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1.
Semin Pediatr Surg ; 32(5): 151338, 2023 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-38042090

RESUMO

Neuroblastoma (NB) is the most common solid extracranial malignancy of childhood with an incidence of 1 per 100,000 in the United States compromising approximately 10 % of childhood cancer. Unfortunately, patients with high-risk NG continue to have long-term survival less than 50 %. Both Children's Oncology Group and the International Society of Paediatric Oncology have demonstrated the important role of surgery in the treatment of high-risk NB. Herein, we compose the results of an extensive literature review as well as expert opinion from leaders in pediatric surgical oncology, to present the critical elements of effective surgery for high-risk neuroblastoma.


Assuntos
Neuroblastoma , Especialidades Cirúrgicas , Criança , Humanos , Neuroblastoma/cirurgia , Estados Unidos
3.
Pediatr Blood Cancer ; 70(5): e30242, 2023 05.
Artigo em Inglês | MEDLINE | ID: mdl-36798020

RESUMO

INTRODUCTION: Wilms tumor therapy in low- and middle-income countries (LMICs) relies on treatment protocols adapted to resource limitations, but these protocols have rarely been evaluated in real-world settings. Such evaluations are necessary to identify high-impact research priorities for clinical and implementation trials in LMICs. The purpose of this study was to identify highest priority targets for future clinical and implementation trials in sub-Saharan Africa by assessing outcomes of a resource-adapted treatment protocol in Malawi. METHODS: We conducted a retrospective cohort study of children treated for Wilms tumor with an adapted SIOP-backbone protocol in Lilongwe, Malawi between 2016 and 2021. Survival analysis assessed variables associated with poor outcome with high potential for future research and intervention. RESULTS: We identified 136 patients, most commonly with stage III (n = 35; 25.7%) or IV disease (n = 35; 25.7%). Two-year event-free survival (EFS) was 54% for stage I/II, 51% for stage III, and 13% for stage IV. A single patient with stage V disease survived to 1 year. Treatment abandonment occurred in 36 (26.5%) patients. Radiotherapy was indicated for 55 (40.4%), among whom three received it. Of these 55 patients, 2-year EFS was 31%. Of 14 patients with persistent metastatic pulmonary disease at the time of nephrectomy, none survived to 2 years. Notable variables independently associated with survival were severe acute malnutrition (hazard ratio [HR]: 1.9), increasing tumor stage (HR: 1.5), and vena cava involvement (HR: 3.1). CONCLUSION: High-impact targets for clinical and implementation trials in low-resource settings include treatment abandonment, late presentation, and approaches optimized for healthcare systems with persistently unavailable radiotherapy.


Assuntos
Neoplasias Renais , Tumor de Wilms , Criança , Humanos , Lactente , Neoplasias Renais/patologia , Estudos Retrospectivos , Malaui/epidemiologia , Tumor de Wilms/patologia , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Terapia Combinada , Nefrectomia , Estadiamento de Neoplasias
4.
Ann Thorac Surg ; 116(4): 803-809, 2023 10.
Artigo em Inglês | MEDLINE | ID: mdl-35489402

RESUMO

BACKGROUND: Intercostal nerve cryoablation with the Nuss procedure has been shown to decrease opioid requirements and hospital length of stay; however, few studies have evaluated the impact on complications and hospital costs. METHODS: A retrospective cohort study was performed for all Nuss procedures at our institution from 2016 through 2020. Outcomes were compared across 4 pain modalities: cryoablation with standardized pain regimen (n = 98), patient-controlled analgesia (PCA; n = 96), epidural (n = 36), and PCA with peripheral nerve block (PNB; n = 35). Outcomes collected included length of stay, opioid use, variable direct costs, and postoperative complications. Univariate and multivariate hierarchical regression analysis was used to compare outcomes between the pain modalities. RESULTS: Cryoablation was associated with increased total hospital cost compared with PCA (cryoablation, $11 145; PCA, $8975; P < .01), but not when compared with epidural ($9678) or PCA with PNB ($10 303). The primary driver for increased costs was operating room supplies (PCA, $2741; epidural, $2767; PCA with PNB, $3157; and cryoablation, $5938; P < .01). With multivariate analysis, cryoablation was associated with decreased length of stay (-1.94; 95% CI, -2.30 to -1.57), opioid use during hospitalization (-3.54; 95% CI, -4.81 to -2.28), and urinary retention (0.13; 95% CI, 0.05-0.35). CONCLUSIONS: Cryoablation significantly reduces opioid requirements and length of stay relative to alternative modalities, but it was associated with an increase in total hospital costs relative to PCA, but not epidural or PCA with PNB. Cryoablation was not associated with allodynia or slipped bars requiring reoperation.


