Lymphoma is a cancer that affects a type of white blood cell called lymphocytes. These cells work as part of the immune system to help fight infection. Although lymphoma in the tonsil area makes up about half of the cases of extranodal non-Hodgkin lymphoma of the head and neck, it is overall a very rare condition.
Tonsil lymphoma, also called primary tonsillar lymphoma, is a type of cancer that forms on the tonsils. The tonsils are located in an area of the body known as the Waldeyer’s tonsillar ring. This area includes different types of lymphoid tissue. Located at the back of the throat are the palatine tonsils, which are the most common site of tonsil lymphoma.
Lymphoma is divided into two main categories, Hodgkin lymphoma and non-Hodgkin lymphoma. Although both types of lymphoma can be found in the tonsils, tonsil lymphoma is typically non-Hodgkin lymphoma. More specifically, it is usually a subtype of non-Hodgkin lymphoma called diffuse large B-cell lymphoma.
The precise cause of tonsil lymphoma is unknown. However, it has been associated with Epstein-Barr virus infection. Human papillomavirus (HPV) infection may also be involved in some cases of tonsil lymphoma, although HPV has been more strongly associated with other forms of tonsil cancer, such as squamous cell carcinoma. Other risk factors include tobacco and alcohol use.
Tonsil lymphoma can cause symptoms that may include:
Similar symptoms may be caused by squamous cell carcinoma of the tonsils, which is more common than tonsil lymphoma.
Symptoms that commonly occur with many forms of lymphoma — including fever, night sweats, and weight loss — may occur in a small percentage of people with tonsil lymphoma, but they are generally uncommon.
Various tests are required to diagnose tonsil lymphoma and distinguish it from squamous cell carcinoma or other diseases of the tonsils.
A lymph node biopsy is required to diagnose tonsil lymphoma. For the biopsy, a sample of tissue is removed from the lymph nodes at the back of the neck and examined for the presence of cancer cells. The procedure can be a surgical biopsy, which removes an entire lymph node from the body. Alternatively, it can be a needle biopsy, which removes only part of a lymph node.
To help diagnose tonsil lymphoma, a doctor may perform a bone marrow biopsy or aspiration to determine if there are cancer cells in the bone marrow.
Your doctor may use imaging technologies to view tissues in the body and to identify a cancerous mass in the tonsils. These technologies may include computerized tomography (CT) or positron emission tomography (PET) combined with CT, called PET-CT. X-rays may be used to determine if the tumor has spread to the lung.
Blood tests are routinely used to measure blood cell counts and levels of the lactate dehydrogenase protein, which can help a doctor determine how advanced the lymphoma is.
A spinal tap, also called a lumbar puncture, is a technique that uses a needle to collect cerebrospinal fluid from the spinal canal. This test may be performed to determine if the cancer is advanced and has spread to the central nervous system.
There are multiple treatment options for tonsil lymphoma. The recommended treatment may depend on how far the cancer has spread.
The standard treatment for tonsil lymphoma is chemotherapy. Specific treatment regimens use multiple drugs in combination. Many people are given a chemotherapy combination known as CHOP, which consists of:
Although chemotherapy may be used alone, it is more commonly used with a drug called rituximab (Rituxan). Rituximab is an antibody that recognizes a specific molecule called CD20, which is present on the surface of lymphoma cells. CHOP chemotherapy together with rituximab is called R-CHOP.
Radiation therapy may be used to treat tonsil lymphoma, often in combination with chemotherapy.
Surgery to remove the affected tonsils, called a tonsillectomy, is also used in some cases to treat tonsil lymphoma.
Tonsil lymphoma is most often diagnosed in the early stages of the disease and, when treated promptly, has a generally good prognosis (outlook). After treatment with chemotherapy and radiation, many people go into remission, meaning there is no evidence of cancer in the body. The overall survival rate is around 80 percent.
However, some people experience relapse, which is when the cancer comes back after treatment. When the disease relapses, the cancer is more difficult to treat and results in a poorer prognosis. A late-stage diagnosis can also result in a worse outlook, but tonsil lymphoma is often misdiagnosed. An accurate diagnosis, as soon as possible, is important for optimizing therapies, giving timely treatment, and improving the prognosis of people with tonsil lymphoma.
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