Manejo quirúrgico del tumor de Wilms bilateral: Nuestra experiencia con 18 casos / Surgical management of the bilateral wilm's tumor: Our experience with 18 cases

Objetivo. Mostrar nuestra experiencia en el manejo quirúrgico del tumor de Wilms bilateral. Material y métodos. Se analizaron los datos de 18 niños con diagnóstico de TW bilateral entre 1971 y 2007, evaluando la edad al momento del diagnóstico, sexo, presentación clínica, métodos diagnósticos, histología, tratamiento quimioterápico, radioterápico y quirúrgico, complicaciones y el estado clínico actual de los pacientes. Resultados. El 65% de los TW sincrónicos se encontraban en estadios I / II; el 30% mostraban al menos un tumor en estadio III. Un caso en estadio IV (5%). Los pacientes con TW metacrónicos se encontraban en el 100% de los casos en estadios I /II. Todos los tumores fueron de bajo o intermedio grado de malignidad, con predominio del tipo mixto. Las complicaciones quirúrgicas fueron 4 suboclusiones intestinales,2 fístulas ureteropiélicas y 1 quiste urinario.15 niños permanecen convida (83%) con un período libre de enfermedad entre 1 y 24 años, delos cuales 3 se han trasplantado con buena evolución. Un paciente falleció por insuficiencia renal progresiva y otros dos pacientes por evolución de la enfermedad. Conclusiones. La quimioterapia preoperatoria citorreductora permite una cirugía renal más conservadora con una alta tasa de supervivencia (80-90%). El tratamiento quirúrgico individualizado conlleva resecciones más conservadoras y una menor incidencia de insuficiencia renal a largo plazo (AU)

Aim. To show our experience in the surgical management of bilateral Wilms’ tumor. Methods. We have reviewed the medical records of 18 patients diagnosed of bilateral Wilms’ tumor between 1971 and 2007, evaluating age, sex, clinical situation, imaging studies, histology, treatment, complications and follow-up. Results. 65% of patients with synchronous Wilms’ tumor was stageI-II, 30% stage III and 5% stage IV. 100% of patients with metachronous Wilms’ tumor was stage I-II. All the tumors had favourable histology. Surgical complications were: 4 bowel pseudobstructions, 2ureteropielic fistulae and 1 urinary cyst. 15 patients are alive (83%) with a mean follow-up of 12 years.3 of these patients had a renal transplant with a good evolution. One patient died of a progressive renal failure and two patients died of the evolution of the oncological disease. Conclusions. Preoperative chemotherapy allows a conservative surgical resection with a high overall survival (80-90%). Individualized surgical treatment offers a conservative surgical resection with a lower incidence of long-term renal failure (AU)

[Surgical management of bilateral Wilms' tumor: our experience with 18 cases]. / Manejo quirúrgico del tumor de Wilms bilateral: nuestra experiencia con 18 casos.

AIM: To show our experience in the surgical management of bilateral Wilms' tumor. METHODS: We have reviewed the medical records of 18 patients diagnosed of bilateral Wilms' tumor between 1971 and 2007, evaluating age, sex, clinical situation, imaging studies, histology, treatment, complications and follow-up. RESULTS: 65% of patients with synchronous Wilms' tumor was stage I-II, 30% stage III and 5% stage IV. 100% of patients with metachronous Wilms' tumor was stage I-II. All the tumors had favourable histology. Surgical complications were: 4 bowel pseudobstructions, 2 ureteropielic fistulae and 1 urinary cyst. 15 patients are alive (83%) with a mean follow-up of 12 years. 3 of these patients had a renal trasplant with a good evolution. One patient died of a progressive renal failure and two patients died of the evolution of the oncological disease. CONCLUSIONS: Preoperative chemotherapy allows a conservative surgical resection with a high overall survival (80-90%). Individualized surgical treatment offers a conservative surgical resection with a lower incidence of long-term renal failure.

Tumoraciones cervicales / Neck masses

Las tumoraciones cervicales son un proceso muy frecuente en la consulta de pediatría. Se deben valorar el momento de aparición, localización y tamaño para suponer su origen. En caso de tumoración que no mejora con antibióticos, en triángulo posterior o supraclavicular, fija o mayor de 2,5 cm, conviene que sea valorada por un cirujano para descartar la patología maligna

Neck masses are very common in the pediatric age group. The time of onset, location and size must be assessed in order to determine its origin. If the mass does not improve with antibiotics, is located in the posterior or supraclavicular triangle of the neck, is immobile or measures more than 2.5 cm, it should be examined by a pediatric surgeon in order to rule out the presence of a malignancy

[Prophylactic thyroidectomy in children at risk for medullary thyroid carcinoma]. / Tiroidectomía profiláctica en pacientes pediátricos con riesgo de carcinoma medular tiroideo.

