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The lymphangiomyomatosis syndrome
Author(s) -
Frack Michael D.,
Simon Leslie,
Dawson Bruce H.
Publication year - 1968
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(196808)22:2<428::aid-cncr2820220221>3.0.co;2-q
Subject(s) - medicine , chylothorax , thoracic duct , chylous ascites , lesion , lymphatic system , pneumothorax , lymphedema , tuberous sclerosis , lymph duct , lymphatic vessel , surgery , radiology , anatomy , pathology , cancer , breast cancer , metastasis
This is a case report of a 40‐year‐old woman with progressively worsening dyspnea culminating in chylothorax and pneumothorax. Chylous ascites, lymphedema of the left lower extremity and chyluria later developed and death ensued from uncontrollable loss of chylous fluid. Lymphangiography revealed a lesion extending from the left inguinal region to the lower para‐aortic zone. Biopsy showed the classic histologic features of lymphangiomyoma 1 and the histologically benign lesion was noted in its earliest stages as a proliferation of the smooth muscle in the walls of lymphatic vessels. This is the twenty‐first acceptable reported case of the condition which occurs almost exclusively in females, frequently presents with progressively worsening dyspnea, frequently involves the thoracic duct, is nearly always associated with chylothorax and sometimes pneumothorax, is frequently associated with a particular pulmonary pathology and may be related to the tuberous sclerosis complex. It is suggested that it be regarded as a syndrome designated by the name lymphangiomyomatosis.