Assuntos
Analgesia Epidural , Criocirurgia , Tórax em Funil , Transtornos Relacionados ao Uso de Opioides , Humanos , Nervos Intercostais/cirurgia , Analgésicos Opioides/uso terapêutico , Estudos Retrospectivos , Dor Pós-Operatória/tratamento farmacológico , Criocirurgia/efeitos adversos , Criocirurgia/métodos , Tórax em Funil/cirurgia , Analgesia Epidural/métodos
5.
J Pediatr Hematol Oncol ; 44(3): e751-e755, 2022 Apr 01.
Artigo em Inglês | MEDLINE | ID: mdl-34224514

RESUMO

Pancreatic angiosarcoma is an exceedingly rare malignancy accounting for <1% of pancreatic neoplasms. A very limited number of pancreatic angiosarcomas have been reported in the literature without any cases described in children. We present the case of a 17-year-old female diagnosed with angiosarcoma of the pancreas following pancreaticoduodenectomy for a pancreatic mass, initially presumed to be a solid pseudopapillary neoplasm of the pancreas. The angiosarcoma was found to have a novel activating internal tandem duplication in the KDR gene (KDR-internal tandem duplication). We discuss the current literature on this disease process. This is the first reported case of pancreatic angiosarcoma in a pediatric patient and the first with an activating KDR-internal tandem duplication.


Assuntos
Hemangiossarcoma , Neoplasias Pancreáticas , Adolescente , Feminino , Hemangiossarcoma/genética , Hemangiossarcoma/patologia , Hemangiossarcoma/cirurgia , Humanos , Pâncreas/patologia , Pâncreas/cirurgia , Pancreatectomia , Neoplasias Pancreáticas/genética , Neoplasias Pancreáticas/cirurgia , Pancreaticoduodenectomia , Receptor 2 de Fatores de Crescimento do Endotélio Vascular
6.
Ann Surg ; 275(3): e575-e585, 2022 03 01.
Artigo em Inglês | MEDLINE | ID: mdl-32649454

RESUMO

OBJECTIVE: To create the first structured surgical report form for NBL with international consensus, to permit standardized documentation of all NBL-related surgical procedures and their outcomes. SUMMARY OF BACKGROUND DATA: NBL, the most common extracranial solid malignant tumor in children, covers a wide spectrum of tumors with significant differences in anatomical localization, organ or vessel involvement, and tumor biology. Complete surgical resection of the primary tumor is an important part of NBL treatment, but maybe hazardous, prone to complications and its role in high-risk disease remains debated. Various surgical guidelines exist within the protocols of the different cooperative groups, although there is no standardized operative report form to document the surgical treatment of NBL. METHODS: After analyzing the treatment protocols of the SIOP Europe International Neuroblastoma Study Group, Children's Oncology Group, and Gesellschaft fuer Paediatrische Onkologie und Haematologie - German Association of Pediatric Oncology and Haematology pediatric cooperative groups, important variables were defined to completely describe surgical biopsy and resection of NBL and their outcomes. All variables were discussed within the Surgical Committees of SIOP Europe International Neuroblastoma Study Group, Children's Oncology Group, and Gesellschaft fuer Paediatrische Onkologie und Haematologie - German Association of Pediatric Oncology and Haematology. Thereafter, joint meetings were organized to obtain intercontinental consensus. RESULTS: The "International Neuroblastoma Surgical Report Form" provides a structured reporting tool for all NBL surgery, in every anatomical region, documenting all Image Defined Risk Factors and structures involved, with obligatory reporting of intraoperative and 30 day-postoperative complications. CONCLUSION: The International Neuroblastoma Surgical Report Form is the first universal form for the structured and uniform reporting of NBL-related surgical procedures and their outcomes, aiming to facilitate the postoperative communication, treatment planning and analysis of surgical treatment of NBL.


Assuntos
Formulários como Assunto , Neuroblastoma/cirurgia , Projetos de Pesquisa/normas , Oncologia Cirúrgica/normas , Criança , Humanos , Cooperação Internacional
7.
Eur J Pediatr Surg ; 32(4): 357-362, 2022 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-34560787

RESUMO

INTRODUCTION: The Nuss procedure is the most common and preferred operative correction of pectus excavatum. Surgeon preference and patient factors can result in variations in Nuss procedure technique. We hypothesize that certain techniques are associated with increased risk of complications. MATERIALS AND METHODS: We performed a single-center retrospective review of Nuss operations from 2016 to 2020. Variations in intraoperative techniques included sternal elevator (SE) use, number of bars placed, and usage of bilateral stabilizing sutures. Patient demographics, intraoperative data, and postoperative outcomes were reported as median with interquartile ranges or percentages. Statistical significance (p < 0.05) was determined with Wilcoxon's rank-sum and chi-square tests. Multivariate analysis was performed to control for introduction of intercostal nerve cryoablation and surgeon volume, and reported as odds ratio with 95% confidence interval. RESULTS: Two hundred and sixty-five patients were identified. Patients repaired with two bars were older with a larger Haller index (HI). Patient demographics were not significantly different for SE or stabilizing suture use. Placement of two bars was associated with significantly increased risk of readmission. Similarly, SE use was associated with increased risk of pleural effusion and readmission. Finally, the use of bilateral stabilizing sutures resulted in less frequent slipped bars without statistical significance. CONCLUSION: Older patients with a larger HI were more likely to need two bars placed to repair pectus excavatum. Placement of multiple bars and SE use are associated with significantly higher odds of certain complications.


