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Lymphangiomyomatosis syndrome with hyperparathyroidism. A case report
Author(s) -
Kreisman Harvey,
Robitaille Yves,
Dionne G. Paul,
Palayew Max J.
Publication year - 1978
Publication title -
cancer
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 3.052
H-Index - 304
eISSN - 1097-0142
pISSN - 0008-543X
DOI - 10.1002/1097-0142(197807)42:1<364::aid-cncr2820420155>3.0.co;2-m
Subject(s) - medicine , thoracic duct , lesion , hyperparathyroidism , pathological , tuberous sclerosis , parathyroid adenoma , adenoma , pathology , lymph , surgery
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.