Results of induction chemotherapy in children older than 18 months with stage-4 neuroblastoma treated with an adaptive-to-response modified N7 protocol (mN7)

Purpose. We report the response rate in children older than 18 months with stage 4 Neuroblastoma, using a modified dose-intensive, response-adaptive, induction mN7 protocol. Methods. From 2005 to 2012, 24 patients were treated with the mN7 protocol. Phase 1 included five MSKCC N7 cycles and surgery and two high-dose cyclophosphamide-topotecan (HD-CT) cycles for those who did not achieve complete remission (CR) and negative bone marrow (BM) minimal residual disease (MRD) status (CR+MRD-). Phase 2 consisted of myeloablative doses of topotecan, thiotepa and carboplatin plus hyperfractionated RT. Phase 3 included isotretinoin and 3F8 immunotherapy plus GM-CSF. BM MRD was monitored using GD2 synthase, PHOX2B and cyclin D1 mRNAs. Results. After 3 cycles, all patients showed BM complete histological clearance and 6 (25 %) were MRD-. Twenty of 21 s-look surgeries achieved macroscopic complete resection. After 5 cycles and surgery, 123I-MIBG scan was negative in 15 (62.5 %) cases, BM disease by histology was negative in 23 (96 %) and 10 (42 %) patients were MRD-. Twelve (50 %) pts were in CR, 2 in very good partial response (VGPR), 9 partial response (PR) and one had progressive disease. With 2 HD-CT extra cycles, 17 (71 %) pts achieved CR+MRD- status moving to phase 2. Overall and event-free survival at 3 years for the 17 patients who achieved CR+MRD- is 65 and 53 %, respectively, median follow-up 47 months. Seven (29 %) patients never achieved CR+MRD-. Univariate Cox regression analysis shows CR+MRD- status after mN7 induction as the only statistically significant prognostic factor to predict overall survival. Conclusions. mN7 induction regimen produced a CR+MRD- rate of 71 %. CR+MRD- status following induction was the only predictive marker of long-term survival (AU)

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Results of induction chemotherapy in children older than 18 months with stage-4 neuroblastoma treated with an adaptive-to-response modified N7 protocol (mN7).

PURPOSE: We report the response rate in children older than 18 months with stage 4 Neuroblastoma, using a modified dose-intensive, response-adaptive, induction mN7 protocol. METHODS: From 2005 to 2012, 24 patients were treated with the mN7 protocol. Phase 1 included five MSKCC N7 cycles and surgery and two high-dose cyclophosphamide-topotecan (HD-CT) cycles for those who did not achieve complete remission (CR) and negative bone marrow (BM) minimal residual disease (MRD) status (CR+MRD-). Phase 2 consisted of myeloablative doses of topotecan, thiotepa and carboplatin plus hyperfractionated RT. Phase 3 included isotretinoin and 3F8 immunotherapy plus GM-CSF. BM MRD was monitored using GD2 synthase, PHOX2B and cyclin D1 mRNAs. RESULTS: After 3 cycles, all patients showed BM complete histological clearance and 6 (25 %) were MRD-. Twenty of 21 s-look surgeries achieved macroscopic complete resection. After 5 cycles and surgery, (123)I-MIBG scan was negative in 15 (62.5 %) cases, BM disease by histology was negative in 23 (96 %) and 10 (42 %) patients were MRD-. Twelve (50 %) pts were in CR, 2 in very good partial response (VGPR), 9 partial response (PR) and one had progressive disease. With 2 HD-CT extra cycles, 17 (71 %) pts achieved CR+MRD- status moving to phase 2. Overall and event-free survival at 3 years for the 17 patients who achieved CR+MRD- is 65 and 53 %, respectively, median follow-up 47 months. Seven (29 %) patients never achieved CR+MRD-. Univariate Cox regression analysis shows CR+MRD- status after mN7 induction as the only statistically significant prognostic factor to predict overall survival. CONCLUSIONS: mN7 induction regimen produced a CR+MRD- rate of 71 %. CR+MRD- status following induction was the only predictive marker of long-term survival.

