Outcomes of event-free survival in patients with Wilms tumor undergoing preoperative chemotherapy. Analysis of lymph-node yield in a single-center cohort.

INTRODUCTION: The clinical value of lymph node sampling in Wilms tumor (WT) lies in its ability to accurately determine lymph node (LN) involvement. LN yield (LNY) is used as a valuable tool to measure LN retrieval, and a minimum of 6 LNs is one of the current recommendations. In patients who are managed with the SIOP strategy, preoperative chemotherapy decreases the retrieval of LN during surgery resulting in lower LNY values. PURPOSE: To determine the mean LNY and to analyze survival outcomes in patients with WT who underwent preoperative chemotherapy at a single center. METHODS: We performed a retrospective chart review of all patients between 6 months and 12 years of age with unilateral WT who underwent preoperative chemotherapy between 2010 and 2018. Patients with bilateral WT or without preoperative chemotherapy were excluded. Collected data included: demographics, tumor volume, tumor histopathology, number of LNs collected (LNY), presence or absence of tumor in the retrieved LNs, and disease stage according to these results. Kaplan Meier curves were calculated to estimate 5-year event-free survival (EFS) and overall survival (OS). RESULTS: 95 patients with a median follow-up of 25 months were included in the study. A total of 19 patients underwent laparoscopic surgery. Mean LNY for the entire cohort was 3.26 (95% CI, 2.4-3.6; SD, 3.62). Six patients (6.3%) had at least one positive LN. The estimated 5-year OS was 89.3% (95% CI, 82.1%-96.5%). EFS was 79.8 (95% CI, 74.8%-84.8%). Recurrence rate was 20% (n: 19). Four patients (4.2%) developed local recurrence and 15 patients (15.7%) developed pulmonary recurrence. The initial mean LNY in patients that relapsed was 4.16 (95% CI, 2.2-5.8; SD: 4.18), which was higher than in patients who did not relapse (LNY: 3; 95% CI, 2.33-3.67; SD, 3.39). No recurrences or deaths occurred in the laparoscopic group. DISCUSSION: The identification of a minimum LNY (i.e. threshold) to minimize the risk of harboring occult metastatic disease has been proposed to standardize the surgical procedure. Preliminary results in this study suggest that a limited LNY with acceptable survival outcomes is a possible scenario in patients treated according to the SIOP protocol. Systematic LN sampling to reduce the rate of false negatives is still strongly recommended. CONCLUSION: Our cohort presented with a relatively low LNY compared to standard recommendations. Our EFS, however, remained acceptable. Multivariate analysis would be necessary to determine the actual role of LN sampling as an isolated prognostic factor in unilateral WT.

Laparoscopic total nephrectomy for Wilms tumor: Towards new standards of care.

INTRODUCTION: Laparoscopic total nephrectomy (LN) in malignant pediatric tumors remains controversial. For selected patients undergoing pre-operative chemotherapy in referral centers, LN has so far shown comparable results to the standard open technique. PURPOSE: To describe the inclusion criteria and preliminary results of laparoscopic nephrectomies (LN) for the treatment of unilateral Wilms tumors (WT). MATERIAL AND METHODS: Between November 2010 and January 2016, a retrospective study of patients with WT and undergoing pre-operative chemotherapy was performed. Inclusion criteria for candidates for LN were: unilateral tumors without venous invasion and central kidney localization. Tumor size and vascular thrombus were estimated with pre-operative computed tomography (CT) scan. Overall survival and recurrence rates were evaluated. RESULTS: Among 105 patients with WT, 14 underwent LN. Tumor bleeding or the lack of response to chemotherapy were not exclusion criteria. Median tumor volume for the patients undergoing LN was 71.5 cc (range 7-169). Patients with small tumors localized near the renal pole and candidates for nephron sparing surgery (NSS) were excluded. Estimated 5-year overall survival for all patients with WT during this period was 88.7% (88.1-103.1). Two patients underwent conversion. No recurrence or related death was found at a mean 32- month follow-up period. DISCUSSION: Reproducing the steps of the open nephrectomy when performing LN for malignant tumors allowed comparable oncologic results to the conventional procedure. However, upstaging of the tumor was not admissible and has become the main goal when approaching these patients laparoscopically. Preliminary results showed that the incidence of intraoperative rupture and incomplete node sampling were not an issue when comparing LN to open nephrectomy. On the other hand, LN for malignant tumors requires experience in advanced laparoscopy and oncologic surgery. Pre-operative chemotherapy changes the tumor's consistency and this is the key point as to why these patients are amenable to be approached laparoscopically. Lifting the tumor along with the fat to avoid capsule fraction, as well as changing the lens to the lateral port to achieve a correct view for lymph node sampling are some of the considerations when performing LN. CONCLUSIONS: Preliminary data suggest that LN for WT is feasible and has promising results in terms of event-free and overall survival. In patients undergoing pre-operative chemotherapy the correct selection for LN is crucial. Following the basic oncological precepts and in experienced centers, LN represents a plausible modality in the care of these patients.

