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.
Cagnano
M
M
Institute of Pathology, Soroka Medical Center, Beer-Sheva, Israel.
Benharroch
D
D
Geffen
D B
DB
D005260:Female; D006801:Humans; D008168:Lung / Q000473:pathology*; D008175:Lung Neoplasms / Q000473:pathology*; D008203:Lymphangiomyoma / Q000473:pathology*; D008875:Middle Aged; D009378:Neoplasms, Multiple Primary / Q000473:pathology*; D012983:Soft Tissue Neoplasms / Q000473:pathology*