Lymphoma of the female genital tract: Current status

Primary lymphomas affecting the female reproductive system are uncommon but often pose a diagnostic challenge if their existence is not suspected. This article reviews the pathological and clinical features of lymphomas occurring in various sites in the female genital tract including the vulva, vagina, cervix, endometrium, fallopian tubes, and ovaries. Using the recent World Health Organization classification, the various types of lymphomas are identified as separate diseases and not as morphological variations of the same disease. The immunophenotypic and cytogenetics features of the major lymphomas are summarized. The incidence, presenting symptoms, gross and microscopic features, major differential diagnostic considerations, response to therapy, and expected outcome are discussed. Using published data on patient outcome, the International Federation of Obstetricians and Gynecologists and Ann Arbor staging systems are compared for their predictive value, and the difficulty in assigning primary and secondary status in extranodal lymphomas is emphasized. The observed differences in the behavior of some lymphomas in gynecological sites compared with their usual nodal location are presented. Finally, the possible etiology of these conditions is discussed in light of the emerging paradigm of mucosa-associated lymphoid tissue lymphomas.