The medullary thyroid carcinoma (MTC) is a rare neoplasia occurring during childhood. At present time the molecular examination of the proto-oncogen RET, related to syndromes of multiple endocrine neoplasia (MEN II) and familial medullary thyroid carcinoma (FMTC) to allows identify patients with risk of suffering of medullary thyroid carcinoma in early ages, before the disease becomes clinically pronunced. Children with familial antecedents of MEN II or FMTC were biochemically (pentagastrin-stimulated) and genetically studied with the purpose of determining the risk of developing a MTC and in order to assess the possibilities of making a prophylactic thyroidectomy.

[Spigelian hernia: two cases associated to cryptorchidism]. / Hernia de Spiegel: a propósito de 2 casos asociados a criptorquidia.

Spigelian hernia (SH) is rare in children, it occurs primarily en adults the mean age of presentacion is 50 years. We present two patients with hernia associated to cryptorchidism one of them bilateral.

Tiroidectomía profiláctica en pacientes pediátricos con riesgo de carcinoma medular tiroideo / Prophylactic thyroidectomy in children at risk for medullary thyroid carcinoma

El carcinoma medular de tiroides (CMT) es una rara neoplasiaen la infancia. En la actualidad el estudio molecular del proto-oncogén RET, relacionado con los síndromes de neoplasia endocrina múltiple (MEN II) y carcinoma medular familiar de tiroides (CMFT)permiten identificar pacientes con riesgo de padecer carcinoma medularde tiroides en edades precoces, antes de que se manifieste la enfermedad clínicamente. Niños con antecedentes familiares de MEN II o CMFT fueron estudiados bioquímica (test de pentagastrina) y genéticamente con el fin dedeterminar el riesgo de padecer CMT y evaluar la posibilidad de realizar tiroidectomía profiláctica (AU)

The medullary thyroid carcinoma (MTC) is a rare neoplasiaoccurring during childhood. At present time the molecular examinationof the proto-oncogen RET, related to syndromes of multipleendocrine neoplasia (MEN II) and familial medullary thyroid carcinoma(FMTC) to allows identify patients with risk of suffering of medullarythyroid carcinoma in early ages, before the disease becomes clinicallypronunced. Children with familial antecedents of MEN II orFMTC were biochemically (pentagastrin-stimulated) and geneticallystudied with the purpose of determining the risk of developing a MTCand in order to assess the possibilities of making a prophylactic thyroidectomyThe medullary thyroid carcinoma (MTC) is a rare neoplasia occurring during childhood. At present time the molecular examinationof the proto-oncogen RET, related to syndromes of multiple endocrine neoplasia (MEN II) and familial medullary thyroid carcinoma(FMTC) to allows identify patients with risk of suffering of medullary thyroid carcinoma in early ages, before the disease becomes clinically pronunced. Children with familial antecedents of MEN II orFMTC were biochemically (pentagastrin-stimulated) and genetically studied with the purpose of determining the risk of developing a MTCand in order to assess the possibilities of making a prophylactic thyroidectomy (AU)

Hernia de Spiegel: A propósito de 2 casos asociados a criptorquidia / Spigelian: two cases associated to cryptorchidism

La hernia de Spiegel es una hernia rara en la población infantil, más común en la población adulta con predominio en la 5ª década de la vida. Presentamos dos pacientes con hernia asociada a criptorquidia, una de ellas bilateral (AU)

Spigelian hernia (SH) is rare in children, it occurs primarily en adults the mean age of presentacion is 50 years. We present two patients with hernia associated to cryptorchidism one of them bilateral (AU)

[Caustic burns of the esophagus in childhood. Our 14 years' experience]. / Causticación de esófago en la infancia. Nuestra experiencia de catorce años.

A 14-years revision (1972-1986) is made of 3,600 children attended to after ingesting some type of caustic agent. Only 81 were admitted to hospital. We found a predominance in the 1-3 year age group. Bleach was the caustic most frequently present but lye accounted for most major lesions. The more relevant symptoms were oropharyngeal lesions (85%), vomiting (26%) and sialorrhea (20%). Oropharyngeal burns was the sign most often found in esophageal lesions (45%). The ingestion of ordinary household bleach did not result in serious esophageal lesions nor posterior complications, and does not require esophagoscopy if no other symptom except vomiting is present. Esophagoscopy is the ideal means for evaluating esophageal lesions, as are esophageal dilatations with Rehbein dilators for stenosis due to scarring. With the use of corticosteroids in the acute phase, there were 8 cases (22%) of esophageal stenosis out of 36 with lesions.