Lymphoma of the testis as primary location: tumour review

Non-Hodgkin's lymphoma as a primary testicular 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 histotype in primary forms is the diffuse large B-cell lymphoma, whereas more aggressive histologies such as Burkitt's lymphoma are principal founded in cases of secondary involvement 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 present 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) combination 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.