[Pulmonary lymphangioleiomyomatosis. A disease which may have a benign progression]. / Linfangioleiomiomatosis pulmonar. Una enfermedad que puede tener un curso benigno.

Pulmonary lymphangioleiomyomatosis (PLLM) is characterized by a benign proliferation of the smooth muscle around lymphatics, venulas and bronchiolus. It is usually fatal within 1 to 9 years. We present a case which remained with scarce symptoms despite receiving no treatment after at least 9 years of evolution of the disease. We highlight the clinical manifestations of the disease and the relevance of pulmonary biopsia with thoracotomy in order to establish the diagnosis.