Lymphoma can affect the testes in several ways. The lymphoma may start in the testis, in which case it is called primary testicular lymphoma, or the lymphoma may involve the testis as part of a broader disease involving many other sites.
The testis is not a common site for lymphoma. In fact, the testes are more susceptible to another type of cancer called germ cell tumors. Fewer than 5% of testicular cancer patients actually have lymphoma.However, in people over the age of 50, lymphoma is the most common testicular cancer because germ cell tumors are rare in this age group.
Testicular lymphoma is almost always a type of non-Hodgkin lymphoma (NHL). The most common type is diffuse large B-cell lymphoma. Other types are immunoblastic lymphoma, Burkitt lymphoma (childhood), and follicular lymphoma.
If you’ve been diagnosed with testicular lymphoma, you’ve probably heard some terms. One is “extra-nodal performance”. Primary extranodal lymphoma simply means that the lymphoma was first discovered (originated) outside the lymphatic system, in other words, it first appeared in the testes, not in the lymph nodes, spleen, bone marrow, or thymus. The testis (along with the central nervous system), also known as the sanctuary, is an area where chemotherapy drugs are difficult to obtain.
It is not known what the cause of testicular lymphoma is, but there are several general risk factors for non-Hodgkin lymphoma. Associated with viral infections, particularly EBV (Epstein-Barr virus that causes monovirus infection), CMV (cytomegalovirus infection), parvovirus B19 (virus that causes “one in five” disease, a common childhood rash-associated viral infection), and HIV.
signs and symptoms
A common symptom is an enlargement of one of the two testicles. There is usually little or no pain associated with this. The testicles may feel heavy. If the lymphoma has spread to other parts of the body, symptoms related to the affected area may occur. Lymphoma may have one or more of the B symptoms — fever, weight loss, or night sweats.
Swollen testicles can have many causes, and your healthcare provider can easily identify some common and simple conditions. If a tumor is suspected, an ultrasound or CT scan of the testis is recommended as a first examination. Blood tests for some tumor markers are done to identify germ cell tumors, the more common type of testicular cancer.
The best way to find out the exact tumor type is to remove the testicle with simple surgery. Needle testing or biopsy is generally not recommended because of the risk of tumor “seeding” (spreading of cancer cells around the area where the needle was inserted). The removed testicular tissue is then observed under a microscope and a final diagnosis is made.
If the testicular tumor is lymphoma, a series of tests are required before starting treatment. This usually always includes CT scans of the abdomen and chest and bone marrow tests. Testicular lymphoma can also spread to the cerebrospinal fluid (CSF) that flows in the brain and spinal cord. A lumbar puncture (spinal tap) may be done to remove a small amount of this fluid from the spine in the lower back and test it for lymphoma cells.
Treatment of testicular cancer usually involves removing the tumor, but more treatment is needed to prevent the cancer from recurring in other testicular, central nervous system, and other extranodal sites.
Surgery: Removal of the testicle (orchiectomy) is the first part of treatment and is usually done as part of the diagnosis.
Chemotherapy: The chemotherapy regimen CHOP is often used with Rituxan (rituximab). CHOP stands for Cytoxan (cyclophosphamide), doxorubicin (hydroxydaunorubicin), Oncovin (vincristine), and prednisone.
Targeted therapy: Rituxan is a monoclonal antibody that is often used with chemotherapy. Our bodies produce antibodies to fight bacteria and viruses. Rituxan is essentially a man-made antibody designed to fight lymphoma cells.
Radiation: Radiation is usually given to the pelvic area, especially to reduce the chance that the cancer will recur in the other testicle.
CNS prophylaxis: CNS prophylaxis is often given as part of treatment to prevent the spread or recurrence of this lymphoma in the central nervous system.
Immunotherapy: Chimeric antigen receptor (CAR) T cell therapy is a type of immunotherapy that modifies a patient’s own T cells to help destroy cancer cells in their body. Yescarta (axicabtagene ciloleucel) and Kymriah (tisagenlecleucel) are two CAR T-cell therapies approved for certain testicular lymphoma patients.
Usually only one testicle needs to be removed, but both chemotherapy and radiation therapy can cause infertility. Thankfully, sperm banks are a viable option for many men. Be sure to discuss maintaining fertility during cancer treatment with your healthcare provider so you know all your options before starting treatment.
Coping and Support
If you’ve been diagnosed with testicular lymphoma, you may feel overwhelmed. Since this is an uncommon tumor, you may feel very lonely and wonder who you can talk to. Connect with family and friends. Consider connecting with the cancer community online. We live in a time where you can find other people with testicular lymphoma all over the world and even support and people recovering their minds 24/7.
Research and understand your disease and be actively involved in your care. Treatment for this disease is aggressive, but unlike many cancers, it offers a good chance for long-term control of the disease.