Image Description
Patients with pulmonary involvement in lymphangiomyomatosis usually experience progressive decline in lung function. Some patients respond to progesterone or anti-estrogen therapy. Sirolimus therapy has been used recently and it appears to improve the quality of life and slow down pulmonary insufficiency. The definitive treatment is heart-lung transplantation; although recurrences have been noted in the transplanted lung. The 10-yr mortality is reported to be between 30% and 70%.
About the Disease
PEComatous tumors arise from perivascular epithelioid cells and can present in the lung in following forms: 1) Lymphangiomyomatosis; 2) Clear cell sugar tumor of lung; 3) PEComatosis with overlapping features between lymphangiomyomatosis and PEComa. Other tumors in this family include renal and extrarenal angiomyolipoma, primary extrapulmonary sugar tumor, clear cell myomelanocytic tumor, and abdominopelvic sarcoma of perivascular epithelioid cells. PEComatous tumors arise in a variety of sites and are tied together through their association with the tuberous sclerosis complex (and the mutations in tuberous sclerosis genes TSC1 and TSC2). References:Goldblum, J.R., Folpe, A.L., Weiss, S.L. (2014). Enzinger & Weisss Soft Tissue Tumors, 6th Ed. Philadelphia, PA: Elsevier Saunders. Travis, W.D., et al. (2015). WHO Classification of Tumors of the Lung, Pleura, Thymus and Heart, 4th Ed. IARC: Lyon, France.