Giant bilateral renal angiomyolipomas and lymphangioleiomyomatosis presenting after two successive pregnancies successfully treated with surgery and rapamycin

摘要

We report the case of a 25-year-old woman who presented with abdominal and flank pain withtwo successive pregnancies and was diagnosed of giant bilateral renal AMLs and pulmonaryLAM associated with TSC in the post-partum of her second pregnancy. This case illustratesthat in women with TSC rapid growth from renal AMLs and development of LAM may occurwith successive pregnancies. It also stresses the potential for preservation of renal functiondespite successive bilateral renal surgery of giant AMLs. Moreover, the treatment with a low-doserapamycin may be an option for LAM treatment. Finally, a low-dose rapamycin may be consideredas an adjuvant treatment together to kidney-sparing conservative surgery for renal AMLs