Autoimmune Diseases In Benign And Malignant Thymoma

Malignant thymomas, as opposed to benign thymomas, constitute a minority of all epithelial tumors of the thymus. These are classified into type I: tumors which are locally invasive, tend to recur and occasionally metastasize widely, but cannot be differentiated cytologically from their benign counterparts [21]; and type II: tumors that are also identified as thymic carcinoma since they exhibit cytological and histological features of malignancy identical to carcinomas arising in extrathymic sites [22],

We recently discussed the difference between the association of autoimmune diseases with benign and invasive thymomas, as we have reported about 6 patients with autoimmune diseases and malignant thymoma [23]. They included 5 patients with type I malignant thymoma and 1 patient with type II malignant thymoma, and also the first reported cases of Graves' disease with thymoma and of Sjogren's syndrome with thymic carcinoma [23]. With respect to the association of malignant thymoma with autoimmune diseases, there is a significant difference between types II and I. Malignant thymoma type I (invasive thymoma) was often described in some case series to occur in patients with MG, the autoimmune disease most frequently found in thymomatous patients (summarized in [24]). In addition, pemphigus, SLE and PRCA were also reported in patients with type I malignant thymoma. As opposed to malignant thymoma type I, there are only few case reports of autoimmune diseases with thymic carcinoma (malignant thymoma type II), usually of MG. The difference between both types in the association with autoimmune conditions is related to their differences in histology. Hence, whether thymic carcinoma is histogenetically distinct from thymoma, or it is a poorly differentiated variant of thymoma, its cellular and environmental features may not be usually sufficient to create a proper stimulus for autoimmunity emergence.

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