European Journal of Biomedical and Pharmaceutical Sciences

A RARE CASE OF CYSTIC LUNG DISEASE

Dr. Pulkit Gupta*, Dr. G. N. Srivastava, Dr. Anbarasan Thiru

ABSTRACT

Lymphangioleiomyomatosis (LAM) is a multisystem disorder, predominantlyaffecting post and premenopausal women, which is characterized by cysticlung lesions, abdominal angiomyolipomas (AML) and lymphaticabnormalities, for example, lymphatic tumors, chylous effusions.[1–5] These pathologic features are caused by the proliferation of a neoplasticsmooth muscle–like LAM cell that also has characteristicsof melanocytes.[6] Inherited and sporadic forms of LAM have beendescribed. Sporadic LAM is caused by somatic mutations in anunknown susceptible cell of the tuberous sclerosis complex 2(TSC2) gene.[7,8] Sporadic LAM is an uncommon disease occurringin approximately 4.9/1,000,000 women.[9] LAM also occurs in TSC, an autosomal dominantdisorder resulting from germline mutations in the TSC1 or TSC2genes that is characterized by widespread hamartomas in severalorgans including the brain, heart, skin, kidney, eyes, lung, and liver,and occurs in 1 of 6000 live births.[10]

Keywords: Lymphatic tumors, chylous effusions.