Lymphangioleiomyomatosis in a Young Male

Abstract

Lymphangioleiomyomatosis (LAM) is a rare multisystem disorder, predominantly affecting females in the reproductive age group, characterised by multiple cysts in lungs. A 15-year-old male was admitted in our institute for right sided renal mass for which he underwent right nephrectomy and was referred to our department for bilateral spontaneous pneumothoraces. CT thorax was done which showed bilateral pneumothoraces with multiple thin walled cysts and subtle ground glass opacities in both lung fields suggestive of changes of LAM. LAM is rarely seen in males, however if present ER/PR status can help in modifying the management. This is the youngest case of LAM reported in a male worldwide.

Keywords

• lungs
• nephrectomy
• pneumothorax