Tuberous sclerosis: Renal angiomyolipoma in association with pulmonary lymphangioleiomyomatosis

Year 1998, Volume: 11 Issue: 2, 96 - 100, 01.03.1998

B.b. Ceyhan E. Akoğlu T. Celikel Ç. Özener N. Kodallı M. Sungur

Abstract

Tuberous sclerosis is a multiple system disease, involving the brain, skin, kidneys, heart and other visceral organs. We report a patient with bilateral renal angiomyolipomas, diffuse pulmonary cysts, and skin adenoma sebaceum. Following the discovery of a renal mass 8 years previously, a left nephrectomy was performed, histopathological examination revealing increased kidney fatty tissue. Exertional dyspnea, spontaneous pneumothorax history, and bilateral multiple cystic lesions in computed tomography of thorax wore consistent with pulmonary lymphangioleiomyomatosis, an unusual entity known to coexist with tuberous sclerosis. Over the last few years, pulmonary symptoms have worsened and the right kidney has enlarged secondary to angiomyolipomatosis.

We want to report this case since it describes an association between pulmonary lymphangiolei myomatosis and renal angiomyomatosis which is not well documented

Keywords

Renal angiomyolipoma, tuberous sclerosis, lymphangioleiomyomatosis, adenoma sebaceum

References

• Dwyer JM, Dickie JB, Qarvan J. Pulmonary tuberous sclerosis, report of three patients and a review of the literature Q J Med 1971 ;40:115- 125.