[3 cases of diffuse lymphangioleiomyomatosis of the lung].
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.
Puente Maestú
L
L
Hospital Gregorio Marañón, Servicio de Neumología, Madrid.
Rodríguez González-Moro
J M
JM
de Lucas Ramos
P
P
Tarancón Llorente
M C
MC
Cubillo Marcos
J M
JM
Hernández Fernández
J
J
Monturiol Rodríguez
J M
JM
D000328:Adult; D000368:Aged; D005260:Female; D006801:Humans; D008175:Lung Neoplasms / Q000150:complications / Q000000981:diagnostic imaging / Q000473:pathology / Q000503:physiopathology / Q000628:therapy; D008203:Lymphangiomyoma / Q000150:complications / Q000000981:diagnostic imaging / Q000473:pathology / Q000503:physiopathology / Q000628:therapy; D008232:Lymphoproliferative Disorders / Q000150:complications / Q000000981:diagnostic imaging / Q000503:physiopathology; D008875:Middle Aged; D011859:Radiography
A propósito de tres casos de linfangioleiomiomatosis pulmonar difusa.