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 die...

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Published inRevista clínica espanõla Vol. 186; no. 7; p. 335
Main Authors 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
Format Journal Article
LanguageSpanish
Published Spain 01.04.1990
Subjects
Adult
Aged
Female
Humans
Lung Neoplasms - complications
Lung Neoplasms - diagnostic imaging
Lung Neoplasms - pathology
Lung Neoplasms - physiopathology
Lung Neoplasms - therapy
Lymphangiomyoma - complications
Lymphangiomyoma - diagnostic imaging
Lymphangiomyoma - pathology
Lymphangiomyoma - physiopathology
Lymphangiomyoma - therapy
Lymphoproliferative Disorders - complications
Lymphoproliferative Disorders - diagnostic imaging
Lymphoproliferative Disorders - physiopathology
Middle Aged
Radiography
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Summary: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.
ISSN:0014-2565