Assuntos
Tórax em Funil , Tórax em Funil/cirurgia , Humanos , Procedimentos Cirúrgicos Minimamente Invasivos/métodos , Complicações Pós-Operatórias/epidemiologia , Complicações Pós-Operatórias/etiologia , Estudos Retrospectivos , Esterno , Resultado do Tratamento
8.
Am J Surg ; 223(1): 106-111, 2022 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-34364653

RESUMO

PURPOSE: We aim to assess the healthcare value achieved from a shared savings program for pediatric appendectomy. METHODS: All appendectomy patients covered by our health plan were included. Quality targets were 15% reduction in time to surgery, length of stay, readmission rate, and patient satisfaction. Quality targets and costs for an appendectomy episode in two 6-month performance periods (PP1, PP2) were compared to baseline. RESULTS: 640 patients were included (baseline:317, PP1:167, PP2:156). No quality targets were met in PP1. Two quality targets were met during PP2: readmission rate (-57%) and patient satisfaction. No savings were realized because the cost reduction threshold (-9%) was not met during PP1 (+1.7%) or PP2 (-0.4%). CONCLUSIONS: Payer-provider partnerships can be a platform for testing value-based reimbursement models. Setting achievable targets, identifying affectable quality metrics, considering case mix index, and allowing sufficient time for interventions to generate cost savings should be considered in future programs.


Assuntos
Apendicectomia/economia , Apendicite/cirurgia , Redução de Custos/estatística & dados numéricos , Seguro de Saúde Baseado em Valor/economia , Adolescente , Apendicectomia/estatística & dados numéricos , Apendicite/economia , Criança , Pré-Escolar , Grupos Diagnósticos Relacionados/economia , Grupos Diagnósticos Relacionados/estatística & dados numéricos , Humanos , Lactente , Recém-Nascido , Masculino , Readmissão do Paciente/economia , Readmissão do Paciente/estatística & dados numéricos , Projetos Piloto , Seguro de Saúde Baseado em Valor/estatística & dados numéricos
9.
J Pediatr Surg ; 57(8): 1544-1553, 2022 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-34366130

RESUMO

INTRODUCTION: Up to a third of children undergoing partial hepatectomy for primary hepatic malignancies experience at least one perioperative complication, with a presumed deleterious effect on both short- and long-term outcomes. We implemented a multidisciplinary treatment protocol in the management of these patients in order to improve complication rates following partial hepatectomy. METHODS: A retrospective chart review was completed for all patients < 18 years of age who underwent liver resection at our institution between 2002 and 2019 for primary hepatic cancer. Demographic, intraoperative, postoperative, pathologic, and outcome data were analyzed for perioperative complications using the CLASSIC and Clavien-Dindo (CD) scales, event-free survival (EFS) and overall survival (OS). RESULTS: A total of 73 patients were included in the analysis with 33 prior-to and 40 after dedicated provider protocol implementation. Perioperative complication rates decreased from 52% to 20% (p = 0.005) with major complications going from 18% to 10% (p = 0.31). On multivariable logistic regression, protocol implementation was associated with a reduction in any (OR 0.29 [95% CI 0.09 - 0.89]) but not major complications. On multivariate cox models, post protocol implementation was associated with improved event free survival (EFS) (HR 0.19 (0.036 - 0.195). Among patients with a diagnosis of hepatoblastoma (n = 62), the occurrence of a major perioperative complication was associated with a worse EFS (HR=5.45, p = 0.03) on multivariate analysis, however this did not translate into an impact on overall survival. CONCLUSIONS: Our results demonstrate that, for children with primary liver malignancies, a dedication of patients to high-volume surgeons can improve rates of complications of liver resections and may improve the oncological outcome of hepatoblastoma.


