[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.
Hospital Gregorio Marañón, Servicio de Neumología, Madrid.
L
L
Puente Maestú
J M
JM
Rodríguez González-Moro
P
P
de Lucas Ramos
M C
MC
Tarancón Llorente
J M
JM
Cubillo Marcos
J
J
Hernández Fernández
J M
JM
Monturiol Rodríguez
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.