Chylous Ascites as a Presentation of Lymphangioleiomyomatosis

Abstract

A 35-year-old woman presented to the hospital with a 4-week history of large-volume chylous ascites refractory to paracentesis and new-onset dyspnea. Thoracic computed tomography revealed diffuse pulmonary cystic lesions with pleural effusions, and abdominal computed tomography showed ascites with large bilateral retroperitoneal masses displaying positron emission tomography avidity. Biopsy of the masses demonstrated lymphatic invasion by a perivascular epithelioid cell neoplasm, a smooth muscle tumor. The patient was diagnosed as having the sporadic form of lymphangioleiomyomatosis and was treated with the mammalian target of rapamycin pathway inhibitor sirolumus with clinical improvement.

Lymphangioleiomyomatosis and mTOR inhibitors in real world—a narrative review

“…Of those, the mechanistic target of rapamycin (mTOR) is the essential component for disease control ( 11 , 12 ). Overactivity of mTOR causes excess cellular growth and proliferation, and primes patients to develop the benign tumors characteristic of LAM ( 13 ). Upregulated bioactive molecules such as angiogenic factor vascular endothelial growth factor (VEGF)-A, and lymphangiogenic factor VEGF-C and -D facilitate formation of blood vessels and lymphatic channels, respectively, contributing to access of LAM cells to the main blood stream and lymphatic circulation ( 10 ).…”

Linfangiomatosis en una mujer posmenopáusica: reporte de caso y revisión de literatura

“…La linfangiomatosis (LAM) es una patología rara que afecta principalmente a mujeres en edad reproductiva (McLain et al, 2021), se manifiesta como una neoplasia de origen epitelial en la región perivascular del parénquima pulmonar. Posee un bajo grado de diferenciación con una morfología similar al músculo liso, presenta una tendencia a dañar el parénquima pulmonar mediante la obstrucción del drenaje linfático (Harari et al, 2018).…”