Rare primary extranodal lymphomas: diffuse large B-cell lymphomas of the genital tract

Péter Rajnics (1) , Judit Demeter (2) , Judit Csomor (3) , László Krenács (4) , László Pajor (5) , Balázs Kollár (1) , Zsuzsanna Kertész (1) , Miklós Egyed (1)

1 Department of Internal Medicine, Division of Hematology
2 First Department of Internal Medicine, Division of Hematology
3 Department of Pathology
4 Laboratory of Tumor Pathology and Molecular Diagnostics
5 Department of Pathology

Primary non-Hodgkin's lymphoma (NHL) of the genital tract is a rare entity. Etiology and pathogenesis of these NHLs are unknown, although there might be a possible association between chronic inflammation and lymphomas. The most common histological subtype is the diffuse large B-cell lymphoma. We report two cases of uterine lymphoma and one case of prostate lymphoma in this paper. The symptoms and the differential diagnosis are also discussed. Because of the low incidence, there is no widely accepted consensus on its treatment. We demonstrate that the rituximab and CHOP (cyclophosphamide, doxorubicin, vincristine, and prednisone; R-CHOP) chemoimmunotherapy is a good and tolerable treatment option in all cases. The two young patients are disease-free nowadays; the older patient with poor prognostic histological-type lymphoma relapsed in a short time and died after second relapse. A multicenter analysis is necessary to evaluate the long-term results of chemoimmunotherapy in these rare extranodal lymphoma entities.

Mots clés: Primary extranodal lymphoma; Diffuse large B-cell lymphoma; Uterine lymphoma; Prostate lymphoma; Rituximab-based therapy

Péter Rajnics, Judit Demeter, Judit Csomor, László Krenács, László Pajor, et al.. Rare primary extranodal lymphomas: diffuse large B-cell lymphomas of the genital tract. Annals of Hematology, 2009, 88 (12), pp.1223-1228. ⟨10.1007/s00277-009-0741-7⟩. ⟨hal-00535052⟩