Nasal lymphoma is a rare cancer originating in the lymphoid tissues of the nasal cavity and adjacent paranasal sinuses. As a subtype of non-Hodgkin lymphoma, it develops from lymphocytes (a type of white blood cell) outside of the lymph nodes, classifying it as an “extranodal” lymphoma.
Symptoms and Initial Signs
The initial signs of nasal lymphoma involve localized issues in the nasal and facial regions. Common symptoms include persistent congestion on one side of the nose, recurrent nosebleeds, a diminished sense of smell, or drainage of pus. As a tumor grows, it can cause facial pain, pressure, or visible swelling around the eyes or cheeks.
A noticeable lump may be felt on the face or seen inside the nose, and some people experience pain or loosening of the upper teeth. Because these symptoms can be mistaken for more common ailments like chronic sinusitis, a delay in diagnosis can occur.
Some individuals also develop systemic “B symptoms” associated with lymphoma. These include unexplained fever that comes and goes, drenching night sweats, and unintentional weight loss of more than 10% of body weight over a six-month period. The presence of these generalized symptoms alongside nasal issues can indicate the disease.
Causes and Risk Factors
The development of nasal lymphoma is strongly associated with the Epstein-Barr virus (EBV). In most cases of extranodal NK/T-cell lymphoma, nasal type, EBV’s genetic material is found within the cancerous cells. The viral genes are thought to alter the growth cycle of lymphocytes, contributing to their transformation into cancer.
This lymphoma is more common in individuals of Asian and Latin American descent compared to those of European ancestry. This suggests a potential genetic predisposition that increases risk when combined with EBV infection.
A compromised immune system is another contributing factor. Conditions that weaken immune defenses, such as HIV/AIDS or the use of immunosuppressive drugs after an organ transplant, increase risk. A weakened immune response can hinder the body’s ability to control the proliferation of EBV-infected cells, allowing lymphoma to develop.
The Diagnostic Process
Diagnosing nasal lymphoma begins with a physical examination and a review of the patient’s medical history, where a physician inspects the head and neck for swelling or lumps. If nasal lymphoma is suspected, the next step is a nasal endoscopy. During this procedure, an ear, nose, and throat (ENT) specialist inserts a thin, flexible tube with a camera into the nasal cavity for direct visual inspection.
A definitive diagnosis requires a biopsy, where a small tissue sample is surgically removed from the suspicious area for laboratory analysis. A pathologist then examines the tissue under a microscope to identify cancer cells.
Once a diagnosis is confirmed, imaging tests are used to determine the cancer’s stage, or how far it has spread. Computed tomography (CT) scans show detailed images of the nasal cavity’s bones, while magnetic resonance imaging (MRI) is better for visualizing soft tissues. A positron emission tomography (PET) scan may also be performed to detect cancer cells in other parts of the body.
Treatment Approaches
The primary treatment for nasal lymphoma confined to the nasal area is radiation therapy, which uses high-energy beams to destroy cancer cells. Radiation is often delivered in daily sessions over several weeks. In many cases, it is combined with chemotherapy in a treatment known as chemoradiation to improve outcomes.
For advanced lymphoma that has spread, chemotherapy is the main treatment. Drug regimens are specifically chosen for this lymphoma type, such as the SMILE regimen, which includes dexamethasone, methotrexate, ifosfamide, L-asparaginase, and etoposide. Nasal lymphoma cells can be resistant to standard chemotherapy drugs, necessitating these specialized combinations.
If the cancer returns (relapsed) or does not respond to initial treatment (refractory), other options are considered. A stem cell transplant may be recommended to allow for higher doses of chemotherapy. Targeted therapy drugs, which focus on specific vulnerabilities in cancer cells, may also be used, often as part of a clinical trial.
Prognosis and Recovery
The outlook for an individual with nasal lymphoma is heavily influenced by the cancer’s stage at diagnosis. For localized disease confined to the nasal cavity, the prognosis is more favorable, with a 5-year relative survival rate of around 86%.
If the cancer has spread to nearby tissues or regional lymph nodes, the 5-year relative survival rate drops to approximately 52%. For distant disease that has metastasized, the rate is about 43%. Other factors affecting the outcome include the patient’s age, overall health, and how well the lymphoma responds to treatment.
After treatment, regular follow-up care is necessary for recovery. These appointments allow the medical team to monitor for any signs of recurrence. This surveillance often includes physical exams and periodic imaging scans.