Primary Diffuse Large B-Cell Lymphoma of Ovary: Report of Cases and Literature Review

Abstract

Objective: Our study aimed to review the characters of primary diffuse large B-cell lymphoma (DLBCL) of ovary and provide recommendations of management for this rare disease. Methods: We presented 4 cases of primary ovarian DLBCL (PODLBCL) from Beijing Chaoyang Hospital between 2003 and 2021. Furthermore, we searched PubMed and Web of Science database for literature published between 1998 and 2021.A total of 16 articles included 24 cases with histopathologically confirmed PODLBCL and detailed evaluation and follow-up were reviewed. Results: The median age of patients with PODLBCL was 43y (5-73y). The main clinical symptoms were pelvic mass, abdominal pain, followed by irregular vaginal bleeding and urinary incontinence. 25% patients had bilateral ovarian tumors, and 61.1% of unilateral tumors were in the left ovary. Cancer antigen 125 (CA125) and serum lactate dehydrogenase (LDH) were usually elevated to varying degrees. Most cases were diagnosed by surgery and presented in early stage. Treatments included surgery, chemotherapy and monoclonal antibody therapy. There was no evidence of recurrence within a median follow-up time of 20 months (range 5-72 months) in 79.1% (19/21) of patients. Conclusions: PODLBCL had no significantly specific character to distinguish from ovarian epithelial malignant tumors. The prognosis of patients with PODLBCL was usually good. We proposed that chemotherapy combined with monoclonal antibody therapy may be the first-line treatment for PODLBCL, and surgical resection of the tumor may be avoided. The key problem is how to make an early preoperative diagnosis. Therefore, more case reports and institutional studies are needed to confirm these conclusions.