV could be a prognostic factor associated with a favourable outcome.
Prospective trials are needed to better evaluate the pathogenic role of HPV and draw conclusions with regards to the prognostic role of HPV.
2.3 What are the symptoms of nasal cavity and paranasal sinus cancers?
The type of sinuses affected may determine the symptoms experienced by patients. The most common symptoms are:
- blocked sinuses that do not clear;
- facial pain (behind the nose, upper teeth);
- swelling around the eyes.
But also: numbness (of the cheek, upper lip, upper teeth, or side of the nose); persistent nosebleeds; headaches; speech changes; double vision.
The presence of these symptoms is not necessarily linked to the presence of a paranasal sinus cancer, since every symptom described could be due to conditions other than cancer. But, like most cancers, sinonasal cancers are best treated when diagnosed at an early stage. For this reason it is important to report to the GP any symptoms that do not improve over a few days, especially if unilateral and persistent.
2.5.1 How is the disease identified as a sinonasal cancer?
In the presence of suspicious symptoms that do not improve with medical treatment, the first step is nasal endoscopy. Nasal endoscopy makes it possible to view the lesion and may help in differentiating an inflammatory polyp from a benign or malignant neoplasm. Depending on the images obtained, after endoscopy a sample (biopsy) may be taken to ascertain the kind of lesion. Biopsy and histopathological examination are essential for a correct diagnosis.
Other exams, such as CT and MRI, may be prescribed to strengthen the diagnosis.
- Endoscopy: a fine tube with a light at the end is inserted into the nose; it allows doctors to look at the sinus area and check if abnormalities are present.
- Biopsy: it is used to take a sample of tissue from an area that appeared abnormal during endoscopy; it requires the use of local or general anaesthesia.
- CT (computerized tomography): this radiographic technique uses a computer to photograph internal parts of the body.
- MRI (magnetic resonance imaging): magnetic waves detect images of the internal organs of the body.
2.5.2 Do these tests involve any risks for patients?
Generally, all of these exams are well tolerated. Biopsy may require the use of a local or general anaesthetic. CT and MRI scans may require the injection of a dye (or contrast medium) in order to obtain clearer images. Use of anaesthetics and contrast media could give rise to side effects.
3. HOW ARE NASAL CAVITY AND PARANASAL SINUS CANCERS TREATED?
3.1 General information
Since nasal cavity and paranasal sinus cancers can start in different types of cells (see 2.1), the type of treatment depends on several factors: the position and stage of the cancer, the type of cancer, its grade, and the patient’s general health. Several treatments may be used alone or in combination with others.
Radiotherapy may be used with two purposes:
- on its own, to treat a paranasal sinus cancer (radical radiotherapy);
- after surgery, to reduce the chance of the cancer coming back (adjuvant radiotherapy).
3.2.1 When is radiotherapy employed?
When treating a disease in its early stage, surgery is generally the first choice. In some cases, postoperative radiotherapy is indicated.
Surgery and postoperative radiotherapy (with or without chemotherapy) is the usual approach in the treatment of more advanced but still resectable tumours.
When dealing with unresectable paranasal sinus cancer, radical radiotherapy or radiochemotherapy (CRT) are the usual treatment approach.
3.2.2 Side effects
Nausea, vomiting, eye irritations (conjunctivitis), dry eyes, and headaches are possible side effects of radiotherapy. Generally, they start to disappear a couple of weeks after treatment interruption.
Where treatment beams enter and leave the body, hair loss occurs. Generally, hair starts to grow again after the end of treatment, but in some cases hair loss may be permanent.
For this kind of tumour, chemotherapy is employed with two purposes:
- curative, as part of a multimodal treatment, together with surgery and radiotherapy;
- palliative, as single therapeutic modality.
The optimal treatment strategy for patients with sinonasal malignancies is currently defined by a multidisciplinary team, based on a careful assessment of the patient’s profile (comorbidities and impact on the feasibility of treatment alternatives) and adequate evaluation of the tumour (histology, local extension, involvement of critical structures, possible regional and distant spread). Patient willingness is another key factor to be taken into account.
Surgery may be used depending on the position of the cancer and whether or not it has spread into the surrounding area and lymph nodes. Surgery can sometimes be quite extensive, and in some cases skin grafts or flaps are needed.
3.4.1 Side effects
The rate and type of postsurgery complications are strictly related not only to the expertise of the surgical team, but also to the extent of the disease and, therefore, to the type of procedure performed, endoscopic resection being generally less risky than craniectomy.
4. STAGES AND GRADES OF NASAL CAVITY AND PARANASAL SINUS CANCER
4.1 Staging of sinonasal cancer
The stage of a tumour indicates its extension and its spread beyond its site of origin (metastasis). It is important in order to choose the most appropriate treatment. The stage of a tumour may be referred to in several ways. This is because there are several staging systems.
4.1.1 TNM staging
This is the most commonly used system, where:
- T refers to the size or position of the primary tumour (where the cancer first starts in the body);
- N refers to which lymph nodes are affected, if any;
- M refers to metastatic disease (when the cancer has spread to other parts of the body).
