Cancer of the nasal cavity and paranasal sinus
What is cancer of the nasal cavity and paranasal sinus?
Cancer of the nasal cavity and paranasal sinus is a disease in which cancer (malignant cells) is found in the tissues of the nasal cavity or paranasal sinuses. The paranasal sinuses are small cavities surrounding the nasal cavity. These cavities are lined by cells, which produce mucus that prevents the nasal cavity from drying out. The mucus also assists in removing harmful agents from the nasal cavity. The sinuses are also spaces through which the voice can echo to make sounds when a person talks or sings. The nasal cavity is a passageway through which air passes on way to the lungs during breathing. The nasal cavity functions as an “air-conditioner” heating and moisturizing inhaled air. There are several paired paranasal sinuses including the maxillary sinuses in the upper jaw, the frontal sinuses above the nose in the lower part of the forehead, the ethmoid sinuses between the nasal cavity and the orbit and the sphenoid sinus behind the nasopharynx area in the center of the lower part of the skull. Cancer of the paranasal sinuses usually begins in the mucus producing cells mentioned above. Rarely the tumor begins in the color producing cells of the nasal lining called melanocytes, and the tumor is called a melanoma. Tumors beginning in a muscle or in connective tissue is called a sarcoma. A slowly growing malignant tumor is called an inverting papilloma. Midline granuloma is another malignant tumor causing normal tissues to break down.
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Symptoms and findings indicating a cancer of the nasal cavity and paranasal sinus
- blocked nose for more than 4 weeks
- recurrent nose-bleeds
- an infection persisting more than 4 weeks after adequate treatment
- problems with dentures that do not fit in the upper jaw
- a lump or sore in the nose that does not heal
- pain in the upper teeth that persists after adequate dental treatment
- frequent headaches or persistent pain in the upper jaw
- swelling around the eyes or other eye problems persisting after treatment
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Diagnosis of cancer of the nasal cavity and paranasal sinus
The physician will take a complete history and then examine the nose using a mirror or by using ridged or flexible endoscopes (nasoscope or rhinoscope) usually after administering a topical anesthesia. If something abnormal is found the doctor will take a biopsy (piece of tissue for examination under a microscope). A CT scan will be ordered (a specialized x-ray using a computer) and often the CT scan will be carried out both without and with an intravenous injection of contrast. If the referring doctor has noted abnormal findings a CT scan of the neck will also be carried out in order to exclude metastasis. In some cases the CT scan is followed-up with an MRI (a x-ray like procedure using magnetic energy). In some cases a small operation has to be carried out in order to get a tissue sample from the sinuses.
The chance of recovery depends on where the cancer is located in the sinuses, whether the cancer has spread to other tissues and the patient’s general condition. In general the prognosis is better the smaller the tumor is and the lower in the mid-face the tumor is located.
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Staging of cancer of the nasal cavity and paranasal sinus
Once cancer is found more tests are needed to find out if the tumor has spread to other tissues. This is called staging and is necessary in order to plan the treatment and for estimating the prognosis. The following is used for the maxillary and ethmoid sinuses. The European Organization for Research and Treatment of Cancer (EORTC) and the American Joint Committee on Cancer (AJCC) accepts this classification.
Maxillary sinus
Stage I
The tumor is located in the mucosa (lining of the sinus) and has not caused bone destruction. No spread to lymph nodes or other tissues.
Stage II
The tumor has started to destroy bone but is located to the floor or front wall of the sinus. No spread to lymph nodes or other tissues.
Stage III
The tumor invades one of the following structures: Bone of the back wall of the sinus, subcutaneous tissue, skin of the cheek, the floor of the eye socket or it’s wall towards the nose, tissues behind the sinus or to the sphenoid sinus. The cancer may have spread to one lymph node (size under 3 cm) on the same side of the neck as the cancer.
