Abstract Non-Hodgkin’s lymphoma as a primary testicu- lar neoplasm accounts approximately 9% of all testicular malignant tumours and about 1–2% of all non-Hodgkin’s lymphoma. This neoplasm is the most common malignant tumour of the testis in the elderly. The most common histo- type in primary forms is the diffuse large B-cell lymphoma, whereas more aggressive histologies such as Burkitt’s lym- phoma are principal founded in cases of secondary involve- ment of the testis. Regarding clinical presentation, the most common sign is a unilateral painless scrotal swelling, sometimes with sharp scrotal pain or hydrocele. In patients with advanced stage, the systematic B symptoms are pres- ent in 25–41% of all cases. In 35% of patients, bilateral testicular involvement is detected. In more advanced stages with para-aortic lymph-node involvement, ascites and abdominal pain is evident. Despite the fact that responses to doxorubicin- containing chemotherapy, especially in early stages, show good results, relapses are often seen, and the prognosis of this tumour is very poor. Testicular lymphoma often disseminates to other extranodal organs, such as contralateral testis, central nervous system (CNS), lung, pleura, Waldeyer’s ring and soft tissue. For patients with limited disease, the recommended first-line treatment is orchiectomy followed by rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisone (R-CHOP) com- bination chemotherapy, with central nervous system (CNS) prophylaxis and prophylactic irradiation of the contralateral testis. In more advanced or relapsed disease, management should follow the worldwide recommendations for nodal diffuse large B-cell lymphoma (DLBCL). Here we present a review of this tumour. Keywords Non-Hodgkin’s lymphoma · Testicular lymphoma · Management · Chemotherapy · Radiation therapy Introduction Non-Hodgkin’s lymphoma with primary location in the testis is a rare disease. It represents about 9% of testicu- lar tumours and 1–2% of all non-Hodgkin’s lymphomas, with an estimated incidence of 0.26/100,000 per year [1]. Contrary to its low overall incidence, it is the most com- mon testicular cancer in men older than 50 years of age, and 85% of all cases are diagnosed in men older than 60 years of age [2]. The incidence of bilateral involvement and a tendency for extranodal spread to the skin, subcu- taneous tissue, central nervous system (CNS), lung, and Waldeyer’s ring is rather high. In primary forms, the most common histotype is the diffuse large B-cell lymphoma (DLBCL), whereas aggressive histologies, particularly Burkitt’s lymphoma, are principally found in cases of secondary involvement of the testis. Despite the fact that responses to doxorubicin-containing chemotherapy, espe- cially in early stages, show good results, relapses are often seen, and the prognosis of this tumour is very poor. In case of relapse, the CNS and contralateral remaining testicle are often involved [2, 3]. The etiology of this malignancy G. Koukourakis () Saint Savvas Anticancer Institute of Athens Alexandra’s Avenue 171 Athens Greece e-mail: gkoyokoyrakis@yahoo.gr V. Kouloulias Attikon University Hospital of Athens Second Radiology Department Radiation Therapy Unit Athens, Greece Clin Transl Oncol (2010) 12:321-325 DOI 10.1007/s12094-010-0513-9 EDUCATIONAL SERIES Blue Series Lymphoma of the testis as primary location: tumour review Georgios Koukourakis · Vassilios Kouloulias Received: 25 February 2010 / Accepted: 16 March 2010 MOLECULAR AND CELLULAR BIOLOGY OF CANCER