4.1.2 Number staging
In this system staging is identified with numbers, from 1 (small cancer that has not spread) to 4 (advanced cancer with metastases).
In order to give more detailed information about cancer size and spread, number stages may be followed by lowercase letters.
4.1.3 Other terms
Other terms are normally used, such as:
- “early” or “local”: a cancer that has not spread;
- “locally advanced”: a cancer that has begun to spread into surrounding tissues or nearby lymph nodes;
- “local recurrence”: the cancer has come back in the same area after treatment;
- “secondary”, “advanced”, “widespread”, “metastatic”: the cancer has spread to other parts of the body.
4.2 Grading of sinonasal cancer
The speed at which cancer may progress is related to the appearance of cancer cells under the microscope. These morphologic characteristics are defined by “grades”.
A low-grade cancer is made of cells looking very like normal cells, growing slowly and less likely to spread. Highly abnormal cells, which tend to grow quickly and spread, are typical of high-grade tumours.
5.1 General information
Prognosis indicates the likelihood that treatment will be successful. It is a statistical measure obtained from different studies that observe the progress of the disease in a high number of patients. It is important to remember that these statistics are averages: no doctor is able to predict exactly what the outcome of treatment in an individual patient will be, nor how long the patient will live, as prognosis depends on several factors, in relation to the individual patient.
5.2 The prognosis of sinonasal cancers
The prognosis of sinonasal cancers is evaluated according to various factors; T classification, histology, localisation, and treatment are independent prognostic factors for survival.
Taking into account TNM staging, the number of patients alive at 5 years after diagnosis varies from 91 out of 100 for T1 to 49/100 for T4 tumours.
When considering the histological type of cancer, 5-year survival is 78% for patient with adenocarcinoma, 60% for those with undifferentiated carcinoma.
Finally, for tumours of the nasal cavity, maxillary sinus, and ethmoid sinus, survival at 5 years after diagnosis is 77%, 62%, and 48%, respectively.
6. WHAT TO DO AFTER TREATMENT
6.1 General information
After completing treatment, the oncologist will plan a series of subsequent visits and additional tests to monitor the effects of treatment and make sure that cancer does not recur. It is important for patients to contact their oncologist immediately in case of new symptoms or side effects, even beyond the already scheduled appointments that are part of the planned follow-up care schedule.
6.2 The follow-up of sinonasal cancer
Patients treated for sinonasal malignancies must be monitored for several years, at least 5, with a follow-up including clinical and imaging assessments. Patients should inform their doctor if they encounter any problems or notice any new symptoms.
7. WHAT TO ASK DOCTORS
Here is a list of questions that you might wish to ask your doctor. As the disease is rare, it is not widely known even within the medical community, so your family doctor might be unsure about the answers to some of these questions and you might want to ask them when you consult a specialist.
- What should I do if the pain increases, or if it appears in other parts of the body?
- May I phone you? What is the best time? If you are not available, may I ask for other specialists? Whom specifically?
- What leaflets, books, or websites could I read to learn more about nasal cavity and paranasal sinus cancer?
- Is there a patient association I could contact?
- Do you have special advice regarding nutrition?
- Do nasal cavity and paranasal sinus cancers run in families?
- Are my children at risk of getting a head and neck cancer?
- Where can I get help dealing with my feelings?
- Can you refer me for counselling?
Diagnosis and exams
- What tests are you going to do?
- What are you looking for?
- Is biopsy painful? Is it performed under general or local anaesthesia?
- What should I do in preparation for a CT scan or MRI?
- How long will the exam take?
- Is it painful?
- Will I be asleep?
- Are CT or MRI dangerous because of radiation exposure?
- How long will it take to confirm diagnosis?
- Should I refer to a second opinion for the evaluation of the histological specimen?
- Which is the Center with the highest expertise in my Country to treat these disease?
- What type of treatment do I need?
- Is there any choice of treatments?
- What are the risks and benefits of the treatments?
- There are any side effect?
- How can I help to reduce the side effects?
- What signs should I recognize so that I can tell the doctor and ask if the approach needs to be changed?
- Is surgery performed under general or local anaesthesia? Will it be painful? What should I do before and after the surgery?
- What are the possible side effects of chemotherapy? Will I lose my hair? Will I have nausea and vomiting?
- Will I have fertility problems because of the disease or treatment?
- Will treatment reduce my pain?
- During treatment should I take special precautions or change my habits?
- Is there any behaviour I could adopt to improve my prognosis?
- Which could be the late effects of the treatments?
- Which supportive therapy is suggested during the treatment?
- How will my treatment affect me?
- Will I ever get back to normal, or will I have some long term effects?
- How will my speech be affected?
- How will I communicate with people after my operation?
- How will my breathing be affected?
- Will my hearing be affected?
- How can I get help with hearing loss?
- Will my sight be affected?
- How will I look after my operation?
- What can be done to hide any scars I may have?
- Will my treatment affect my sex life?
- If I have difficulty eating, who can I go to for help?
- Will the treatment affect my sense of smell?
- Will my sense of smell improve over time?
- Will I need a special diet?
- Is there anything I should not eat?
- Will I be able to go back to work?