Outcome of Nephroblastoma Treatment According to the SIOP-2001 Strategy at a Single Institution in Argentina.

OBJECTIVE: Wilms tumor (WT) is a disease with a good prognosis. The aim of this study was to evaluate the outcome of patients with WT, treated according to the SIOP-2001 strategy. METHODS: A retrospective analysis of 141 consecutive patients with WT diagnosed at our institution between December 2001 and 2013 was performed. RESULTS: A total of 114 patients, median age 38.8 months (3 to 155 mo), were assessable for analysis. Fine-needle aspiration was initially performed in 88 patients (84.6%). Stage distribution was: I: 33%, II: 9.6%, III: 28%, IV: 14%, V: 14.9%. Six patients were stage III because of tumor spillage. The remaining patients received preoperative chemotherapy. Adjuvant chemotherapy was given without randomization, using vincristine-actinomycin for stage II and vincristine-doxorubicin-actinomycin plus radiotherapy for stage III. After a median follow-up of 52 months, 5-year overall survival and event-free survival were 91% and 85%, respectively. Overall survival according to stage was: I: 96%, II: 99%, III: 88%, IV: 78%, V: 90% (P=0.16). There was no significant difference in event-free survival (P=0.7). Seventy-eight (85.7%) were intermediate-risk and 11 (12%) were high-risk patients. Seventeen patients (14.9%) relapsed within 2 to 99 months (median 29.9 mo). Eight patients (7%) died of progressive disease. There were no treatment-related deaths. CONCLUSIONS: The SIOP-01 protocol proposes a treatment strategy that is feasible in our institution, achieving good results.

[Pediatric adamantinoma. Case report]. / Adamantinoma en pediatría. Presentación de caso.

Adamantinoma is a primary tumor of long bones, which affects mainly the shaft of the tibia, and is extremely rare in pediatrics. It frequently presents during the second decade of life, with a slight predominance in males. It is a low grade tumor, with local aggressiveness and low rate of metastasis and recurrence once it is completely removed. Its diagnosis is difficult, not only because it is a rare disease in children, but also because of the difficulty in the differential diagnosis with other benign lesions. We report the case of a 15-year-old patient with a painless swelling of the distal tibia, whose diagnosis was confirmed with the piece of amputation, as imaging features and both initial biopsies were not enough to achieve diagnosis. Though most of the literature consists of case reports, and very few in pediatric patients, they all agree on the difficulty in achieving the diagnosis of adamantinoma.

Adamantinoma en pediatría: Presentación de caso / Pediatric adamantinoma: Case report

El adamantinoma es un tumor primario de los huesos largos, que afecta principalmente la diálisis de la tibia, y es extremadamente raro en pediatría. Se presenta a partir de la segunda década de vida, con un ligero predominio en el sexo masculino. Se trata de un tumor de bajo grado, con alta agresividad a nivel local y bajo índice de metástasis y recurrencia una vez resecado en forma completa. Su diagnóstico resulta difícil, no solo por tratarse de una patología poco frecuente en pediatría, sino también por la dificultad para el diagnóstico diferencial con otras lesiones benignas. Presentamos el caso de un paciente de 15 años, con una tumoración indolora de larga evolución en la tibia distal, cuyo diagnóstico fue confirmado histológicamente con la pieza de amputación, ya que los estudios complementarios y las dos biopsias iniciales no fueron concluyentes. Aunque la mayoría de la bibliografía publicada consta de reportes de casos, y muy pocos en pacientes pediátricos, es de común acuerdo la dificultad para arribar al diagnóstico de adamantinoma.

Adamantinoma is a primary tumor of long bones, which affects mainly the shaft of the tibia, and is extremely rare in pediatrics. It frequently presents during the second decade of life, with a slight predominance in males. It is a low grade tumor, with local aggressiveness and low rate of metastasis and recurrence once it is completely removed. Its diagnosis is difficult, not only because it is a rare disease in children, but also because of the difficulty in the differential diagnosis with other benign lesions. We report the case of a 15-year-old patient with a painless swelling of the distal tibia, whose diagnosis was confirmed with the piece of amputation, as imaging features and both initial biopsies were not enough to achieve diagnosis. Though most of the literature consists of case reports, and very few in pediatric patients, they all agree on the difficulty in achieving the diagnosis of adamantinoma.

