[Pleuropulmonary blastoma in children: imaging findings and clinical patterns]. / Blastoma pleuropulmonar en niños: manifestaciones clínicas y radiológicas.

PURPOSE: To describe the imaging findings, clinical presentation and follow up of pleuropulmonary blastoma (PPB) in children. MATERIALS AND METHODS: Authors present a retrospective review of three young children with pathologically proven PPB seen from 1992 to 2006 in a pediatric hospital. The imaging findings on simple views, CT and MRI are presented. RESULTS: The first patient showed two bilateral well-defined solid lung lesions on chest X-rays, with homogeneous low attenuation on CT. Patient is free of disease following chemotherapy and surgical treatment. The second patient displayed a right tension pneumothorax. After drainage, he presented on chest X-rays and CT an underlying multicystic lesion. Following chemotherapy and surgical treatment, he presented two pulmonary metastases, which were treated with chemotherapy and surgery. The patient is now free of disease. The third patient showed a complete opacification of the left hemithorax due to a massive pleural effusion; a pleuropulmonary solid mass was seen on US, CT and MRI. The disease progressed with mediastinal, orbital and abdominal metastasis. The patient eventually died. CONCLUSION: PPB is a rare chest tumor seen in young children that can present with diverse radiological findings, and sometimes can arise in congenital cystic lung lesions. CT is the gold standard technique both for diagnosis and follow-up of these tumors.

Blastoma pleuropulmonar en niños: manifestaciones clínicas y radiológicas / Pleuropulmonary blastoma in children: imaging findings and clinical patterns

Objetivos. Describir los hallazgos por imagen del blastoma pleuropulmonar (BPP) en niños, así como su presentación clínica y evolución. Material y métodos. Estudio retrospectivo de tres pacientes consecutivos con confirmación anatomopatológica de BPP, recogidos entre 1992 y 2006 en un hospital de referencia pediátrico. Se analizan los hallazgos en la radiografía (Rx) de tórax, la tomografía computarizada (TC) y la resonancia magnética (RM). Resultados. Un paciente presentó en la Rx de tórax dos masas pulmonares sólidas bilaterales bien delimitadas, que en la TC se mostraban hipocaptantes y homogéneas. Tras tratamiento quimioterápico y cirugía, el paciente permanece libre de enfermedad. El segundo paciente comenzó con neumotórax a tensión derecho. Tras el drenaje del mismo se observó en la Rx de tórax y en la TC la presencia de una lesión multiquística subyacente. Posterior a la cirugía y al tratamiento quimioterápico presentó dos metástasis pulmonares que fueron tratadas con quimioterapia (QT) y cirugía. Actualmente está libre de enfermedad. El tercer caso presentó una opacificación completa del hemitórax izquierdo por derrame pleural masivo y masa sólida pleuropulmonar vista en la ecografía, la TC y la RM. La enfermedad progresó con extensión al mediastino, la órbita y el abdomen, y el paciente falleció. Conclusiones. El BPP es un tumor pulmonar infrecuente que se da en niños, con manifestaciones radiológicas diversas, que en ocasiones se asocia a una patología pulmonar quística congénita. La TC es la técnica de elección (patrón oro) tanto para el diagnóstico como para el seguimiento de estos tumores

Purpose. To describe the imaging findings, clinical presentation and follow up of pleuropulmonary blastoma (PPB) in children. Materials and methods. Authors present a retrospective review of three young children with pathologically proven PPB seen from 1992 to 2006 in a pediatric hospital. The imaging findings on simple views, CT and MRI are presented. Results. The first patient showed two bilateral well-defined solid lung lesions on chest X-rays, with homogeneous low attenuation on CT. Patient is free of disease followingchemotherapy and surgical treatment. The second patient displayed a right tension pneumothorax. After drainage, he presented on chest X-rays and CT an underlying multicystic lesion. Following chemotherapy andsurgical treatment, he presented two pulmonay metastases,which were treated with chemotherapy and surgery. The patient is now free of disease. The third patient showed a complete opacification of the left hemithorax due to a massive pleural effusion; a pleuropulmonary solid mass was seen on US, CT and MRI. The disease progressed with mediastinal, orbital and abdominal metastasis. The patient eventually died. Conclusion. PPB is a rare chest tumor seen in young children that can present with diverse radiological findings, and sometimes can arise in congenital cystic lung lesions. CT is the gold standard technique both for diagnosis and follow-up of these tumors (AU)

Tumores renales en niños menores de un año / Renal tumors in infants aged less than 1 year