Primary lung tumors in children: 24 years of experience at a referral center.

PURPOSE: Primary lung tumors are rare during childhood and encompass a wide variety of histological types. Each has a different biology and a different therapeutic approach. The aim of this article is to review the experience of a pediatric referral center with this kind of tumors during the last 24 years. METHODS: A retrospective chart review was performed for patients with diagnosis of primary lung tumor between the years 1990-2014. The variables analyzed were age, sex, course of the disease, symptoms, localization, surgery, histology and outcome. RESULTS: Between 1990 and 2014, 38 patients with primary lung tumors were treated at our institution. Age at presentation was 6.6 ± 5.2 years (r 0.91-16.58) and the female:male relationship was 1.37. Inflammatory myofibroblastic lung tumor (n = 13), carcinoid tumor (n = 6) and pleuropulmonary blastoma (n = 6) were the most frequent histological types. Persistent radiographic abnormality was the most frequent presenting sign (34 %). Global mortality was 15.8 % varying according to histology. CONCLUSION: Although the diagnosis of primary lung tumor is rare, the persistence of a radiographic abnormality in spite of adequate treatment for inflammatory processes forces us to evaluate further. The age of the patient is an important factor in the decision of the diagnostic work-up.

[Surgical treatment of tracheal stenosis in pediatrics]. / Tratamiento quirúrgico de la estenosis traqueal en pediatría.

Twenty patients with tracheal stenosis were surgically treated between July 2005 and May 2008; ten patients had a congenital stenosis and ten an acquired one. Global survival was 85%. Three patients died: 1 with acquired stenosis and 2 with congenital stenosis. From 17 alive patients, 15 remain asymptomatic; 2 patients operated on due to congenital stenosis, are under continuous follow-up (both with stents placed in the trachea). Surgical treatment of acquired stenosis presents less severe complications, requires less ventilatory support and hospital stay. Conversely, congenital stenosis often requires more than one procedure to control the disease, and presents a higher mortality rate.

Tratamiento quirúrgico de la estenosis traqueal en pediatría / Surgical treatment of tracheal stenosis in pediatrics

Veinte pacientes con estenosis de tráquea fueron intervenidos quirúrgicamente entre julio de 2005y mayo de 2008, diez con estenosis congénita y diez de origen adquirido.La sobrevida global fue de 85 por ciento. Fallecieron tres pacientes, uno con estenosis adquirida y dos conestenosis congénita.De los diecisiete sobrevivientes, quince se encuentran asintomáticos; dos pacientes operados por estenosis congénita requieren controlesperiódicos (ambos con tutores endoluminales [stents] colocados).La estenosis traqueal adquirida presenta menos complicaciones, requiere menos asistencia respiratoria y menor estadía hospitalaria en el posoperatorio. Los pacientes con estenosis traquealcongénita necesitan, generalmente, másde un procedimiento terapéutico y presentan mayor mortalidad.(AU)

Tratamiento quirúrgico de la estenosis traqueal en pediatría / Surgical treatment of tracheal stenosis in pediatrics

Veinte pacientes con estenosis de tráquea fueron intervenidos quirúrgicamente entre julio de 2005y mayo de 2008, diez con estenosis congénita y diez de origen adquirido.La sobrevida global fue de 85 por ciento. Fallecieron tres pacientes, uno con estenosis adquirida y dos conestenosis congénita.De los diecisiete sobrevivientes, quince se encuentran asintomáticos; dos pacientes operados por estenosis congénita requieren controlesperiódicos (ambos con tutores endoluminales [stents] colocados).La estenosis traqueal adquirida presenta menos complicaciones, requiere menos asistencia respiratoria y menor estadía hospitalaria en el posoperatorio. Los pacientes con estenosis traquealcongénita necesitan, generalmente, másde un procedimiento terapéutico y presentan mayor mortalidad.

