RESUMO
Lymphangioleiomyomatosis(LAM) is rare and essentially limited to women in the reproductive ages. A 39-year-old female was admitted due to progressive exerUional dyspnea and intermittent productive cough. Chest PA showed marked hyperinflation of the lung associated with a diffuse reticulo-nodular pattern. High resolution CT scan of the thorax demonstrated that diffusely scattered thin-walled cysts were distributed throughout the bilateral lung fields. Abdominal CT scan showed variable sized multiple angiomyolipoma of both kidney. By open lung biopsy, she was diagnosed as pulmonary LAM associated with Tuberous sclerosis and renal Angiomyolipoma. We present the case and discuss the connection between pulmonary LAM and Tuberous sclerosis.
Assuntos
Adulto , Feminino , Humanos , Angiomiolipoma , Biópsia , Tosse , Dispneia , Rim , Pulmão , Esclerose , Tórax , Tomografia Computadorizada por Raios X , Esclerose TuberosaRESUMO
Primary Pulmonary leiomyosarcomas are rare tumors. These tumors may arise at any level of the lung in which smooth muscle fibers are found. The highest incidence of sarcoma is during the fourth decade whereas for bronchogenic carcinoma the maximum incidence is during the sixth and seventh decades. Leiomyosarcomas are aggressive and progressive. Treatment is mainly surgical. The chemotherapy is ineffective and the effectiveness of radiotherapy depends on the total dose of irradiation. Prognosis and significant survival rate are related to the size of the lesion. We report one patient with primary pulmonary leiomyosarcoma involving the right lower lung.