A Case of Pulmonary Lymphangioleiomyomatosjs Associated with Thberous Sclerosis and Renal Angiomyolipoma.

Baik, Jung Min; Hong, Han Ki; Oh, Young Bae; Lee, Sang Moo; Park, Man Sil; Yoo, Tak Keun; Ko, Eun Joo; Kim, Eun Kyung

doi:1997.44.5.1184

Tuberc Respir Dis > Volume 44(5); 1997 > Article

Case Report

Tuberculosis and Respiratory Diseases 1997;44(5):1184-1193.
DOI: https://doi.org/10.4046/trd.1997.44.5.1184 Published online October 1, 1997.

A Case of Pulmonary Lymphangioleiomyomatosjs Associated with Thberous Sclerosis and Renal Angiomyolipoma.

Jung Min Baik, Han Ki Hong, Young Bae Oh, Sang Moo Lee, Man Sil Park, Tak Keun Yoo, Eun Joo Ko, Eun Kyung Kim

¹Department of Internal Medical Nowon Eulji Medical Center, Seoul, Korea.
²Department of Chest Surgery Nowon Eulji Medical Center, Seoul, Korea.
³Department of Uroloy Nowon Eulji Medical Center, Seoul, Korea.
⁴Department of Radiology Nowon Eulji Medical Center, Seoul, Korea.
⁵Department of Anatomical Pathology Nowon Eulji Medical Center, Seoul, Korea.

Abstract

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.

Key Words: Tuberous sclerosis, Pulmonary lymphangioleiomyomatosis