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Vol. 56. Issue 3.
Pages 247-250 (1 March 2002)
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Vol. 56. Issue 3.
Pages 247-250 (1 March 2002)
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Tumor de Wilms bilateral metacrónico
Metachronous Bilateral Wilms& Tumor
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M. Mambié Meléndeza,
Corresponding author
mariannamambie@hotmail.com

Correspondencia: Dra. M. Mambié Meléndez. Robert Graves, 59, bjos. 07015 Palma de Mallorca. Baleares.
, M. Guibelalde del Castilloa, N. Nieto del Rincóna, D. Rodrigo Jiméneza, A. Femenia Reusb, J.M. Román Piñanaa
a Servicio de Pediatría.
b Servicio de Cirugía Pediátrica. Hospital Universitario Son Dureta. Palma de Mallorca. Baleares.
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La incidencia del tumor de Wilms bilateral es entre el 5 y 10 % de los casos de nefroblastoma. La forma metacrónica representa el 2-3 %. El 96,2 % de las formas metacrónicas aparecen durante los primeros 5 años tras el tumor primario. Las malformaciones asociadas son más frecuentes en las formas bilaterales. La aparición de un tumor metacrónico constituye una dificultad terapéutica. Se describe el caso de una niña de 11 años con hemihipertrofia izquierda diagnosticada de recidiva metacrónica de tumor de Wilms tras 7 años del primer diagnóstico. Recibió 5 ciclos de quimioterapia preoperatoria. Se realizó tumerectomía. Por complicación posquirúrgica se realizó nefrectomía del único riñón. La paciente se encuentra en insuficiencia renal crónica por su condición de anéfrica, dependiente de hemodiálisis. Se continuó el tratamiento posquirúrgico con carboplatino y etopósido. Actualmente se encuentra en remisión completa. Es excepcional la presentación de las formas metacrónicas del tumor de Wilms después de los primeros 5 años del tumor primitivo. Cuando aparece el tumor contralateral la quimioterapia debe mantenerse hasta conseguir la reducción del tamaño tumoral para poder preservar la función renal y evitar la diálisis. En los casos de insuficiencia renal crónica secundaria a una nefrectomía bilateral la elección de los quimioterápicos efectivos y el conocimiento de la farmacocinética y farmacodinámica de éstos hace posible continuar el tratamiento en estos pacientes con el soporte adecuado de hemodiálisis.

Palabras clave:
Wilms
Nefroblastoma
Bilateral
Metacrónico
Quimio-terapia
Hemodiálisis
Insuficiencia renal
Hemihipertro-fia
Anaplasia

Wilms' tumor occurs in 5-10 % of all cases of nephroblastoma. The metachronous form represents 2-3% of cases. Most (96.2 %) metachronous tumors appear within the first 5 years of the primary tumor. Associated malformations are more common in bilateral cases. Metachronous tumors are a therapeutic challenge. We describe the case of an 11-year-old girl with left hemihypertrophy. The diagnosis was metachronous relapse of Wilms' tumor 7 years after the first diagnosis. The patient received five courses of preoperative chemotherapy and tumorectomy was performed. Because of post-surgical complications, nephrectomy was performed on her only kidney. Since she is anephric, the patient is in chronic renal failure and is dependent on dialysis. Treatment with carboplatin and etoposide was continued after surgery and the patient is currently in complete remission. The appearance of a metachronous Wilms' tumor 5 years after that of the primary tumor is rare. When a contralateral tumour develops, chemotherapy must be given until the size of the tumor is reduced in order to preserve renal function and avoid dialysis. In patients with chronic renal failure caused by bilateral nephrectomy, ongoing treatment with dialysis support can be achieved through the choice of effective drugs and knowledge of their pharmacokinetics and pharmacodynamics.

Key words:
Wilms
Nephroblastoma
Bilateral
metachronous
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