Primary ovarian lymphoma is an extremely rare disease and limited count reports about it have been reported in the literature. Traditionally, patients with rheumatoid arthritis (RA) have increased risk of nodal and extranodal lymphoid malignancies such as non-Hodgkin's lymphoma (NHL). Recently, some studies have also reported association between patients with juvenile rheumatoid arthritis (JRA) treated with Methotrexate (MTX) and malignant lymphoma developing.
We report a 17-year old JRA patient with primary ovarian diffuse large B-cell non-Hodgkin's lymphoma (NHL). The patient had seronegative (rheumatoid factor negative) poliarticular form of JRA and was receiving low dose weekly Methotrexate (MTX) during the past 2 years. Initial presentation was adnexial mass and chronic pelvic pain. The patient was treated with surgery and combined cytotoxic chemotherapy.
In conclusion, because of increased lymphoid malignancy risk, ovarian masses in JRA patients should be carefully evaluated.