Stage IV
The cancer grows into the eye socket or into the frontal sinus, sphenoid sinus, the nasopharynx or into the area under the frontal lobes of the brain. The tumor may have spread more than one lymph node on the same side of the neck or to lymph nodes on both sides of the neck or to any lymph node that measures more than 6 cm (2 inches) or to other parts of the body
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Ethmoid sinuses
Stage I
The tumor is located in the ethmoid sinus and can have caused bone destruction. No spread to lymph nodes or other tissues.
Stage II
The cancer has spread to the nasal cavity. No spread to lymph nodes or other tissues.
Stage III
Invasion of eye socket or maxillary sinus. The tumor may have spread to a lymph node less than 3 cm (1 inch = 2.5 cm) in size.
Stage IV
Invasion into the base of the skull or into the eye socket including the area surrounding the optical nerve, or into the sphenoid or frontal sinus or the skin of the nose. The tumor may have spread to a large lymph node, several small nodes or to other tissues of the body.
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Treatment
Overview
There are four treatment modalities: Radiotherapy, surgery, chemotherapy and photodynamic therapy
Radiotherapy
Radiotherapy uses high energy x-rays to kill cancer cells. External beam radiation is given once or twice every weekday for 5 to 6 weeks. A special treatment plan is made for each patient. Cancer cells are more sensitive to radiation than normal cells. The dose is adjusted to kill cancer cells and to let normal cells survive. Normal tissues will however be affected by the treatment and will cause side effects of the treatment such as dryness of the mouth, pain, swelling of the throat etc. These side effects are usually temporary but may sometimes persist especially if the tumor is large or if the tumor has spread to the lymph nodes of the neck. The thyroid gland can be damaged after treatment of the neck and may require life long substitution therapy (one tablet per day). The neck area is often included into the treatment area to reduce the risk of neck metastasis. Thyroid stimulating hormone levels need to be followed during several years after radiation treatment of the neck.
Surgery
Surgery is commonly used as a primary treatment. One needs to remove normal tissue surrounding the cancer (radical surgery). If the hard palate must be removed is has to be replaced by a prosthesis or preferably by transplanted tissue often a part of the hipbone with muscle usually vascularized by microvascular anastomosis (the tissue gets blood by connecting vessels of the graft to other vessels in the face or neck). Immediate reconstruction after removal of the tumor is often the optimal treatment but usually requires three surgical teams each with two surgeons; head and neck surgeons, plastic surgeons and maxillo-facial surgeons. Sometimes one has to remove an eye. The eye is usually replaced with a prosthesis. Surgery is often followed by radiation therapy. If the cancer has spread to the neck one usually carries out a “neck dissection” (removal of lymph nodes in the neck).
Chemotherapy
The tumor is treated by administering drugs either as pills or as intravenous injections. Chemotherapy is a systemic treatment and kills cancer cells throughout the body. It is used to shrink tumors, sometimes in combination with concurrent radiation especially is a tumor is very large. Chemotherapy is rarely used as a single therapy except in cases where surgery or radiation is not useful or possible.
Photodynamic therapy
A drug is injected intravenously. One can sometimes feel a burning sensation locally which rapidly disappears. The patient will have to wait 2 to 6 days before treatment while the drug is excreted from most tissues but retained in tumors and a few other tissues such as skin. The point of the waiting is to achieve an optimal ratio of the drug concentration between tumor and normal surrounding tissue. After the waiting period the tumor is exposed to red laser light with a wavelength adjusted to induce an energy transfer to normal triplet oxygen to convert oxygen to the highly cell toxic singlet stage. This will kill cancer cells. This treatment spares collagen fibers (the skeleton of soft tissues) which explains the excellent healing after this therapy. This therapy can be used for small tumors in patients with a recurrence after radiation when surgery is not possible due to poor general health. The only side effect is a hypersensitivity to light for 3 to 13 weeks depending on the specific drug. Some patients have returned to work as soon as 2 weeks after treatment. A simple light-meter is issued to each patient with instructions to increase the light dose
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How is the optimal treatment chosen?