Utilidad de la tomografía por emisión de positrones con 18F-FDG en un caso de papilomatosis pulmonar recurrente juvenil / Utility of positron emission tomography with 18F-FDG in a case of juvenile recurrent respiratory papillomatosis

La papilomatosis pulmonar recurrente juvenil (PPRJ) es una enfermedad infecciosa que provoca el crecimiento de papilomas en la vía respiratoria en la que hasta en un 4% de los casos degeneran hacia un carcinoma de células escamosas. Presentamos el caso de una paciente de 17 años con PPRJ en la que se valora la utilidad de la 18F-FDG-PET/TC ante la sospecha de malignización de las lesiones papilomatosas. Las técnicas de imagen morfometabólicas, la TC y la PET/TC fueron sugestivas de malignidad. Sin embargo, esta no fue confirmada en el análisis anatomopatológico tras su resección. La FDG-PET/TC no parece una herramienta útil para la detección precoz de malignidad en la PPRJ, aunque sí aumenta la rentabilidad diagnóstica de la biopsia al identificar las lesiones más activas y, por lo tanto, con mayor posibilidad de ser malignas (AU)

Juvenile recurrent respiratory papillomatosis (JRRP) is an infectious disease caused by the growth of papillomas in the airway. Up to 4% of these cases degenerate into squamous cell carcinoma. We present the case of a 17-year-old female patient with JRRP in which the utility of 18F-FDG-PET/CT in the characterization of suspicious papillomatous lesions of malignancy is evaluated. Morphometabolic techniques, CT scan and PET/CT scans were suggestive of malignancy. However, this was not confirmed in the histopathological analysis after its resection. The 18F-FDG-PET/CT does not seem to be a useful tool for early detection of malignancy in JRRP. However, it does increase the diagnostic accuracy of the biopsy as it identifies the most active lesions and, therefore, those most likely to be malignant (AU)

[Utility of positron emission tomography with 18F-FDG in a case of juvenile recurrent respiratory papillomatosis]. / Utilidad de la tomografía por emisión de positrones con 18F-FDG en un caso de papilomatosis pulmonar recurrente juvenil.

Juvenile recurrent respiratory papillomatosis (JRRP) is an infectious disease caused by the growth of papillomas in the airway. Up to 4% of these cases degenerate into squamous cell carcinoma. We present the case of a 17-year-old female patient with JRRP in which the utility of (18)F-FDG-PET/CT in the characterization of suspicious papillomatous lesions of malignancy is evaluated. Morphometabolic techniques, CT scan and PET/CT scans were suggestive of malignancy. However, this was not confirmed in the histopathological analysis after its resection. The (18)F-FDG-PET/CT does not seem to be a useful tool for early detection of malignancy in JRRP. However, it does increase the diagnostic accuracy of the biopsy as it identifies the most active lesions and, therefore, those most likely to be malignant.

Carcinoma de tiroides en Pediatría: seguimiento a 30 años / Thyroid carcinoma in pediatrics: a 30-year follow-up

Introducción. Los tumores tiroideos son neoplasias de presentación infrecuente en la población pediátrica. El objeto de este estudio fue valorar la evolución terapéutica y seguimiento (supervivencia) a lo largo de 30 años. Materiales. Se revisaron 28 casos con diagnóstico de carcinoma de tiroides desde 1978 a 2008. Las variables a estudiar fueron: epidemiológicas, métodos diagnósticos utilizados, tipo de histología, tratamiento y supervivencia a 300 meses posterior al tratamiento. Consideramos como significativo una p<0,05. Resultados. El 73,1% de los casos fueron de sexo femenino. La edad promedio fue 12,3 años (1,6-19). La ecografía fue la prueba más solicitada (78,5%). La mutación RET se presento en tres casos asociados a MEN. El tipo histológico papilar fue el más frecuente (50%). Latiroidectomía total (TT) fue la cirugía más practicada (67,9%). La radioiodoablación (RIA) se usó en el 46,4% de los casos. La terapia de sustitución hormonal se usó en 27 pacientes de la serie. El 89,29% de la serie al cabo de 300 meses se encontraron libres de enfermedad. Conclusiones. El tratamiento de la serie ha variado a lo largo de (..) (AU)

