We report on a 25-year-old HIV-positive woman diagnosed with Ann Arbor
Stage IEB primary extranodal immunoblastic lymphoma arising in the vu
lva. This is the first documented instance of an HIV-associated malign
ant lymphoma originating in the lower female genital tract, and only t
he 16th reported case of primary malignant lymphoma of the vulva. The
nonspecific clinical presentation coupled with the unanticipated findi
ng of lymphoma made diagnostic confirmation an arduous task, requiring
weeks of persistence. The patient's disease was refractory to three c
ourses of chemotherapy, but did respond to a brief palliative course o
f external beam irradiation prior to her demise 7 months after present
ation. Our findings are presented, and 15 cases from the literature, t
he largest series to date, are discussed. (C) 1996 Academic Press, Inc
.