Testicular Lymphoma

Lymphoma of the Tests

What is testicular lymphoma and how is it treated?. Istockphoto.com/Stock Photo©claudiobaba

What is testicular lymphoma and how does it differ from other forms of non-Hodgkin lymphoma?

Testicular Lymphoma - Definition

Lymphoma can affect the testes in a few ways. A lymphoma may start in the testes, in which case it is referred to as primary testicular lymphoma, or a lymphoma may involve the testes as part of a widespread disease that involves many other sites.

Is Testicular Lymphoma Common?

The testes are not a common site for lymphoma.

In fact, the testes are far more commonly affected by another cancer called germ cell tumors. Less than 5% of individuals with cancer in the testes actually have lymphoma. In people over the age of 50 years, however, lymphoma is the most common cancer of the testes as germ cell tumors in this age group are rare.

Type of Lymphoma of the Testes

Lymphoma of the testes is almost always a type of non-Hodgkin's lymphoma (NHL). The most common type is diffuse large B-cell lymphoma. Other types are immunoblastic lymphoma, Burkitt lymphoma (in children), and follicular lymphoma.

There are a few terms you may hear if you are diagnosed with testicular lymphoma. One is "extranodal presentation."  Primary extranodal lymphoma simply means that the lymphoma is first found (originated) outside of the lymphatic system, in other words, it is found in the testes first rather than in a lymph node, the spleen, the bone marrow, or thymus.

The testes (along with the central nervous system) are also referred to as sanctuary sites.

Causes of Testicular Lymphoma

It's not known what the causes of testicular lymphoma are, but there are several general risk factors for non-Hodgkin lymphoma. There is an association with viral infections, particularly EBV (the epstein barr virus which causes mono), CMV (cytomegalovirus infection), parvovirus B19 (the virus that causes "fifths" disease, a common rash associated viral infection in children), and HIV.

Signs and Symptoms of Testicular Lymphoma

The common symptom is the enlargement of one of the two testes. There is usually little or no pain associated with this. The testis may feel heavy. If the lymphoma has spread to other parts of the body, there may be symptoms related to the parts affected. There may be one or more of the B-symptoms of lymphoma -- fever, weight loss or sweating at night.

Diagnosis

An enlarged testicle can have many causes, and your doctor can easily identify some common and simple conditions. If there is a suspicion of a tumor, an ultrasound or CT scan of the testes will be recommended as a first test. Some blood tests for tumor markers are done in order to identify germ cell tumors, the more common type of testis cancer. The best way to find out the exact type of tumor is to remove the testis with simple surgery. A needle test or a biopsy is usually not recommended because of the risk of tumor "seeding" - spreading cancer cells around the area where the needle is inserted.

The removed testicle tissue is then viewed under a microscope, and a final diagnosis is made.

Tests After Diagnosis

If the testicular tumor is a lymphoma, a set of tests is required before treatment can start. This usually always includes CT scans of the abdomen and the chest and a bone marrow test. Testicular lymphoma can also spread into the cerebrospinal fluid (CSF) that flows inside the brain and spinal cord. A lumbar puncture (spinal tap) may be performed to remove a small amount of this fluid from the spine in the lower back and test it for lymphoma cells.

Treatment and Results

The treatment of testicular cancer usually involves removing the tumor, but more treatment is required to prevent the cancer from coming back in the other testicle, central nervous system, and other extranodal sites. 

Surgery - Removing the testicle (orchiectomy) is the first part of treatment and often gets done as a part of diagnosis.

Chemotherapy - The chemotherapy regimen CHOP is often given along with Rituxan.  The drugs in this regimen include Cytoxan (cyclophosphamide), Adriamycin (doxorubicin), Oncovin (vincristine), and prednisone.

Targeted therapy - Rituxan (rituximab) is a monoclonal antibody that is usually given along with chemotherapy. Our bodies make antibodies to fight off bacteria and viruses. Rituxan is essentially a man made antibody designed to fight lymphoma cells.

Radiation - Radiation is often done to the pelvic region, especially to decrease the likelihood of the cancer recurring in the other testicle.

Central nervous system prophylaxis - Central nervous system prophylaxis is usually included as part of treatment to prevent this lymphoma from spreading or recurring in the central nervous system.

Preserving Fertility

Often only one testicular needs to be removed, but both chemotherapy and radiation therapy can result in infertility. Thankfully sperm banking is a viable option for many men. Make sure to talk with your doctor about preserving fertility during cancer treatment so you are aware of all of your options before you begin treatment.

Coping and Support

If you've been diagnosed with testicular lymphoma you're probably feeling overwhelmed. Since this is an uncommon tumor you may feel very alone, and wonder who you can talk to. Reach out to family and friends. Consider connecting with the cancer community online. We live in an age when you can find others with testicular lymphoma all around the world, and can even find support and people to bounce thoughts off of 24/7. Research and learn about your disease, and take an active part in your care. The treatment of this disease is aggressive, but unlike many cancers offers a good chance for long term control of the disease.

Sources:

Cancer: Principles and Practice of Oncology 7th Edition. Editors: DeVita VT, Hellman S, Rosenberg SA. Lipincott Williams & Wilkins 2005.

Chapuy, B., Roemer, M., Steward, C. et al. Targetable genetic features of primary testicular and primary central nervous system lymphomas. Blood. 2016. 127(7):869-81.

Cheah, C., Wirth, A., and J. Saymour. Primary testicular lymphoma. Blood. 2014. 123(4):486-93.

Huang, Y., Zhang, Z., Tashkin, D., Feng, B., Straif, K., and M. Hashibe. An epidemiologic review of marijuana and cancer: an update. Cancer Epidemiology Biomarkers and Prevention. 2015. 24(1):15-31.

Vitolo, U., Chiappella, A., Ferreri, A. et al. First-line treatment for primary testicular diffuse large B-cell lymphoma with rituximab-CHOPD, CNS prophylaxis, and contralateral testis irradiation: final results of an international phase II trials. Journal of Clinical Oncology. 2011. 29(20):2766-72.

Yousif, L., Hammer, G., Blettner, M., and H. Zeeb. Testicular cancer and viral infections: a systematic literature review and meta-analysis. Journal of Medical Virology. 2013. 85(12):2165-75.

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