Introduction. The thyroid tumours are infrequent neoplasms of presentation in paediatric. The object of this study was to value therapeutic evolution and the survival throughout 30 years. Materials. 28 cases with diagnosis of thyroid carcinoma. Were reviewed from 1978 to 2008. Were the variables to study: epidemiologists, methods used diagnostic, type of histology, later treatment and survival to 300 months to the treatment. We like considered significant p <0.05. Results. 73.1% of the cases were of feminine sex. The average age was 12.3 years (1.6 to 19). The ultrasound was asked for the test more(78.5%). The RET mutation associated appear in three cases to MEN. The histology type to papillary was most frequent (50%). The total thyroidectomy (TT) was the (..) (AU)

Lobectomía toracoscópica: Experiencia inicial / Thoracoscopic lobectomy: initial experience

Objetivo. Analizar nuestra experiencia inicial en el tratamiento de malformaciones pulmonares mediante lobectomía toracoscópica. Material y método. Hemos revisado nuestros primeros 6 casos del obectomía toracoscópica realizados en el último año (2008-09). Hemos realizado 6 lobectomías toracoscópicas en 6 pacientes varones con una edad media de 16 meses (6 m-4 años). Por localización se realizaron: 3lobectomías inferiores (2 derechas y una izquierda), 2 lobectomías superiores derechas y una lobectomía superior izquierda con preservación lingular. La patología de base fue la malformación adenomatoidea quística en 5 casos (dos de ellos asociados a secuestro intralobar) y un enfisema lobar por atresia bronquial. Resultados. Se utilizó una intubación selectiva en 4 casos y una intubación traqueal con bloqueador bronquial en dos casos. Ningun caso requirió reconversión a toracotomía. El tiempo operatorio medio fue de (..) (AU)

Aim. Analizing our preliminary experience in the treatment of pulmonary malformations with thoracoscopic lobectomy. Material and methods. We have reviewed our first cases of thoracoscopic lobectomy during last year (2008-09). We have performed6 thoracoscopic lobectomies in 6 male patients with a mean age of 16months (6 months-4 years). They were 3 lower lobectomies (2 right and one left), 2 right upper lobectomies and one lingular sparing left upper lobectomy. Lung lesion was a congenital cystic adenomatoid malformation in 5 (2 of them asociated with intralobar sequestration) and one lobar emphysema due to bronquial atresia. Results. Selective bronchial intubation was used in 4 cases and and tracheal intubation with bronchial blocker in 2. There were no conversions to open thoracotomy. Mean operative time was 202’ (155-250’). There were no intraoperative nor postoperative complications. None of these patients needed blood transfusion. Mean drainage time (..) (AU)

[Thyroid carcinoma in pediatrics: a 30-year follow-up]. / Carcinoma de tiroides en pediatría: seguimiento a 30 años.

INTRODUCTION: The thyroid tumours are infrequent neoplasms of presentation in paediatric. The object of this study was to value therapeutic evolution and the survival throughout 30 years. MATERIALS: 28 cases with diagnosis of thyroid carcinoma. Were reviewed from 1978 to 2008. Were the variables to study: epidemiologists, methods used diagnostic, type of histology, later treatment and survival to 300 months to the treatment. We like considered significant p <0.05. RESULTS: 73.1% of the cases were of feminine sex. The average age was 12.3 years (1.6 to 19). The ultrasound was asked for the test more (78.5%). The RET mutation associated appear in three cases to MEN. The histology type to papillary was most frequent (50%). The total thyroidectomy (TT) was the surgery practice more (67.9%). The radioiodine (RI) was used in 46.4% of the cases. The substitute hormonal therapy was used 27 patients. 89.29% of the were series after 300 months free of disease. CONCLUSIONS: The treatment of the series these are hundreds throughout 30 years, but when valuing our experience we include / understand that: 1) the TT with selective lymphatic dissection, 2) it in the RI cases with positive to the body tracking and/or of factors of risk and 3) the substitute hormonal therapy, is the at the moment best offer alternatives to than we can these paediatric patients and prolong the survival in adult its age.

