Background:    Primary ovarian non-Hodgkin’s lymphoma (PONHL) is an uncommon entity; its pathology is usually diffuse large B cell lymphoma (DLBCL).
    Case Reports:    We report 3 cases of ovary involvement of DLBCL, 1 of which rapidly developed to central nervous system involvement. Diagnosis and subsequent treatment are discussed and the literature on the origin, epidemiology, clinical presentation, diagnosis, treatment and prognosis of ovary lymphoma are reviewed. All patients were diagnosed as having DLBCL after ovary biopsy, and were subsequently given regular chemotherapy. Two of them obtained remission and 1 of them had central nervous system involvement.
    Conclusions:    Ovary involvement of DLBCL is rare; prognosis is related to the overall clinical manifestation and some serum biomarkers. Diagnosis is established by ovary biopsy. Inaccurate or delayed diagnosis is often due to the lesions presenting as a mass resembling ovary cancer and may lead to poor outcome. Treatment regimen mainly consists of chemotherapy (CHOP) associated with rituximab. Intrathecal chemotherapy may play an important role in prevention of central nervous system involvement.

Keywords: Ovary, DLBCL-lymphoma, central nervous system (CNS), intrathecal chemotherapy