Annals of Oncology Advance Access published online on October 25, 2009
Annals of Oncology, doi:10.1093/annonc/mdp454
Subcutaneous ‘lipoma-like’ B-cell lymphoma associated with HCV infection: a new presentation of primary extranodal marginal zone B-cell lymphoma of MALT
1 Pathology Section, Department of Human Pathology
2 Division of Hematology, Fondazione IRCCS Policlinico San Matteo, University of Pavia, Pavia
3 Division of Hematology, Department of Medical Sciences and IRCAD, Amedeo Avogadro University of Eastern Piedmont, Novara
4 Department of Dermatology, University of Milano-Bicocca, Milano
5 Division of Tropical and Infectious Diseases, Fondazione IRCCS Policlinico San Matteo, University of Pavia, Pavia, Italy
* Correspondence to: Dr M. Paulli, Anatomic Pathology Section, Department of Human Pathology, Fondazione IRCCS Policlinico San Matteo, University of Pavia, via Forlanini 14, 27100 Pavia, Italy. Tel: +39-0382501241; Fax: +39-0382525866; E-mail: immunopat{at}smatteo.pv.it
Background: Hepatitis C virus (HCV) infection has been linked to lymphoproliferative disorders. Marginal zone B-cell lymphoma (MZL) represents one of the most frequent lymphoma subtypes associated with HCV infection. We describe an unusual subset of HCV-associated MZL characterized by subcutaneous presentation.
Materials and methods: A series of 12 HCV-positive patients presenting with subcutaneous nodules that revealed lymphoma infiltration at biopsy. Molecular analysis of immunoglobulin heavy chain (IGH) gene rearrangement and FISH investigations for t(11;18)(q21;q21) and t(14;18)(q32;q21) were carried out in nine patients.
Results: The 12 patients (median age 69.5 years), all with positive HCV serology, presented with single or multiple subcutaneous nodules resembling lipomas. Histologically the lesions showed lymphoid infiltrates, consistent with extranodal MZL of mucosa-associated lymphoid tissue (MALT). Functional IGH gene rearrangements were identified in nine tested patients, with somatic mutations in 82%, indicating a histogenesis from germinal center-experienced B cells. The t(11;18) was found in two of nine cases. Staging did not show any other lymphoma localization. In two patients, a response was achieved with antiviral treatment. Extracutaneous spread to MALT sites occurred in a case.
Conclusions: Our observations expand the spectrum of HCV-associated lymphomas to include a subset of extranodal MZL characterized by a novel primary ‘lipoma-like’ subcutaneous presentation and indolent clinical course.
hepatitis C virus, IGH rearrangement, MALT, marginal zone lymphoma, subcutaneous tissue
Received for publication November 25, 2008. Revision received June 23, 2009. Accepted for publication August 10, 2009.