CASE REPORT

Low grade marginal zone B cell lymphoma of the breast associated with localised amyloidosis and corpora amylacea in a woman with long standing primary Sjögren’s syndrome

M Kambouchner¹, P Godmer², L Guillevin², M Raphaël³, D Droz⁴, A Martin¹

¹ Department of Pathology, Avicenne Hospital, 125 Route de Stalingrad, Bobigny 93009 Cedex, France
² Department of Internal Medicine, Avicenne Hospital
³ Department of Haematology, Avicenne Hospital
⁴ Department of Pathology, Saint-Louis Hospital, Paris, France

Correspondence to:
Correspondence to:
Dr Kambouchner, Service d’Anatomie Pathologique, Hopital Avicenne, 125 Route de Stalingrad, Bobigny 93009 Cedex, France;
marianne.kambouchner{at}avc.ap-hop-paris.fr

Primary low grade marginal zone B cell lymphoma (MZL) of the breast and localised mammary amyloidosis are exceedingly rare entities. This report describes the case of a woman with long standing Sjögren’s syndrome presenting with asymptomatic MZL of the breast showing plasmacytic differentiation, associated with local ductular amyloidosis. The lesion was discovered incidentally in breast tissue resected for microcalcifications. Immunohistochemistry revealed light chain restriction, supporting the neoplastic nature of the infiltrate. A retrospective molecular study of the salivary gland biopsy showed a B cell clone. This is the first report of the association of human mammary ductular amyloidosis with cartwheel shaped material identical to corpora amylacea, usually seen in brain, lung, and prostate, but unknown in the human breast. The excellent outcome without treatment seen in this patient further emphasises the need to distinguish between MZL with plasmacytic differentiation and extramedullary plasmacytoma.

Keywords: marginal zone B cell lymphoma; breast; amyloidosis; corpora amylacea; ultrastructure; Sjögren’s syndrome

Abbreviations: MZL, marginal zone B cell lymphoma; PAS, periodic acid Schiff; PBS, phosphate buffered saline

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