Comparación de distintos métodos de preservación traqueal / Comparison of different methods of tracheal preservation

El objetivo de este estudio es comparar distintos métodos de preservación traqueal en un modelo isogénico heterotópico de transplante inmediato y con 24 horas de preservación. Ochenta ratas Sprague-Dawley endocriadas, con un peso de 300-340 gramos fueron utilizadas como donantes y receptores divididas en 8 grupos: A: trasplante inmediato, no Euro-Collins, no criopreservación, B: trasplante inmediato, si Euro-Collins, no crioperservación, C: trasplante 24 horas, no Euro-Collins, no criopreservación, D: trasplante 24 horas, si Euro-Collins, no criopresenrvación, E: criopreservación luego de 24 horas a 4ºC, no Euro-Collins, F: criopreservación inmediata, no Euro-Collins, G: criopreservación luego de 24 horas a 4ºC, si Euro-Collins, H: criopreservación inmediata, si Euro-Collins. Todos los injertos fueron implantados en epiplón. Tres muestras para estudio microscópico fueron tomadas de cada espécimen: inmediatamente después de la ablación, inmediatamente antes del implante (al finalizar el período de preservación) y en el momento del sacrificio. No hubo muertes antes del tiempo establecido. Los escores de daño histológico del material obtenido en el momento del sacrificio fueron: grupo A: 3.1; grupo B: 1.7; grupo C: 3; grupo D: 1.1; grupo E: 3.2; grupo F: 3.7; grupo G: 1.3 y grupo H: 1. Todos los injertos presentaban la luz ocupadas por granulomas. La preservación con Euro Collins es superior a la preservación sin Euro Collins tanto en tráqueas criopreservadas como no criopreservadas. La criopreservación es una estrategia válida para la obtención de injertos traqueales. Los injertos criopreservados presentan lesiones leves aunque más severas que aquellos no criopreservados. La irrigación de Euro-Collins permite preservar tráqueas hasta 24 horas a 4ºC. (AU)

Comparación de distintos métodos de preservación traqueal / Comparison of different methods of tracheal preservation

El objetivo de este estudio es comparar distintos métodos de preservación traqueal en un modelo isogénico heterotópico de transplante inmediato y con 24 horas de preservación. Ochenta ratas Sprague-Dawley endocriadas, con un peso de 300-340 gramos fueron utilizadas como donantes y receptores divididas en 8 grupos: A: trasplante inmediato, no Euro-Collins, no criopreservación, B: trasplante inmediato, si Euro-Collins, no crioperservación, C: trasplante 24 horas, no Euro-Collins, no criopreservación, D: trasplante 24 horas, si Euro-Collins, no criopresenrvación, E: criopreservación luego de 24 horas a 4ºC, no Euro-Collins, F: criopreservación inmediata, no Euro-Collins, G: criopreservación luego de 24 horas a 4ºC, si Euro-Collins, H: criopreservación inmediata, si Euro-Collins. Todos los injertos fueron implantados en epiplón. Tres muestras para estudio microscópico fueron tomadas de cada espécimen: inmediatamente después de la ablación, inmediatamente antes del implante (al finalizar el período de preservación) y en el momento del sacrificio. No hubo muertes antes del tiempo establecido. Los escores de daño histológico del material obtenido en el momento del sacrificio fueron: grupo A: 3.1; grupo B: 1.7; grupo C: 3; grupo D: 1.1; grupo E: 3.2; grupo F: 3.7; grupo G: 1.3 y grupo H: 1. Todos los injertos presentaban la luz ocupadas por granulomas. La preservación con Euro Collins es superior a la preservación sin Euro Collins tanto en tráqueas criopreservadas como no criopreservadas. La criopreservación es una estrategia válida para la obtención de injertos traqueales. Los injertos criopreservados presentan lesiones leves aunque más severas que aquellos no criopreservados. La irrigación de Euro-Collins permite preservar tráqueas hasta 24 horas a 4ºC.