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Rev Clin Esp. 1992 Jul;191(3):144-7.

[Pulmonary lymphangiomatosis associated with renal angiomyolipoma. Presentation of a case and review of the literature].

[Article in Spanish]

Author information

1
Servicios de Neumología y de Anatomía Patológica, Hospital del Aire, Madrid.

Abstract

The case concerned is one of lymphangiomyomatosis (LAM) in a woman aged 39, that after a episode of spontaneous pneumothorax was subject to medical research for progressive dyspnea of medium efforts and bloodstained sputum. High resolution CT showed numerous thin-walled cystic airspaces of varying sizes distributed diffusely throughout the lungs. The abdominal CT and ultrasound developed multiple right renal angiomyolipomas. The histopathological study of the sample obtained by open lung biopsy was conclusive of LAM. The main feature of this rare disease, which occurs almost exclusively in women of reproductive age, is the abnormal proliferation of immature smooth muscle at the level of the distal airway, small blood vessels and lung lymphatic system, including sometimes the mediastinal and retroperitoneal lymphatic system. The clinical, functional, radiological and differential diagnostic aspects with entities histopathologically similarities are discussed and the therapeutic possibilities are reviewed.

PMID:
1502405
[Indexed for MEDLINE]

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