title: 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.
authors: Servicio de Nefrología, Hospital Universitario La Paz, IdiPAZ, 28046 Madrid, Spain. cpeces@varnetmail.com
authors: Ramón
authors: R
authors: Peces
authors: Emilio
authors: E
authors: Cuesta-López
authors: Carlos
authors: C
authors: Peces
authors: Rafael
authors: R
authors: Selgas
mesh_terms: D000328:Adult; D018207:Angiomyolipoma / Q000150:complications / Q000188:drug therapy / Q000601:surgery / Q000628:therapy*; D005260:Female; D006801:Humans; D007680:Kidney Neoplasms / Q000150:complications / Q000188:drug therapy / Q000601:surgery / Q000628:therapy*; D018192:Lymphangioleiomyomatosis / Q000150:complications / Q000188:drug therapy / Q000601:surgery / Q000628:therapy*; D011247:Pregnancy; D011252:Pregnancy Complications, Neoplastic / Q000188:drug therapy / Q000601:surgery / Q000628:therapy*; D020123:Sirolimus / Q000627:therapeutic use*; D014057:Tomography, X-Ray Computed; D016896:Treatment Outcome
chemical_list: D020123:Sirolimus