title: Pulmonary lymphangioleiomyomatosis. Report of a case with associated multiple soft-tissue tumors.
abstract: 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.
authors.affiliation: Institute of Pathology, Soroka Medical Center, Beer-Sheva, Israel.
authors.forename: M
authors.initials: M
authors.lastname: Cagnano
authors.forename: D
authors.initials: D
authors.lastname: Benharroch
authors.forename: D B
authors.initials: DB
authors.lastname: Geffen
mesh_terms: 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*