Abstract:  Objective To investigate the clinicopathological characteristics, diagnosis, treatment and prognosis of primary ovarian diffuse large B⁃cell lymphoma (PODLBCL). Methods The clinical data of 4 patients with PODLBCL admitted to Beijing Chao⁃yang Hospital, Capital Medical University from January 2003 to January 2021 were retrospectively analyzed and the literature was reviewed. Results The 4 cases of PODLBCL patients were diagnosed by postoperative histopathology. The main clinical symptoms were pelvic masses and abdominal pain; 2 cases with B symptoms, 2 cases with bilateral ovarian tumors, 2 cases with unilateral tumors (both occurred in the right ovary) and 3 cases with high expression of CA125 and lactate dehydrogenase (LDH). Microscopically, the normal ovary was destroyed and diffusely infiltrated by relatively single and large lymphocytes. The cytoplasm was usually basophilic, and the nucleus was round or oval, with single or multiple nucleoli; mitotic figures were common, and CD20 staining was positive. The treatment included surgery, chemotherapy and monoclonal antibody treatment. After 2-238 months of follow⁃up, 3 patients survived without recurrence and 1 patient died. Conclusions PODLBCL is a rare diffuse large B⁃cell lymphoma with no specific clinical manifestation. The diagnosis depends on postoperative histopathology, and the prognosis is good. Chemotherapy combined with monoclonal antibody therapy may be the first⁃line treatment option to avoid surgery, but a definitive diagnosis before surgery is critical.

Key words: Diffuse large B?cell lymphoma, Primary ovarian lymphoma, Diagnosis, Treatment