Objective: Lymphomas with signet ring cell features are
rare, as is uterine dissemination of lymphomas. We report
an exceptional case of a uterine tumor combining these two
characteristics.
Method: A 61-year-old female was diagnosed in 2004 with
localized nodal grade 2 follicular lymphoma (stage IA). She
received local radiation therapy, experienced total remission,
and did well until 2009 when a systematic CT scan
evidenced a pelvic anterior-lateral mass. Total enlarged
hysterectomy was performed.
Results: The anterior uterine wall contained a 4.8-cm fish
flesh well-delineated mass corresponding to a mostly
diffuse and focally nodular proliferation of medium to
large cells with extensive signet ring cell changes. Tumor
cells were CD20-, CD10-, Bcl2-, and Bcl6-positive with a
low proliferation rate (<10-15%); CD21 underlined a focal
follicular architecture. The vacuoles were PAS-negative and
did not stain for immunoglobulin; ultrastructural analysis
revealed nonspecific degenerative vacuoles. No lymph
nodes were identified isolated from the surgical specimen.
The tumor was considered as a secondary localization of
the systemic follicular lymphoma, though no signet ring
cells were evidenced in the cervical lymph node biopsy
(reviewed). Follow-up showed retroperitoneal tissue infiltration
(PET-CT) and normal medullar biopsy. She recently
started R-CHOP chemotherapy.
Conclusion: This case illustrates both an unusual site of
dissemination and challenging cytological characteristics in
a follicular lymphoma. The signet ring cell changes
challenged the adequate classification of this lymphoma as
either a large B cell or a follicular B cell lymphoma.