BACKGROUND: Primary ovarian lymphoma represents a rare clinical entity accounting for 0.5% of non-Hodgkin lymphomas and 1.5% of ovarian neoplasms. Current literature is limited to small case series, with poorly defined prognostic factors and optimal treatment strategies. METHODS: This retrospective cohort study utilized the SEER database (2000-2020) to identify patients with ovarian lymphoma. Patients were categorized as localized primary ovarian lymphoma (L-POL, stage I) or lymphoma involving the ovary (LIO, stages II-IV or unknown). Cox proportional hazards regression identified independent prognostic factors for overall survival (OS) and cancer-specific survival (CSS). RESULTS: A total of 221 patients were identified, representing the largest ovarian lymphoma cohort ever reported. The cohort included 75 L-POL (33.9%) and 146 LIO (66.1%) patients. Median age was 49 years. Diffuse large B-cell lymphoma was the most common histology (62.0%). L-POL patients demonstrated higher proportions of Black patients (13.3% vs 4.1%, p=0.022), follicular lymphoma (22.7% vs 12.3%), and unilateral disease (89.3% vs 61.6%). At median follow-up of 80 months, 5-year and 10-year CSS rates were 77.7% and 75.3% overall, and 87.4% and 85.5% for L-POL patients. In multivariate analysis, age (HR 1.035, p<0.001), L-POL (grade I) classification (HR 0.435, p=0.023), and high-grade histology (HR 4.898, p=0.009) were independent prognostic factors for CSS. Surgery, radiotherapy, and chemotherapy showed no survival benefit in multivariate analysis. Surgery was performed in 86.9% of patients, with 9.9% undergoing inappropriate debulking procedures. CONCLUSIONS: L-POL represents a distinct clinical presentation with excellent long-term prognosis. Age, L-POL classification, and histological grade are independent prognostic factors. The high rate of inappropriate surgical procedures, ranging from hysterectomy and debulking to pelvic exenteration, underscores the critical need for improved preoperative recognition. Optimal management should emphasize limited surgical intervention for diagnosis and fertility preservation, followed by systemic chemotherapy tailored to lymphoma subtype and stage.
Loap et al. (Thu,) studied this question.