We describe three cases of primary low-grade B-cell lymphoma of the en
dometrium and contrast the histological, immunohistochemical, and mole
cular features with two examples of benign endometrial lymphoid infilt
rates. The first case was an incidental finding in a curettage specime
n, confirmed on a subsequent hysterectomy. The other two cases of lymp
homa were incidental findings on hysterectomy procedures performed for
prolapse and cervical dysplasia, respectively. All three lymphomas oc
curred in patients in their sixties; none formed gross tumors. Histolo
gic examination revealed lymphoid nodules adjacent to endometrial glan
ds. The lymphoid cells showed mild nuclear enlargement and slight irre
gularities of the nuclear contour. None of the three patients had evid
ence of disease outside the endometrium by physical examination, bone
marrow biopsy, or sampling of pelvic lymph nodes. Immunohistochemistry
demonstrated a B-cell phenotype of the lymphoid cells (CD20 positive,
CD79a positive) with aberrant coexpression of the T-cell-associated m
arker CD43. Polymer ase chain reaction (PCR) amplification of the VDJ
region of the immunoglobulin heavy-chain was performed on DNA isolated
from paraffin sections. These studies demonstrated a clonal prolifera
tion of B-lymphocytes in two cases. In the third case, a faint band wa
s found superimposed on a background smear, suggesting the presence of
a B-cell clone. In contrast, the two examples of histologically benig
n lymphoid aggregates of the endometrium consisted predominantly of T
cells with rare B-lymphocytes; there was no evidence of coexpression o
f CD43 by B-cells. The PCR amplification from the benign lymphoid aggr
egates did not support a clonal process, Primary lymphoid neoplasms of
the endometrium are rare, and all cases described so far have been hi
gh-stage, high-grade neoplasms. To our knowledge, this is the first re
port of primary low-grade B-cell lymphoma of the endometrium, presumab
ly arising from endometrial lymphoid tissue.