Abstract
Primary testicular non-Hodgkin's lymphoma was first described as a clinical entity in 1866. It is a rare disease and accounts for 1% of all non-Hodgkin's lymphoma, 2% of all extranodal lymphomas and 5% of all testicular neoplasms. It is the most common testicular tumor in males between sixty and eighty years of age. Testicular non-Hodgkin's lymphoma is unique in its high incidence of bilateral involvement (8-38%), and it is also the most common bilateral testicular tumor. Testicular non-Hodgkin's lymphoma has a predilection for spreading to non-contiguous extranodal sites, especially the central nervous system. Advanced-stage disease is usually managed with doxorubicin-based chemotherapy. For early-stage disease, opinion is divided regarding systemic chemotherapy following orchidectomy. The high incidence of spreading, especially to the central nervous system, leads to advocacy of the use of central nervous system prophylaxis with intrathecal chemotherapy. Prospective multicenter trials incorporating a large number of patients may lead to better guidelines for optimal management of this subtype of non-Hodgkin's lymphoma.
Highlights
Testicular non-Hodgkin’s lymphoma (TNHL) is a rare entity and constitutes only 1-7% of all testicular neoplasms, it is the most common testicular tumor in males between sixty and eighty years of age
testicular non-Hodgkin’s lymphoma (TNHL) is unique in its high incidence of bilateral involvement (8-38%), and it is the most common bilateral testicular tumor
Primary TNHL was first described as a clinically entity in 1866.1,3 It is a rare disease and accounts for 1% of all non-Hodgkin’s lymphoma (NHL) cases, 2% of all extranodal lymphomas and 5% of all testicular neoplasms.[16]
Summary
Testicular non-Hodgkin’s lymphoma (TNHL) is a rare entity and constitutes only 1-7% of all testicular neoplasms, it is the most common testicular tumor in males between sixty and eighty years of age. TNHL has a predilection for spreading to non-contiguous extranodal sites, especially the central nervous system (CNS).[1,2,3,4,5,6] The majority of reported TNHL cases present diffuse histology, and usually B-cell type.[1,3,4,7,8,9] As it is a rare tumor, and population-based studies are few, its treatment is controversial, especially regarding its early stage.[10] the use of systemic chemotherapy following orchidectomy is a standard treatment for advanced disease.[7,10,11] The prognosis for TNHL is poor, if disseminated disease is evident within one year after diagnosis.[5,12] CNS prophylaxis with intrathecal chemotherapy is advocated by many authors, in view of the fact that the majority of patients present relapse within the first two years following treatment, and such recurrence is especially in the CNS.[10,13,14,15]
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