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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 Sjogren'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 [kappa] 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 plasma cytoma.
Primary breast non-Hodgkin's lymphomas, which comprise less than 0.5% of all malignant tumours of the breast, are mainly of the large B cell type. (1) Marginal zone B cell lymphomas (MZL) are exceedingly rare in the breast. Many patients with MZL have a history of chronic inflammation or autoimmune disorders. Patients with Sjogren's syndrome have a 44 fold increased risk of developing lymphoma. (2) Mammary tissue amyloidosis is also rare and has never been described predominantly in ducts. A highly unusual mammary MZL with ductal amyloidosis admixed with corpora amylacea in the setting of Sjogren's syndrome is described.
"Patients with Sjogren's syndrome have a 44 fold increased risk of developing lymphoma"
A 37 year old woman with a 10 year history of primary Sjogren's syndrome presented with left axilary lymphadenopathy and …