Primary B-cell lymphoma of the tongue in a patient with systemic sclerosis

Abstract

We report a patient with systemic sclerosis who had the unusual development of a large-cell B-cell lymphoma of the base of his tongue. Patients with systemic sclerosis display an increased incidence of malignant neoplasms, particularly, breast and lung carcinomas [Arthritis. Rheum. 36 (1993) 460; Ann. Rheum. Dis. 52 (1993) 531]. The description of this case adds further evidence to the possible interrelationship of systemic sclerosis and the development of malignant neoplasms. A careful review of the English medical literature disclosed that B-cell lymphoma of the tongue in a patient with systemic sclerosis is an association which has not been described previously.

Introduction

Inflammatory connective tissue disorders such as dermatomyositis, polymyositis and Sjögren's syndrome have been linked to an increased prevalence of malignant neoplasms.1, 2, 3 Early epidemiological studies in large cohorts of systemic sclerosis patients did not show an increased incidence of malignant neoplasias⁴ although more recent studies described a statistically significant excess of lung and breast neoplasms in patients with systemic sclerosis; the discrepancy being possibly related to improved screening for cancer.5, 6 In Sjögren's syndrome patients this increased risk appears to be almost exclusively related to the development of lymphoproliferative malignancies, and more specifically, to non-Hodgkin's lymphoma of the salivary glands.² Increased risk of developing lymphoproliferative malignancies was also described in patients with rheumatoid arthritis, although in these patients it appears that either the use of certain disease modifying anti-rheumatic agents such as methotrexate or the presence of an undiagnosed secondary Sjögren's syndrome may be responsible.³ Lymphoproliferative disorders have been associated less frequently with systemic sclerosis and in large epidemiological studies the absolute incidence of these malignant neoplasms does not appear to be different from that in the general population.4, 5, 6

We report herein a patient with systemic sclerosis, who 2 years after this diagnosis, developed an expanding oral mass found to be a primary large-cell B-cell lymphoma of the tongue. This report describes this previously unreported rare association.