Objetivo: Conocer la frecuencia y distribución de los tumores renales diagnosticados en una unidad de oncología pediátrica en niños menores de un año, sus características clínicas, anatomopatológicas, tratamientos utilizados y evolución. Material y métodos: Estudio retrospectivo de tumores renales primarios diagnosticados en lactantes en un hospital pediátrico, desde 1972 hasta febrero de 2003. Resultados: Obtuvimos 25 niños menores de un año (18,2 %), de un total de 137 tumores renales registrados. Diecisiete niños y 8 niñas. El más frecuente fue el nefroblastoma (15/25), seguido del nefroma mesoblástico (9/25), y uno fue un tumor rabdoide. La media de edad al diagnóstico fue de 4,8 meses (rango, 1 día-11 meses), la mediana de 5,03 meses; para los nefromas mesoblásticos la mediana fue de 1 día (rango, 1 día-3 meses). La forma de presentación fue como masa abdominal en 20 de ellos, en cuatro hematuria y un recién nacido empezó con obstrucción intestinal (nefroma mesoblástico). Presentaron hipertensión arterial 12/25 niños; 9/15 casos de nefroblastomas eran estadio I; uno, estadio II; uno, estadio III; dos, estadio IV; uno, estadio V, y uno falleció antes de la cirugía. La supervivencia global de los nefroblastomas a los 5 años es de 0,67 (error estándar [EE]: 0,12); y del nefroma mesoblástico de 0,89 (EE: 0,1), con un tiempo de supervivencia media de 290 meses. Conclusiones: En los niños menores de 6 meses el nefroma mesoblástico es más frecuente que el tumor de Wilms, siendo el tratamiento inicial de elección la cirugía, ya que este tipo de tumor es poco quimiosensible y la quimioterapia es peor tolerada en los lactantes

Objective: To determine the frequency and distribution of primary renal tumors diagnosed in a pediatric oncology unit in children younger than 1 year and identify their clinical and histopathological characteristics, the treatment used, and outcomes. Material and methods: We retrospectively reviewed the medical records of infants with primary tumors of the kidney diagnosed between January 1972 and February 2003. Results: A total of 137 tumors were diagnosed in our unit during the period studied. Of these, 25 (18.2 %) occurred in infants aged less than 1 year. There were 17 boys and 8 girls. The most prevalent tumor in this age group was Wilms' tumor (WT) in 15 patients, followed by mesoblastic nephroma (MN) in 9 patients and rhabdoid tumor in 1 patient. The mean age at diagnosis of WT was 4.8 months (range: 1 day-11 months), with a median of 5.03 months. The median age at diagnosis of MN was 1 day (range: 1 day-3 months). Presenting symptoms consisted of abdominal mass in 20 patients, hematuria in 4 patients and intestinal pseudo-occlusion (MN) in 1 patient. High blood pressure was found in 12 of the 25 patients. Among the 15 WT, 9 were stage I, 1 was stage II, one was stage III, 2 were stage IV, and 1 was stage V. One patient died before surgery. Overall survival at 5 years was 0.67 (SE 0.12) for WT and 0.89 (SE 0.1) for MN, respectively, with a mean follow-up of 290 months. Conclusions: MN was more frequent than WT in infants aged less than 6 months. The first-line therapy in these patients is surgery since this type of tumor shows little chemosensitivity and chemotherapy is poorly tolerated in infants

[Neonatal urinary ascites. Considerations on its diagnosis and treatment]. / Ascitis urinosa neonatal. Consideraciones sobre el diagnóstico y su tratamiento.

Seven cases with neonatal urinary ascites are described. In all of the cases the underlying cause was an anatomical or functional obstructive uropathy of the lower urinary tract, the most common pathology being neurogenic bladder secondary to lumbosacral dysphagia. The techniques utilized for patient evaluation (ultrasound, serial micturition cystourethrography and IVP) are described and the findings afforded by each diagnostic technique are presented. The value of serial micturition cystourethrography is underscored. In our view, it is convenient to perform initial diversion by percutaneous nephrostomy, suprapubic punction or placement of a urethral catheter, according to the level of the etiologic process. The definitive surgical approach will depend on the results of subsequent radiologic controls and the existing uropathy.

[Pyeloureteral junction syndrome. Associated vesicoureteral reflux]. / Síndrome de la unión pieloureteral. Reflujo vésicoureteral asociado.

We reviewed our series comprised of 106 children that had undergone surgery for congenital hydronephrosis from ureteropelvic junction obstruction. Following our protocol, all patients were evaluated by serial micturition cystourethrography. The only anomaly that had been detected was vesicoureteral reflux in 11.32% of the cases. This incidence is reduced to 8.25% if only the renoureteral systems with both congenital anomalies are considered. This finding is not statistically significant (p less than 0.05) relative to patient age, sex or clinical symptoms. The results of the present study show that children with ureteropelvic junction obstruction are not at risk of developing vesicoureteral reflux. Thus, routine serial micturition cystourethrography appears to be unwarranted.