[Thoracoscopic lobectomy: initial experience]. / Lobectomía toracoscópica: experiencia inicial.

AIM: Analizing our preliminary experience in the treatment of pulmonary malformations with thoracoscopic lobectomy. MATERIAL AND METHODS: We have reviewed our first cases of thoracoscopic lobectomy during last year (2008-09). We have performed 6 thoracoscopic lobectomies in 6 male patients with a mean age of 16 months (6 months-4 years). They were 3 lower lobectomies (2 right and one left), 2 right upper lobectomies and one lingular sparing left upper lobectomy. Lung lesion was a congenital cystic adenomatoid malformation in 5 (2 of them asociated with intralobar sequestration) and one lobar emphysema due to bronquial atresia. RESULTS: Selective bronchial intubation was used in 4 cases and and tracheal intubation with bronchial blocker in 2. There were no conversions to open thoracotomy. Mean operative time was 202' (155-250'). There were no intraoperative nor postoperative complications. None of these patients needed blood transfusion. Mean drainage time was 3.5 days (3-4 d) and mean discharge time was 4.8 days (4-7 d). With a mean follow-up time of 5.3 months (2 m-12 m), all of the patients have no symptoms and X-ray are normal. CONCLUSIONS: Thoracoscopic lobectomy is a safe and efective approach to treat congenital pulmonary pathology. The keys to perform it safely are good anesthetic management, adequate instruments for children size, and careful disection of broncovascular structures. Advantages of the minimal access surgery and implementation and miniaturization of the instruments and sealing devices will favor its progressive use.

Long-term follow-up of nineteen cystic lymphangiomas treated with fibrin sealant.

BACKGROUND: Surgical exeresis is regarded as the first choice treatment for cystic lymphangioma. Surgery may be extremely complex, giving rise to complications. Several therapeutic methods have been described to avoid the complications derived from the conventional surgical approach. The idea of treating lymphangioma by means of suction and injection of fibrin sealant (Tissucol), is an alternative to surgery. METHODS: The authors present 19 cases of cystic lymphangioma, treated with fibrin sealant injected into the lesion, during the 1991 to 1997 period. Two of the patients had been treated surgically and experienced recurrence of the tumor previous to treatment. In the other 17 cases, puncture was the only therapy applied. One patient required 3 punctures, another 6 patients required 2 punctures, and only 1 of them, after 2 unsuccessful punctures, was treated with surgical resection. The rest of the 10 cases subsided after the first puncture. Follow-up ranged between 3 and 72 months, with a mean of 40 months. RESULTS: The ultrasonographic (US) follow-up showed a complete remission in 17 patients treated with puncture. One patient remained with a small intermittent tumor, the appearance of which is related to catarrhal processes, and another patient rejected further puncture after the second one. No complications appeared. CONCLUSION: These results support the fact that the puncture, aspiration, followed by injection of Tissucol, is a choice in the surgical treatment of cystic lymphangioma.

Traumatismo esplénico en el niño / Spleen injuries in children

Se revisa los resultados de una conducta conservadora del bazo frente a un traumatismo esplénico en una población de 28 pacientes menores de edad. Todos habían sufrido un traumatismo abdominal cerrado y el diagnóstico fue hecho por laparatomía, ecografía o TAC. El tratamiento a que fueron sometidos incluye la abstención quirúrgica, la cirugía reparadora, el autotrasplante y la esplenectomía. Se analiza las diversas alternativas y sus resultados

[Ureteral multiplicity. Review of case records]. / Multiplicidad ureteral. Revisión de la casuística.

We present distribution by age, sex, clinical picture and clinical examination of 86 cases of renoureteral duplication and one of triplication of which 47 underwent surgical treatment. Intravenous urography provided direct evidence of the existence of duplication in 94%, indirect evidence in 100%, and it disclosed ipsi-or contralateral complications in 23%. Cystography demonstrated vesico ureteral reflux in 62%. Injection of contrast into the ectopic ureteral meatus, echography and gammagraphy provided data of interest in some cases. Accounts are given of indication for operation, approach, surgical procedures, direct of indirect complications, the association of this entity with other pathological conditions and of other procedures which have been described in the treatment of this anomaly.