Nasal cavity and paranasal sinus cancers

ALSO KNOWN AS: Nose cancers, sinus cancers

RELATED CONDITIONS: Lymphoma, melanoma, hemangiopericytoma, osteosarcoma, chondrosarcoma, adenosarcoma, squamous cell carcinoma

DEFINITION: Nasal cavity and paranasal sinus cancers arise in the paranasal sinuses or the nose. Many types of cancer can originate in the paranasal sinuses and nose. These include squamous cell carcinoma, adenocarcinoma, adenoid cystic carcinoma, lymphomas, chondrosarcoma, osteosarcoma, hemangiopericytoma, melanoma, and esthesioneuroblastoma, as well as metastatic lesions from cancers of the kidney, lung, and breast.

Squamous cell carcinomas arise from the sinuses' epithelial (skin) cells and are the most common type of paranasal sinus tumor. Adenocarcinoma usually arises in the mucus-producing glands of the upper nasal cavity. It is most common in woodworkers and people working in the shoe and leather industries. Adenoid cystic carcinomas arise out of the salivary gland tissue and tend to migrate to nearby nerve tissue. These tumors are slow-growing but often metastasize to distant organs. Lymphomas arise from the cells of the lymph nodes and exhibit ulceration and necrosis (tissue death) of the lymph tissue in the nasal cavity or paranasal sinuses. Chondrosarcomas are rare in the nose and sinuses and arise from connective tissue. Osteosarcomas is a cancer of the facial bones. Hemangiopericytomas are tumors of blood vessels. They are rare in the nose and sinuses. Malignant melanomas arise out of the epithelial tissue of the nasal septum and the lateral nasal wall and metastasize early. Esthesioneuroblastomas arise out of the sensory epithelial cells that control olfaction (smelling) and are quite rare.

Risk factors: The primary risk factor for nasal and paranasal sinus cancer is occupational exposure to inhaled toxic substances, including glue, formaldehyde, solvents, mustard gas, isopropyl alcohol, and radium. Dust from wood, flour, textiles, leather, chromium, and nickel also contribute to these cancers. Heavy air pollution and smoking also contribute to nasal and paranasal sinus cancers.

Mutations (genetic changes) in the TP53 (also known as p53) tumor-suppressor gene and the NUTM1 gene contribute to the development of many head and neck cancers, including nasal and paranasal sinus cancers. The human papillomavirus (HPV) likely contributes to some nasal cancers, and a family history of retinoblastoma increases an individual's risk.

Etiology and the disease process: Nasal cavity and paranasal sinus cancers develop in the nose or the six sinuses. Each side of the face has a maxillary, ethmoid, and sphenoid sinus. The paranasal sinuses are spaces in the nasal and facial bones. When a single cell mutates and grows uncontrollably, the tumor, usually squamous cell cancer, may invade nearby structures, causing symptoms of this type of cancer. Most of these occur in the nasal cavity or maxillary sinuses, sometimes in the ethmoid sinuses. Rarely, cancer may occur in the frontal or sphenoid sinuses.

Incidence: Cancers of the nasal and paranasal sinuses are rare, making up 3 to 5 percent of head and neck cancers. Around 80 percent of diagnoses are in individuals over fifty-five. Men are nearly twice as likely to develop nasal or paranasal cancer than women, and White Americans are at an increased risk compared to Black Americans.

Symptoms: The symptoms of nasal and paranasal sinus cancers are much like those of chronic sinus disease. They include blocked sinuses, decreased sense of smell, frequent sinus headaches, purulent drainage from the nose, facial swelling, epistaxis (bleeding from the nose), double or blurred vision, and frequent infections. Some patients experience more definitive symptoms, such as a growth or mass on the nose, face, or soft palate; a lump inside the nose; numbness in areas of the face or head, especially the upper cheek; loosening, pain, or numbness of the teeth; continuous tearing of the eyes; trouble opening the mouth; and swelling of the eyes.

Screening and diagnosis: No routine screening is performed for nasal or paranasal sinus cancers because of their rarity. Methods of diagnosis include physical examination, nasal endoscopy, computed tomography (CT) scan of the nose and sinuses, or magnetic resonance imaging (MRI) of the sinuses and orbits. If there is a visible mass, it is biopsied (a slice of tissue is removed for microscopic examination) to determine whether it is cancer and, if so, what type.

Only esthesioneuroblastomas and cancers of the maxillary sinuses, nasal cavity, and ethmoid sinuses are staged. The sinus cancers are staged using the American Joint Committee on Cancer (AJCC) staging system, which uses the TNM (tumor/lymph node/metastasis) groupings. Staging is performed using the Kadish or UCLA systems for esthesioneuroblastomas.

Stages of cancers of the maxillary sinus:

• Stage 0 (T0, N0, M0): The cancer is confined to the epithelium and resembles normal tissue.
• Stage I (T1, N0, M0): The cancer is confined to the nasal mucosa and has not spread to other sinuses or invaded the bones of the nose.
• Stage II (T2, N0, M0): The cancer has invaded the bones of the maxillary sinus, excluding the posterior wall. These bones include the hard palate and the opening into the maxillary sinus. The cancer has not spread beyond the maxillary sinus.
• Stage III (T1–3, N0–1, M0): The cancer has invaded the posterior wall of the maxillary sinus or has grown through the other bones of the sinus into the skin, the eye socket, or the ethmoid sinus. It may have metastasized to a single lymph node on the same side as the tumor. The involved node is a maximum of 3 centimeters (cm) in width.
• Stage IV (T1–4, N2–3, M0–1): The cancer has spread to the eye, the skull, the nasopharynx, or the sphenoid and frontal sinuses. There may be lymph node involvement. For a tumor to be Stage IV, there must be more than one node involved, nodes of greater than 3 cm, or involvement of nodes on the side opposite the tumor. Any maxillary cancer that has metastasized to other organs is Stage IV.

