Primary lymphoma of the female genital tract: a retrospective survey of the International Extranodal Lymphoma Study Group (IELSG35)

Introduction: Primary lymphoma of the female genital tract (PLFGT) is an uncommon extranodal lymphoma. There are few reported series of PLFGT in the literature, and most of them are case reports. Methods: We retrospectively collected and analyzed data on presentation, treatment, and outcome of 60 female patients (pts) diagnosed with PLFGT between 1982 and 2013. Our aim was to investigate baseline features associated with patient outcome (including age, stage, LDH, IPI, PS, bulky disease, primary site, aggressive histology, and use of rituximab). Univariable and multivariable analyses were performed using the log-rank test and a stepwise Cox regression, respectively. Results: The median age at diagnosis was 52 years. Ann Arbor stage I-II was observed in 32 pts while 38 pts had localized disease according to the FIGO staging systems. Uterus was the primary site in 26 pts, 23 had ovarian involvement and 11 had vaginal or vulvar involvement. Fourteen pts had multiple gynecologic sites affected at diagnosis. Diffuse large B-cell lymphoma (DLBCL) was the most common subtype, occurring in 39 patients, followed by extranodal marginal zone lymphoma and follicular lymphoma (6 patients each). Surgery alone was given to 2 patients as first-line therapy, while systemic therapy was administered to 58, 16 of whom had undergone previous major surgery. Consolidation radiotherapy was given to 13 patients, all but one of whom had pelvic lesions. Six patients received central nervous system (CNS) prophylaxis (4 high-dose methotrexate, 1 intrathecal methotrexate, and 1 unspecified prophylaxis). Fifty-four patients responded to treatment (49 complete and 5 partial responses), while 20 experienced disease progression or relapse. Of those, 6 relapsed in the CNS (which was the only recurrence site in 5). All but one patient with CNS relapse had ovarian involvement, 3 had bulky disease, and none had received previous prophylaxis. With a median follow-up of 60 months, progression-free survival (PFS) at 5 and 10 years was 66% and 57%, respectively. At the last follow-up, 44 patients were alive (42 of whom were in complete remission), 13 had died from lymphoma, and 2 had died from other causes, while 1 patient was lost to follow-up. The median overall survival was 12.7 years with 5- and 10-year overall survival (OS) of 77% and 68%, respectively. Only FIGO advanced disease remained significantly associated with poorer PFS and OS at multivariable analysis. Conclusions: This PLFGT survey showed the prognostic impact of gynecological staging procedures and a sizeable risk of CNS relapse. These findings may have treatment implications and highlight the utility of multidisciplinary management, as well as the need for further research to identify predictive factors for CNS relapse Keyword: Extranodal non-Hodgkin lymphoma No conflicts of interests pertinent to the abstract.