Adamantinoma en pediatría: Presentación de caso / Pediatric adamantinoma: Case report

El adamantinoma es un tumor primario de los huesos largos, que afecta principalmente la diálisis de la tibia, y es extremadamente raro en pediatría. Se presenta a partir de la segunda década de vida, con un ligero predominio en el sexo masculino. Se trata de un tumor de bajo grado, con alta agresividad a nivel local y bajo índice de metástasis y recurrencia una vez resecado en forma completa. Su diagnóstico resulta difícil, no solo por tratarse de una patología poco frecuente en pediatría, sino también por la dificultad para el diagnóstico diferencial con otras lesiones benignas. Presentamos el caso de un paciente de 15 años, con una tumoración indolora de larga evolución en la tibia distal, cuyo diagnóstico fue confirmado histológicamente con la pieza de amputación, ya que los estudios complementarios y las dos biopsias iniciales no fueron concluyentes. Aunque la mayoría de la bibliografía publicada consta de reportes de casos, y muy pocos en pacientes pediátricos, es de común acuerdo la dificultad para arribar al diagnóstico de adamantinoma.(AU)

Adamantinoma is a primary tumor of long bones, which affects mainly the shaft of the tibia, and is extremely rare in pediatrics. It frequently presents during the second decade of life, with a slight predominance in males. It is a low grade tumor, with local aggressiveness and low rate of metastasis and recurrence once it is completely removed. Its diagnosis is difficult, not only because it is a rare disease in children, but also because of the difficulty in the differential diagnosis with other benign lesions. We report the case of a 15-year-old patient with a painless swelling of the distal tibia, whose diagnosis was confirmed with the piece of amputation, as imaging features and both initial biopsies were not enough to achieve diagnosis. Though most of the literature consists of case reports, and very few in pediatric patients, they all agree on the difficulty in achieving the diagnosis of adamantinoma.(AU)

Adamantinoma en pediatría: Presentación de caso / Pediatric adamantinoma: Case report

El adamantinoma es un tumor primario de los huesos largos, que afecta principalmente la diálisis de la tibia, y es extremadamente raro en pediatría. Se presenta a partir de la segunda década de vida, con un ligero predominio en el sexo masculino. Se trata de un tumor de bajo grado, con alta agresividad a nivel local y bajo índice de metástasis y recurrencia una vez resecado en forma completa. Su diagnóstico resulta difícil, no solo por tratarse de una patología poco frecuente en pediatría, sino también por la dificultad para el diagnóstico diferencial con otras lesiones benignas. Presentamos el caso de un paciente de 15 años, con una tumoración indolora de larga evolución en la tibia distal, cuyo diagnóstico fue confirmado histológicamente con la pieza de amputación, ya que los estudios complementarios y las dos biopsias iniciales no fueron concluyentes. Aunque la mayoría de la bibliografía publicada consta de reportes de casos, y muy pocos en pacientes pediátricos, es de común acuerdo la dificultad para arribar al diagnóstico de adamantinoma.(AU)

Adamantinoma is a primary tumor of long bones, which affects mainly the shaft of the tibia, and is extremely rare in pediatrics. It frequently presents during the second decade of life, with a slight predominance in males. It is a low grade tumor, with local aggressiveness and low rate of metastasis and recurrence once it is completely removed. Its diagnosis is difficult, not only because it is a rare disease in children, but also because of the difficulty in the differential diagnosis with other benign lesions. We report the case of a 15-year-old patient with a painless swelling of the distal tibia, whose diagnosis was confirmed with the piece of amputation, as imaging features and both initial biopsies were not enough to achieve diagnosis. Though most of the literature consists of case reports, and very few in pediatric patients, they all agree on the difficulty in achieving the diagnosis of adamantinoma.(AU)

Adamantinoma en pediatría. Presentación de caso. / [Pediatric adamantinoma. Case report].

Adamantinoma is a primary tumor of long bones, which affects mainly the shaft of the tibia, and is extremely rare in pediatrics. It frequently presents during the second decade of life, with a slight predominance in males. It is a low grade tumor, with local aggressiveness and low rate of metastasis and recurrence once it is completely removed. Its diagnosis is difficult, not only because it is a rare disease in children, but also because of the difficulty in the differential diagnosis with other benign lesions. We report the case of a 15-year-old patient with a painless swelling of the distal tibia, whose diagnosis was confirmed with the piece of amputation, as imaging features and both initial biopsies were not enough to achieve diagnosis. Though most of the literature consists of case reports, and very few in pediatric patients, they all agree on the difficulty in achieving the diagnosis of adamantinoma.