Stages of nasal cavity and ethmoid cancers:

• Stage 0 (T0, N0, M0): The cancer is confined to the epithelium and is very early stage.
• Stage 1 (T1, N0, M0): The cancer is localized to either the nasal cavity or the ethmoid sinus and its bones. There is no presence of lymph node involvement or metastases.
• Stage II (T2, N0, M0): The cancer has invaded another cavity close to the tumor, but there is no lymph node involvement or metastases.
• Stage III (T1–3, N0–1, M0): Either the cancer has invaded other structures, such as the eye socket, the palate, or the maxillary sinus, and there is no lymph node involvement or metastases, or it has invaded those structures, and one lymph node is involved. This node is a maximum of 3 cm.
• Stage IV (any T, N2–3, M0–1): The cancer has invaded other structures, such as the eye, skull, or the sphenoid or frontal sinuses, or the cancer has spread to two or more lymph nodes, and these nodes are larger than 3 cm, or the cancer has spread to distant organs.

Treatment and therapy: Nasal cavity and paranasal cancers are treated with a combination of surgical resection, radiation therapy, and chemotherapy. The actual surgical procedure performed will depend on the location of the tumor, its stage, and whether it can be removed en bloc (as one piece of tissue). Cancer cells can be left in the surgical site if the tumor is incised (cut into).

Stage I and II cancers can be treated with computer-aided transnasal endoscopic surgery, which is performed using an endoscope that is inserted into the nostrils and then the sinuses. Other surgical procedures used for tumors that remain within the nasal cavity are sublabial (under the upper lip) or lateral rhinotomy (incision along one side of the nose) approaches. Surgical procedures for Stage III and IV tumors may include midfacial degloving (separating the skin, subcutaneous tissue, nerves, and tendons from the facial bones), orbital exenteration (removal of the eye and orbital bones), or craniofacial resections. After the latter surgical procedures, it may be necessary to perform grafts of skin or fascia (the fibrous connective tissue that separates body structures) and to insert dental, orbital, or other prostheses to reconstruct the face. Tumors that have metastasized to the brain, spinal column, and optic nerve and into the cavernous sinus (bilateral large venous blood vessels that drain blood from the dura mater that covers the brain) are generally considered inoperable.

Radiation therapy may be performed before or after surgery and applied internally or externally. Intensity-modulated radiation therapy (IMRT) is the most commonly used radiation therapy in nasal or paranasal cancers. Induction radiation, which is radiation therapy before surgery, can decrease the size of the tumor and simplify tumor resection. This may save the eye in individuals with advanced-stage cancers. After surgery, it destroys any remaining tumor cells.

Radiation therapy uses an external beam or radioactive objects placed in the nasal cavity, like seeds, wires, or catheters. A mask is created to position the head precisely for radiation administered by an external beam. Radiation beams must be aimed carefully to prevent radiation exposure to the thyroid and pituitary glands. Research indicates that proton therapy may be the superior method of radiation therapy. Proton beams allow medical professionals to deliver higher doses of radiation while causing fewer side effects and damaging less surrounding tissue compared to traditional X-rays. Varying radiation schedules, called accelerated fractionation, may allow doctors to better control cancer growth and decrease total treatment time. For example, rather than one large dose over two days, the dose may be more effective if given over six or seven days.

Nasal cavity and paranasal sinus cancers can be treated with chemotherapy either before or after surgery. Chemotherapy before surgery may decrease the size of the tumor to make surgical removal more manageable. Chemotherapy administered after surgery is intended to destroy any remaining cancer cells. Chemotherapy drugs may be given with radiation. Traditional chemotherapy drugs include cisplatin and 5-fluorouracil for paranasal sinus cancers. Vincristine (Oncovin), cyclophosphamide (Cytoxan), doxorubicin (Adriamycin), and cisplatin treat esthesioneuroblastomas. Epidermal growth factor receptor (EGFR) inhibitor drugs are also used to treat nasal cavity and paranasal sinus cancers. These drugs interfere with the growth and division of tumor cells by inhibiting a hormone that encourages their growth. EGFR drugs include cetuximab (Erbitux), gefitinib (Iressa), and erlotinib (Tarceva). Modern research focuses on genetic therapy and drugs that target tumors with TP53 mutations.

Prognosis, prevention, and outcomes: Prognosis depends on the tumor's stage and location and the patient's age and condition. Patients with cancer localized to the nasal cavity have a five-year survival rate of 86 percent. Those with a regional presentation in which the cancer has spread to the lymph nodes or nearby structures have a five-year survival rate of 52 percent, while those with cancers that spread to distant areas of the body have a 43 percent five-year survival rate. Nasal cavity and paranasal sinus cancers cannot be prevented, but avoiding risk factors can decrease the likelihood of developing one of these cancers.

The outcome of a nasal cavity or paranasal sinus cancer depends on the type of tumor and the tissue from which it arises, the size of the tumor, and whether it has metastasized. Some types, like melanoma, are rapidly fatal. Others grow slowly and may be resected successfully. The more extensive the tumor, the more likely it is to affect the cranial nerves and the sense of smell. More advanced tumors are likely to cause facial deformity and interfere with tasting, smelling, and vision. Surgery damage to the cranial nerves can affect the ability to open, close, and move the eyes, chew and swallow, and change facial expressions.

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