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pubmed:abstractText |
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.
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