Pulmonary lymphangioleiomyomatosis. Report of a case with associated multiple soft-tissue tumors

We describe a 55-year-old woman with pulmonary lymphangioleiomyomatosis. The patient died of respiratory failure 9 years after diagnosis. An autopsy confirmed the diagnosis and disclosed multiple soft-tissue tumors, including a large solitary fibrous tumor of the lung, a huge cavernous hemangioma of the liver, a meningioma of the right pontocerebellar angle, and a focus of nodular stromal hyperplasia of the ovary. In addition, endocrine tumors, including a papillary carcinoma of the thyroid gland and a parathyroid adenoma, were also found. There was no evidence of tuberous sclerosis or angiomyolipoma of the kidney. The connection between pulmonary lymphangioleiomyomatosis and tuberous sclerosis is discussed.