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

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 g...

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Detalles Bibliográficos
Autores: Peces, Ramón, Cuesta López, Emilio, Peces, Carlos, Selgas Gutiérrez, Rafael
Tipo de recurso: artículo
Fecha de publicación:2011
País:España
Institución:Universidad Autónoma de Madrid
Repositorio:Biblos-e Archivo. Repositorio Institucional de la UAM
Idioma:inglés
OAI Identifier:oai:repositorio.uam.es:10486/668937
Acceso en línea:http://hdl.handle.net/10486/668937
https://dx.doi.org/10.1100/2011/263137
Access Level:acceso abierto
Palabra clave:Angiomyolipomas
Lymphangioleiomyomatosis
mTOR
Nephrectomy
Pregnancy
Tuberous sclerosis complex
Medicina
Descripción
Sumario: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