Tuberous Sclerosis Associated With Renal Angiomyolipoma, Pulmonary Lymphagioleiomyomatosis and Subungual Fibroma

Abstract

We report a case of tuberous sclerosis associated with bilateral renal angiomyolipomas (AMLs), pulmonary lymphangioleiomyomatosis (LAM) and subungual fibroma of hands and feet. A 42-year-old woman who was diagnosed as tuberous sclerosis at the age of 18 complained of left flank pain and abdominal fullness. Bilateral renal AMLs were pointed out when complete examinations were performed for hypertension at the age of 32. She suffered from severe left flank pain and abdominal distension due to the left renal tumor. Left nephrectomy and excision of the renal hilar tumor were performed. The left renal tumor weighed 1120 g, the perirenal space was filled with the tumor. histopathological diagnosis of the left renal tumor and renal hilar tumor was AML. In our case, bilateral pneumothorax appeared, and chest CT scan revealed bilateral multiple pulmonary cysts. Histopathological diagnosis of pulmonary cysts was LAM. Other complications of our case are intracranial calcification and adenoma sebaceum.

Renal Smooth Muscle Tumor with Features of Lymphangioleiomyomatosis

“…LL is a rare hamartomatous disorder composed of varying proportions of smooth muscle and lymphatic vessels that can occur alone [2,3] or rarely in association with tuberous sclerosis [4]. Most LLs occur in the lung [2], but sometimes may appear in the mediastinum and retroperitoneum [3].…”