Assuntos
Hepatoblastoma , Neoplasias Hepáticas , Criança , Hepatectomia/métodos , Hepatoblastoma/patologia , Hepatoblastoma/cirurgia , Humanos , Neoplasias Hepáticas/patologia , Neoplasias Hepáticas/cirurgia , Complicações Pós-Operatórias/epidemiologia , Complicações Pós-Operatórias/etiologia , Complicações Pós-Operatórias/cirurgia , Estudos Retrospectivos , Resultado do Tratamento
10.
J Pediatr Surg ; 57(8): 1538-1543, 2022 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-34281709

RESUMO

PURPOSE: Ganglioneuroma (GN) and ganglioneuroblastoma-intermixed (GNB-I) represent benign variants of neuroblastic tumors in children; however, differentiating from more aggressive histological variants of GNB including the nodular subtype (GNB-N) prior to resection can be challenging, even with biopsy. Currently, no standard treatment guidelines exist. The purpose of this study was to identify pre-operative characteristics of benign neuroblastic tumors and evaluate outcomes for patients who underwent surgical resection or observation. METHODS: Retrospective chart review of children treated at a single institution between 2009 and 2019 for non-metastatic tumor with a tissue diagnosis of GN, GNB-N or GNB-I. Demographics, imaging, labs, operative details and outcomes were recorded and analyzed. RESULTS: Of 53 patients, 45% were male. The most common tumor location was abdomen (49%), followed by thorax (34%). Forty-five percent had at least one image defined risk factor. Biopsy was performed in 32% (17/53) and upfront surgery in 68% (36/53). Three patients (3/53, 5.6%) with biopsy demonstrating GN tumors were observed due to high surgical risk. Pathology of resected specimens demonstrated GN in 52% (26/50) and GNB-I or GNB-N in 48% (24/50). The majority of GNB tumors (75% (18/24) were GNB-I and 25% (6/24) were GNB-N. Therefore, 88% of the resected tumors were benign spectrum neuroblastic tumors (GN & GNB-I). Seven (7/50, 14%) patients experienced perioperative complication (temporary paralysis, Horner's syndrome, chylothorax, vocal cord paralysis). Recurrence was noted in 1 patient with GN (1/50, 2%) and 3 with GNB-N (3/50, 6%). There were no tumor-related deaths. Patients with GN were older than those with GNB (8.8 years (IQR 6-11.25) vs 5.6 years for GN (IQR 3-7); p = 0.01). GNB tumors were also more likely to have calcifications on imaging (63% vs. 38%, p = .01) and more commonly had MIBG avidity (88% vs 66%, p = .04). There were no significant differences in tumor size or symptoms at presentation. CONCLUSIONS: In children with neuroblastic tumors, older age, CT without tumor calcifications, lack of MIBG avidity, and/or normal urine catecholamines may indicate benign GN. Close observation could be considered for asymptomatic patients meeting these criteria with biopsy-proven GN, with resection reserved for progressive growth or symptom development. However, larger, multicenter studies are needed for further validation. LEVEL OF EVIDENCE: IV.


Assuntos
Ganglioneuroblastoma , Ganglioneuroma , Neuroblastoma , Criança , Feminino , Ganglioneuroblastoma/diagnóstico , Ganglioneuroblastoma/patologia , Ganglioneuroblastoma/cirurgia , Ganglioneuroma/diagnóstico , Ganglioneuroma/patologia , Ganglioneuroma/cirurgia , Humanos , Masculino , Neuroblastoma/diagnóstico , Neuroblastoma/patologia , Neuroblastoma/cirurgia , Estudos Retrospectivos
11.
J Laparoendosc Adv Surg Tech A ; 31(12): 1475-1479, 2021 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-34788157

RESUMO

Background: Thoracic inlet (TI) tumors are rare, and can be particularly challenging to resect due to proximity to mediastinal vessels and nerves. Traditional resection is typically performed through "trapdoor" or sternoclavicular incisions. The purpose of our study was to evaluate the feasibility and effectiveness of thoracoscopic resection of this group of tumors. Methods: We performed a single-center retrospective chart review for children who presented with TI neuroblastic tumors between 2011 and 2020. Demographics, tumor characteristics, treatment, operative complications, and outcomes were collected and analyzed. Results: Eight patients were identified. The median age at diagnosis was 13 months (interquartile range [IQR] 6-32) with median tumor size at diagnosis of 4.1 cm (IQR 3.6-4.4). Neoadjuvant chemotherapy was given in 50% (4/8) with 38% (3/8) undergoing upfront surgery; 1 patient was observed without chemotherapy or surgery. Ultimately, 6 patients had thoracoscopic resection. For thoracoscopic resections, median intraoperative estimated blood loss was 15 mL (IQR 10-28), median operative room time was 199 minutes (IQR 152-259), and median hospital length of stay was 2 days (IQR 2-3). There were two complications: one recurrent laryngeal nerve injury and one new-onset Horner's syndrome. Complete gross total resection was achieved for all children and there were no recurrences or mortalities with a median follow-up of 3 years. Conclusion: Thoracoscopic resection for TI neuroblastic tumors is feasible with minimal morbidity and can lead to adequate oncological resection.


