Primary lymphomas of the female genital tract are rare. Most involve the ce
rvix rather than the uterine corpus. All of those previously reported have
been B-cell lymphomas, with the exception of 1 case report of an endometria
l T-cell lymphoma in a Japanese woman. We report the case of a white woman
from the United States with a diffuse large cell lymphoma of the endometriu
m, characterized as a peripheral T-cell type on the basis of immunophenotyp
ic and molecular probe studies. Staging evaluation revealed tumor limited t
o the endometrium (stage IE). The patient underwent a total abdominal hyste
rectomy, bilateral salpingo-oophorectomy and lymph node dissection mid rece
ived 6 cycles of combination chemotherapy, after which she remained free of
disease at last follow-up of 36 months. Unusual features of this lymphoma
case are discussed, with emphasis on differential diagnosis and speculation
on histogenesis. This case illustrates that while most peripheral T-cell l
ymphomas are widely disseminated at presentation those limited to a single
extranodal site may have a favorable outcome akin to that associated with h
igh-grade extranodal B-cell lymphomas of early stage.