Primary ovarian non-Hodgkin lymphoma (PONHL) is extremely rare, accounting for 0.5% of all NHLs and 1.5% of all ovarian tumors. Establishing an early and accurate clinical as well as histopathological diagnosis, although challenging, is essential for the appropriate management of these patients and to avoid unnecessary surgeries. Furthermore, it is important to exclude secondary involvement of the ovary by disseminated NHL in these patients, as the two entities carry significantly different prognosis. The diagnostic struggle is mainly due to its rarity, morphological similarities with other malignancies and the relative lack of awareness among the histopathologists. In this article, we present a case of primary ovarian diffuse large B cell lymphoma in a young nulliparous female, who was diagnosed based on histopathological and immunohistochemical findings.
Keywords: chemotherapy; diffuse large B cell lymphoma; non-Hodgkin lymphoma; primary ovarian lymphoma; primary ovarian non-Hodgkin lymphoma.