Lymphangiomyomatosis syndrome with hyperparathyroidism: a case report.
A 48-year-old woman presented with the classical clinicopathological features of the lymphangiomyomatosis syndrome. After a three year stable period, there was the onset of a rapidly progressive downhill course unresponsive to dietary, bronchodilator and corticosteroid therapy. Pathological findings were characterized by widespread pulmonary, thoracic duct and lymph node involvement. There was a mediastinal lymphangiomyoma growing within the distal thoracic duct, and a similar lesion within the left kidney which could clinically mimic an angiomyolipoma. Comments are also made on the finding of a parathyroid adenoma. The physiopathology and possible resemblance to "formes frustes" of tuberous sclerosis are discussed.
D000236:Adenoma / Q000473:pathology*; D000402:Airway Obstruction / Q000473:pathology; D002916:Chylothorax / Q000473:pathology; D003937:Diagnosis, Differential; D005260:Female; D006222:Hamartoma / Q000473:pathology; D006391:Hemangioma / Q000473:pathology; D006801:Humans; D007680:Kidney Neoplasms / Q000473:pathology; D008067:Lipoma / Q000473:pathology; D008175:Lung Neoplasms / Q000473:pathology*; D008206:Lymphatic Diseases / Q000473:pathology*; D008854:Microscopy, Electron; D008875:Middle Aged; D009378:Neoplasms, Multiple Primary / Q000473:pathology*; D010282:Parathyroid Neoplasms / Q000473:pathology*; D013577:Syndrome; D013897:Thoracic Duct / Q000473:pathology; D014402:Tuberous Sclerosis / Q000473:pathology