Cancer of the maxillary sinus
As stated above several methods are available. If surgery is possible – this would be our first line of treatment. Surgery is almost always followed by radiotherapy. Chemotherapy is used, often concomitant with radiation therapy, if the tumor is unusually large. Surgery is then carried out to remove any residual tumor. Concomitant chemo-radiotherapy is not attempted if a patient is very fragile. Surgery or radiation therapy might be easier to tolerate if the general health is poor. Lymph nodes in the neck are treated with radiation therapy or surgery (a neck dissection). If small nodes disappear after radiation therapy we would usually not follow- up with surgery. Larger nodes or nodes that do not completely disappear after radiation therapy will be surgically removed.
Cancer of the nasal cavity and ethmoid sinuses
Stage I tumors are treated with surgery and radiation therapy is also carried out if surgical margins are inadequate.
Stage II-IV tumors are always treated with radiation after surgical resection. Larger tumors are treated as stated above and lymph nodes in the neck are also treated as already described above.
Adenocarcinoma of the nasal cavity and paranasal sinuses
These tumors are first irradiated and if the cancer cells are undifferentiated a very aggressive surgery is carried out even if the procedure will cause serious life long side effects. The specific procedure is of course discussed in detail with the patient before surgery.
Malignant melanoma
Almost always treated with surgery first and sometimes later followed-up with a specialized radiation therapy (high doses during a shorter period of time – as compared to regular radiation therapy protocols).
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What happens after treatment?
Management of surgical defects
Prostheses
In many cases half of the hard palate needs to be removed. Rarely, one needs to remove the entire hard palate. In both cases there will be a communication between the oral cavity and the nasal cavity. This affects speech and swallowing (food may come out from the nose). Immediately after the surgical resection our patients are fitted with a temporary prosthesis. This prosthesis is made using a preoperative impression of the upper jaw. If the patient is edentulous the prosthesis will be fasted with screws directly into the remaining half of the upper jaw. A prosthesis is often made with a locking device that permits the physician to remove the part corresponding to the surgical defect in order to permit inspection and treatment of the cavity. After 3-6 months the temporary prosthesis is removed and substituted with a “permanent” prosthesis. This is a denture with a superstructure that is pressed into the surgical cavity. Prostheses needs frequent adjustments in order to function adequately. We try to avoid the use of these devices by carrying out a primary repair of the defect.
Secondary reconstruction
If for some reason a primary surgical reconstruction has not been carried out immediately after the tumor resection it can be done at a later time. In our experience many patients, especially older patients, are not interested of more surgery if the first procedure is successful in removing the cancer. This is the reason for our strong recommendation that a surgical reconstruction be carried out at the time of primary surgery. The additional cost for an immediate reconstructive procedure may well be offset by a reduction in need for management of post-surgical prosthetic devices.
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Management of non-surgical side effects
Dry mouth
Radiation therapy may cause a life long problem with dryness of the mouth. All salivary glands are damaged by radiation. Patients need to use an artificial substitute for saliva, use drinking water or both. The first few months post therapy are worst because the radiation also causes an inflammation of the normal lining of the mouth (the mucosa). With time the side effects tend to subside.
Prognosis
The survival rate for cancer of the paranasal sinus is 24 % at 2 years and 0 % at 5 years at our institution. This includes deaths from other diseases and old age. In total 37 % of all patients, regardless of stage, became free from tumor after treatment.
Cancer of the nasal cavity has a better prognosis. The 2-year survival rate is 63 % and the 5-year rate is 30 %. 72 % of all patients with cancer of the nasal cavity became free from cancer.
Treatment of pain
Pain can be treated effectively in all stages and after all procedures. Patients rarely need treatment for pain once a treatment is completed. The treatment ranges from acetaminophen (Tylenol) and topical anesthesia to intravenously or subcutaneously injected morphine.
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