Nephrotic syndrome revealing malignant thymoma
Schillinger, F.; Milcent, T.; Wolf, C.; Gulino, R.; Montagnac, R.
Presse Medicale 27(2): 60-63
1998
ISSN/ISBN: 0755-4982 PMID: 9768051 Document Number: 490254
A well-established manifestation of neoplastic disease, nephrotic syndrome is infrequently associated with thymoma. Only 18 cases have been reported in the literature. A 65-year-old man and a 60-year-old woman were seen for nephrotic syndrome. Minimal change renal disease was observed in the first patient whose nephrotic syndrome was steroid resistant. The second patient had membranous glomerulopathy and pure red cell aplasia. In both cases, nephrotic syndrome revealed thymoma. The histological lesions in 17 of the 18 biopsied cases reported in the literature were minimal change in 10, focal segmental glomerulonephritis in 4, proliferative glomerulonephritis in 2, and membranous glomerulopathy in only one. The outcome of the nephrotic syndrome was dependent on the success of the thymoma treatment. Some patients responded to steroid and immunosuppressive agents. Pure red cell aplasia is uncommon and prognosis is poor. It can be successfully treated with cyclosporin A as in our second case.