Assuntos
Neuroblastoma , Neoplasias Torácicas , Baías , Criança , Pré-Escolar , Humanos , Lactente , Neuroblastoma/cirurgia , Duração da Cirurgia , Complicações Pós-Operatórias , Estudos Retrospectivos , Neoplasias Torácicas/cirurgia , Toracoscopia , Resultado do Tratamento
12.
J Pediatr Surg ; 56(1): 104-109, 2021 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-33139029

RESUMO

BACKGROUND/PURPOSE: Pediatric oncology patients often undergo open operations for tumor resection, and epidural catheters are commonly utilized for pain control. Our purpose was to evaluate whether a subcutaneous analgesic system (SAS) provides equivalent post-operative pain control. METHODS: An IRB approved, retrospective chart review of children age <18 undergoing open abdominal, pelvic or thoracic surgery for tumor resection between 2017 and 2019 who received either epidural or SAS for post-operative pain control was performed. Comparisons of morphine milligram equivalents (MME), pain scores, and post-operative course were made using parametric and non-parametric analyses. RESULTS: Of 101 patients, median age was 7 years (2 months-17.9 years). There were 65 epidural and 36 SAS patients. Transverse laparotomy was the most common incision (41%), followed by thoracotomy (29%). Pain scores, MME, urinary catheter days, and post-operative length of stay (LOS) were similar between the two groups. Urinary catheter use was more common in epidural patients (70% vs 30%, p = <0.001). SAS patients had faster time to ambulation and time to regular diet by 1 day (p = 0.02). Epidural patients more commonly had a complication with the pain device (20% vs 3%, p = 0.02) and were more likely to be discharged with narcotics (60% vs. 40%, p = 0.04). Charges associated with the hospital stay were similar between the two groups. CONCLUSION: In pediatric oncology patients undergoing open abdominal, pelvic, and thoracic surgery, SAS may provide similar pain control to epidural, but with faster post-operative recovery, fewer complications, and less discharge narcotic use. A prospective study is needed to validate these results. TYPE OF STUDY: Retrospective Comparative LEVEL OF EVIDENCE: Level III.


Assuntos
Analgesia Epidural , Neoplasias , Analgésicos/uso terapêutico , Analgésicos Opioides/uso terapêutico , Criança , Humanos , Neoplasias/complicações , Neoplasias/cirurgia , Dor Pós-Operatória/tratamento farmacológico , Estudos Prospectivos , Estudos Retrospectivos
13.
Pediatr Blood Cancer ; 67(10): e28417, 2020 10.
Artigo em Inglês | MEDLINE | ID: mdl-32729196

RESUMO

BACKGROUND/OBJECTIVES: Standard supportive care during induction therapy for high-risk neuroblastoma (HR-NBL) includes primary prophylactic granulocyte colony-stimulating factor (G-CSF) aimed at limiting duration of neutropenia, reducing infection risk, and minimizing treatment delays. Preclinical models suggest that G-CSF promotes maintenance of neuroblastoma cancer stem cells and may reduce the efficacy of chemotherapy. This study's objective was to determine the safety and feasibility of administering induction chemotherapy without routine use of prophylactic G-CSF. DESIGN/METHODS: Children with newly diagnosed HR-NBL received six-cycle induction chemotherapy regimen without prophylactic G-CSF in four cycles. G-CSF was administered for stem cell mobilization after cycle 3 and granulocyte-monocyte colony-stimulating factor after cycle 5 prior to surgical resection of primary disease. The primary outcome measure was the incidence of grade 3 or higher infection. We hypothesized that the per patient infection rate would be comparable to our institutional baseline rate of 58% in patients with HR-NBL receiving induction chemotherapy with prophylactic growth factor support. The trial used an A'Hern single-stage design. RESULTS: Twelve patients with HR-NBL received 58 cycles of chemotherapy on study. Three patients completed the entire six-cycle regimen with no infections. Nine patients experienced grade 3 infections (bacteremia four, urinary tract infection two, skin/soft tissue infection three). No patients experienced grade 4 infections or required intensive care treatment for infection. CONCLUSION: A greater than expected number of serious bacterial infections were observed during administration of induction chemotherapy for HR-NBL without primary prophylactic G-CSF. These results support continued prophylactic administration growth factor during induction chemotherapy.


Assuntos
Infecções Bacterianas/prevenção & controle , Fator Estimulador de Colônias de Granulócitos/administração & dosagem , Mobilização de Células-Tronco Hematopoéticas/métodos , Quimioterapia de Indução/métodos , Neuroblastoma/tratamento farmacológico , Neutropenia/prevenção & controle , Adolescente , Criança , Pré-Escolar , Feminino , Seguimentos , Humanos , Lactente , Masculino , Neuroblastoma/patologia , Projetos Piloto , Prognóstico , Estudos Prospectivos , Taxa de Sobrevida , Tempo para o Tratamento
14.
Pediatr Pulmonol ; 55(8): 2074-2081, 2020 08.
Artigo em Inglês | MEDLINE | ID: mdl-32511892

RESUMO

Patients with pulmonary Langerhans cell histiocytosis (LCH) typically have a benign course but may have extensive cystic lung disease with rare life-threatening complications including multiple and recurrent pneumothoraces and respiratory failure. We report seven severely affected pediatric patients treated with chemotherapy, aggressive chest tube management, and pleurodesis of whom five survived. Patients with extraordinary amounts of pulmonary cystic disease and multiple pneumothoraces due to LCH can have remarkable, curative outcomes with early recognition, optimal LCH-directed therapy, and supportive care.


