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

Abstract

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

Keywords: Angiomyolipomas; lymphangioleiomyomatosis; mTOR; nephrectomy; pregnancy; tuberous sclerosis complex.

Figure 1

(a) Coronal CT revealing a well-circumscribed heterogenic mass lateral to the right kidney with appearance of a giant angiomyolipoma measuring 129.2 × 51.5 mm. (b) Axial CT appearance of the right renal angiomyolipoma measuring 57.3 × 40.3 mm. Small intraparenchymatous cystic lesions were present.

Figure 2

(a) Coronal CT revealing pulmonary lymphangioleiomyomatosis with a moderate degree of pulmonary cystic affectation. Three lesions in each lung are identified by arrows. (b) Coronal CT revealing a marked resolution of pulmonary cysts after a 6-month treatment with low-dose rapamycin. One lesion in the right lung and three in the left had become imperceptible (arrows and asterisks).

Figure 3

MRI showing the right kidney 2 months after kidney-sparing conservative surgery. (a) Coronal appearance of the right kidney. (b) Axial appearance of the right kidney. Good kidney preservation was apparent.

Figure 4

(a) Axial CT appearance of the right kidney after a 12-month treatment with low-dose rapamycin. (b) Axial MRI appearance of the right kidney after a 12-month treatment with low-dose rapamycin. No angiomyolipomas regrowth was apparent. Only small intraparenchymatous cystic lesions less than 14 mm were present.