HIV-related primary non-Hodgkin's lymphoma of the vulva

We reported on a 25-year-old HIV-positive woman diagnosed with Ann Arbor Stage IEB primary extranodal immunoblastic lymphoma arising in the vulva. This is the first documented instance of an HIV-associated malignant lymphoma originating in the lower female genital tract, and only the 16th reported case of primary malignant lymphoma of the vulva. The nonspecific clinical presentation coupled with the unanticipated finding of lymphoma made diagnostic confirmation an arduous task, requiring weeks of persistence. The patient's disease was refractory to three courses of chemotherapy, but did respond to a brief palliative course of external beam irradiation prior to her demise 7 months after presentation. Our findings are presented, and 15 cases from the literature, the largest series to date, are discussed.