Assuntos
Histiocitose/terapia , Pneumopatias/terapia , Pneumotórax/terapia , Adolescente , Tubos Torácicos , Pré-Escolar , Feminino , Humanos , Lactente , Recém-Nascido , Masculino , Pleurodese
15.
J Surg Res ; 247: 34-51, 2020 03.
Artigo em Inglês | MEDLINE | ID: mdl-31810638

RESUMO

BACKGROUND: Wilms tumor accounts for more than 90% of all malignant kidney neoplasms in children. Survival after diagnosis and treatment is excellent in most high-income countries. Low- and middle-income countries (LMICs) continue to struggle with Wilms tumor detection and treatment. The purpose of this study was to compare the global incidence and outcomes of Wilms tumor. MATERIAL AND METHODS: Wilms tumor incidence data from the World Health Organization (WHO), International Incidence of Childhood Cancer, Volume III, was analyzed according to world region and country socioeconomic status using descriptive statistics and independent-sample Kruskal-Wallis Test. A literature review was also performed to assess outcomes and identify common themes. RESULTS: Wilms tumor was most common in children aged 0-4 y (median incidence 15.1 [IQR 11.8-18.7] ASR/million). High-income countries reported significantly higher median incidence than middle-income countries (8.6 [7.4-9.3] versus 6.1 [4.9-8.7] ASR/million; P < 0.01), although low-income countries reported the highest median incidence overall (9.8 [6.2-16.4] ASR/million). Low-income countries had the fewest countries with registries (n = 6). Overall survival ranged from 70% to 97% in high-income countries, 61%-94% in upper-middle-income countries, 0%-85% in lower-middle-income countries, and 25%-53% in low-income countries. Delay in diagnosis, lack of available treatment, and inadequate follow up contributed to the large variations in outcomes. CONCLUSIONS: Reported Wilms tumor incidence is highest in low-income countries, and these are also the countries that have the lowest survival. Lack of significance may reflect incomplete and absent data reporting from lower income countries. Accurate and comprehensive registries are the first steps to appropriate resource allocation in order to improve outcomes for this highly curable childhood malignancy.


Assuntos
Carga Global da Doença/estatística & dados numéricos , Disparidades nos Níveis de Saúde , Neoplasias Renais/epidemiologia , Tumor de Wilms/epidemiologia , Criança , Conjuntos de Dados como Assunto , Saúde Global/economia , Saúde Global/estatística & dados numéricos , Humanos , Incidência , Neoplasias Renais/economia , Neoplasias Renais/terapia , Sistema de Registros/estatística & dados numéricos , Alocação de Recursos , Classe Social , Taxa de Sobrevida , Resultado do Tratamento , Tumor de Wilms/economia , Tumor de Wilms/terapia
16.
Mol Cancer Ther ; 18(6): 1045-1056, 2019 06.
Artigo em Inglês | MEDLINE | ID: mdl-30962318

RESUMO

Neuroblastoma is the most common extracranial malignant solid tumor in children, and drug resistance is a major reason for poor outcomes. Elevated proteasome activity plays an important role in neuroblastoma tumor development and resistance to conventional chemotherapy. Ubiquitin-specific protease 14 (USP14), one of three deubiquitinases associated with the regulatory subunit of the proteasome, is emerging as a potential therapeutic target in multiple tumor types. However, the role of USP14 in neuroblastoma is yet to be elucidated. We found that USP14 inhibition in neuroblastoma via knockdown or a specific inhibitor such as b-AP15 suppressed cell proliferation by inducing cell apoptosis. Furthermore, b-AP15 significantly inhibited neuroblastoma tumor growth in NGP and SH-SY5Y xenograft mouse models. For combination treatment, b-AP15 plus conventional chemotherapeutic agents such as doxorubicin or VP-16 resulted in synergistic antitumor effects on neuroblastoma. Our study demonstrates that USP14 is required for cell viability and is a novel therapeutic target in neuroblastoma. Moreover, USP14 inhibition may add value in combination therapy due to its powerful synergistic effects in treating neuroblastoma.


Assuntos
Proliferação de Células/efeitos dos fármacos , Proliferação de Células/genética , Neuroblastoma/tratamento farmacológico , Piperidonas/farmacologia , Ubiquitina Tiolesterase/antagonistas & inibidores , Animais , Antibióticos Antineoplásicos/uso terapêutico , Antineoplásicos Fitogênicos/uso terapêutico , Apoptose/efeitos dos fármacos , Apoptose/genética , Linhagem Celular Tumoral , Sobrevivência Celular/efeitos dos fármacos , Sobrevivência Celular/genética , Intervalo Livre de Doença , Doxorrubicina/uso terapêutico , Resistencia a Medicamentos Antineoplásicos/efeitos dos fármacos , Sinergismo Farmacológico , Etoposídeo/uso terapêutico , Feminino , Seguimentos , Técnicas de Silenciamento de Genes , Células HEK293 , Humanos , Camundongos , Camundongos Nus , Neuroblastoma/patologia , Piperidonas/uso terapêutico , Desdobramento de Proteína/efeitos dos fármacos , Carga Tumoral/efeitos dos fármacos , Ubiquitina Tiolesterase/genética , Ubiquitina Tiolesterase/metabolismo , Ensaios Antitumorais Modelo de Xenoenxerto
17.
J Pediatr Surg ; 54(10): 2103-2106, 2019 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-30954230

RESUMO

BACKGROUND: Pancreatic islet cell tumors are rare in adolescents, and most studies published to date focus on older patients. We utilized a national database to describe the histology and clinical pattern of pancreatic islet cell tumors in adolescent and young adult (AYA) patients, and to compare AYAs to older adults. We hypothesized that AYAs with pancreatic islet cell tumors would have better overall survival. METHODS: The National Cancer Data Base (NCDB, 1998-2012) was queried for AYA patients (15-39 years) with a pancreatic islet cell tumor diagnosis. Demographics, tumor characteristics, treatment modalities, and outcomes were abstracted and compared to adults (≥40 years). RESULTS: 383 patients (56.4% female, 65% non-Hispanic Whites) were identified, with a median age of 27 (IQR 16-34) years. Islet cell carcinoma was the most common histology. Of patients with known stage of disease, 49% presented with early stage (I or II). Seventy percent of patients underwent surgical resection, including local excision 44%, Whipple procedure 37.5%, or total pancreatectomy 19%. Chemotherapy was utilized in 27% and radiotherapy in 7%. All-cause mortality was 36%. AYA patients underwent more extensive resections (p = 0.001) and had lower mortality rates (p < 0.001), with no differences in tumor stage or use of adjuvant therapies, when compared to adults. CONCLUSIONS: AYA patients with pancreatic islet cell tumors had comparable utilization of adjuvant therapies but underwent more extensive resections and demonstrated a higher overall survival rate than adult counterparts. Further investigation into approaches to earlier diagnosis and tailoring of multimodality therapy of these neoplasms in the AYA population is needed. LEVELS OF EVIDENCE: Prognostic Study, Level II - retrospective study.


Assuntos
Tumores Neuroendócrinos/cirurgia , Neoplasias Pancreáticas/cirurgia , Adenoma de Células das Ilhotas Pancreáticas , Adolescente , Adulto , Fatores Etários , Idoso , Terapia Combinada , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Estadiamento de Neoplasias , Tumores Neuroendócrinos/mortalidade , Tumores Neuroendócrinos/patologia , Pancreatectomia/estatística & dados numéricos , Neoplasias Pancreáticas/mortalidade , Neoplasias Pancreáticas/patologia , Pancreaticoduodenectomia/estatística & dados numéricos , Prognóstico , Estudos Retrospectivos , Taxa de Sobrevida , Estados Unidos/epidemiologia , Adulto Jovem
18.
J Pediatr Surg ; 54(5): 1029-1034, 2019 May.
Artigo em Inglês | MEDLINE | ID: mdl-30824240

RESUMO

PURPOSE: We sought to compare the presentation, management, and outcomes in gastric adenocarcinoma cancer for pediatric and adult patients. METHODS: Using the 2004 to 2014 National Cancer Database (NCDB), patients ≤21 years (pediatric) were retrospectively compared to >21 years (adult). Chi-squared tests were used to compare categorical variables, and Cox regression was used to estimate hazard ratios (HR) for survival differences. RESULTS: Of the 129,024 gastric adenocarcinoma cases identified, 129 (0.10%) occurred in pediatric patients. Pediatric cases presented with more advanced disease, including poorly differentiated tumors (81% vs 65%, p = 0.006) and stage 4 disease (56% vs 41%, p = 0.002). Signet ring adenocarcinoma comprised 45% of cases in the pediatric group as compared to 20% of cases in the adults (P < 0.001). Similar proportions in both groups underwent surgery. However, near-total gastrectomy was more common in the pediatric group (16% vs 6%, p < 0.001). The proportions of patients with negative margins, nodal examination, and presence of positive nodes were similar. There was no overall survival difference between the two age groups (HR 0.92, 95% Confidence interval 0.73-1.15). CONCLUSION: While gastric adenocarcinoma in pediatric patients present with a more advanced stage and poorly differentiated tumors compared to adults, survival appears to be comparable. TYPE OF STUDY: Retrospective cohort study. LEVEL OF EVIDENCE: III.


Assuntos
Adenocarcinoma , Neoplasias Gástricas , Adenocarcinoma/diagnóstico , Adenocarcinoma/mortalidade , Adenocarcinoma/patologia , Adenocarcinoma/terapia , Adulto , Fatores Etários , Idoso , Criança , Terapia Combinada , Bases de Dados Factuais , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Estadiamento de Neoplasias , Estudos Retrospectivos , Neoplasias Gástricas/diagnóstico , Neoplasias Gástricas/mortalidade , Neoplasias Gástricas/patologia , Neoplasias Gástricas/terapia , Análise de Sobrevida , Resultado do Tratamento
19.
J Pediatr Surg ; 54(6): 1108-1111, 2019 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-30905415

RESUMO

BACKGROUND/PURPOSE: Elective ambulatory surgical care traditionally involves three independent visits. Single-Visit Surgery (SVS) is an alternative surgical model that consolidates care into one visit. Evaluation of the effect of this novel program on hospital operations is limited. The objective of this study was to analyze SVS from an institutional perspective. METHODS: We retrospectively reviewed patients scheduled for SVS at a freestanding children's hospital between January 2016 and August 2017. Data collected included clinic "no show" rates, operating room (OR) utilization, reimbursement rates, and postoperative visits. RESULTS: There were 89 patients scheduled for SVS, of which 63% (n=56) were male, and the median age was 6 years [IQR, 4-9]. The SVS clinic "no show" rate was 2% (n=2) compared to the pediatric surgery clinic "no show" rate of 11% (p=0.01). The SVS OR block utilization rate was 90%. Payment was received from third-party payors for 92% of consultations and 100% of operative procedures without securing prior authorization. Postoperatively 25% (n=17) of patients presented to clinic for follow-up, and one child presented to the emergency department for vomiting. There were no hospital admissions. CONCLUSION: Single-Visit Surgery is an alternative model of ambulatory surgical care that improves institutional efficiency while also enhancing the patient experience. TYPE OF STUDY: Retrospective cohort review LEVEL OF EVIDENCE: III.


Assuntos
Instituições de Assistência Ambulatorial/estatística & dados numéricos , Procedimentos Cirúrgicos Ambulatórios/estatística & dados numéricos , Procedimentos Cirúrgicos Eletivos/estatística & dados numéricos , Criança , Pré-Escolar , Feminino , Humanos , Masculino , Estudos Retrospectivos
20.
J Surg Res ; 236: 119-123, 2019 04.
Artigo em Inglês | MEDLINE | ID: mdl-30694744

RESUMO

BACKGROUND: In patients requiring gastrostomies, ventriculoperitoneal (VP) shunts are a frequently encountered comorbidity. The objective of this study is to evaluate the postoperative management of children with VP shunts that undergo laparoscopic gastrostomy placement and determine their incidence of complications. MATERIALS AND METHODS: Children 18 y old or younger who underwent laparoscopic gastrostomy placement at a freestanding academic children's hospital between January 2014 and October 2016 were reviewed. Data collected included demographics, management, and outcomes. Patients were compared based on their presence of a VP shunt before laparoscopic gastrostomy. Statistical analysis was performed using chi square, Fisher's exact, and Wilcoxon rank-sum tests. RESULTS: We reviewed the medical records of 270 children that underwent laparoscopic gastrostomy placement by 15 pediatric surgeons. Of these, 9% (25) had a previously placed VP shunt. In comparing patients with a VP shunt with those without a VP shunt, there was no significant difference in median age (4 versus 3 y, P = 0.92), gender (48% versus 51% males, P = 0.80), body mass index (15 versus 16, P = 0.69), preoperative diet (48% versus 47% nasogastric tube dependent, P = 0.60), or procedure time (43 versus 42 min, P = 0.37). The postoperative management of these children was similar: day of initiation of postoperative feeds (84% versus 73% on postoperative day #1, P = 0.70), method of initiation of feeds (60% versus 55% continuous, P = 0.25), and type of initial feeds (83% versus 71% Pedialyte, P = 0.24). Similarly, there was no difference in hospital length of stay, return to the emergency department, or postoperative complications within 90 d (P > 0.05). CONCLUSIONS: Children with ventriculoperitoneal shunts do not have a higher rate of immediate complications after laparoscopic gastrostomy placement and may be managed similar to other children in the postoperative period.


Assuntos
Nutrição Enteral/métodos , Gastrostomia/efeitos adversos , Laparoscopia/efeitos adversos , Complicações Pós-Operatórias/epidemiologia , Derivação Ventriculoperitoneal/efeitos adversos , Criança , Pré-Escolar , Comorbidade , Transtornos de Deglutição/epidemiologia , Transtornos de Deglutição/terapia , Feminino , Gastrostomia/métodos , Humanos , Incidência , