3 cases of diffuse lymphangioleiomyomatosis of the lung
Puente Maestú, L.; Rodríguez González-Moro, J.M.; de Lucas Ramos, P.; Tarancón Llorente, M.C.; Cubillo Marcos, J.M.; Hernández Fernández, J.; Monturiol Rodríguez, J.M.
Revista Clinica Espanola 186(7): 335-340
1990
ISSN/ISBN: 0014-2565 PMID: 2203120 Document Number: 360064
Three new cases of diffuse pulmonary lymphangioleiomyomatosis are presented. Case number 1 is a female patient who presented repeated pneumotorax and dyspnea without evidence by CT scan and gynecological ultrasound of extrathoracic lesions. This patient did not respond to medroxiprogesterone and died 5 years after the initial diagnosis having suffered chronic, severe, global respiratory failure for 4 years. Case number 2 is a female patient who presented dyspnea, chyloptysis and chylothorax, with iliac, paraaortic and mediastinic lymphangioleiomyomas. The last time she was seen, she was still alive after 6 years without treatment. Case number 3 presented lymphangioleiomyomatosis associated to undifferentiated breast carcinoma. The evolution was apparently slow probably because it was diagnosed 14 years after menopause, and died due to a relapse of the neoplasia. All three patients had radiographic images and respiratory functional studies characteristic of this disease and diagnosis was confirmed by biopsy. The literature is reviewed.