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Annals of Oncology Advance Access published online on July 1, 2009

Annals of Oncology, doi:10.1093/annonc/mdp238
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© The Author 2009. Published by Oxford University Press on behalf of the European Society for Medical Oncology. All rights reserved. For permissions, please email: journals.permissions@oxfordjournals.org

Primary follicular and marginal-zone lymphoma of the breast: clinical features, prognostic factors and outcome: a study by the International Extranodal Lymphoma Study Group

G. Martinelli1,*, G. Ryan2, J. F. Seymour3, L. Nassi1, S. Steffanoni1, A. Alietti1, L. Calabrese1, G. Pruneri4, L. Santoro5, M. Kuper-Hommel6, R. Tsang7, P. L. Zinzani8, A. Taghian9, E. Zucca10 and F. Cavalli10

1 Division of Haematology, European Institute of Oncology, Milan, Italy
2 Division of Radiation Oncology, Peter MacCallum Cancer Centre
3 Department of Haematology, Peter MacCallum Cancer Centre and University of Melbourne, Melbourne, Australia
Divisions of
4 Pathology and Laboratory Medicine
5 Statistical Analysis, European Institute of Oncology, Milan, Italy
6 Department of Internal Medicine, Bernhoven Hospital, Oss, The Netherlands
7 Department of Radiation Oncology, Princess Margaret Hospital, University Health Network, Toronto, Canada
8 Institute of Haematology and Medical Oncology, Policlinico Sant'Orsola, Bologna, Italy
9 Department of Radiation Oncology, Massachusetts General Hospital, Boston, MA, USA
10 Department of Medical Oncology, Oncology Institute of Southern Switzerland, Bellinzona, Switzerland

* Correspondence to: Dr. G. Martinelli, Division of Haematology, European Institute of Oncology, Via Ripamonti 435, I-20141 Milan, Italy. Tel: +39-02-57489538; Fax: +39-02-94399219; E-mail: giovanni.martinelli{at}ieo.it

Background: Primary breast lymphoma (PBL) of low-grade histology is a rare disease. This multicentric retrospective study was carried out to determine clinical features, prognosis and relapse.

Patients and methods: Patients with histologically proven, previously untreated follicular or marginal-zone PBL (MZL PBL) diagnosed from 1980 to 2003 were included in the study. Major end points were progression-free survival (PFS), overall survival (OS) and potential prognostic factors.

Results: We collected data on 60 cases of PBL [36 follicular and 24 marginal-zone lymphoma (MZL)]. Stage was IE or IIE in 57 patients and IVE in three patients due to bilateral breast involvement. Surgery, chemotherapy and radiotherapy (RT), alone or in combination, were used as first-line treatments in 67%, 42% and 52% of patients, respectively. Overall response rate was 98%, with a 93% complete response rate. Five-year PFS were 56% for MZL and 49% for follicular PBL (P = 0.62). Relapses were mostly in distant sites (18 of 23 cases); no patients relapsed within RT fields.

Conclusions: Our data showed an indolent behaviour of MZL PBL, comparable to other primary extranodal MZL. Conversely, patients with follicular PBL had inferior PFS and OS when compared with limited-stage nodal follicular non-Hodgkin's lymphomas, suggesting an adverse prognostic role of primary breast localisation in this histological subgroup.

breast malignancies, follicular lymphomas, MZLs, primary extranodal lymphomas

Received for publication March 20, 2009. Accepted for publication March 23, 2009.


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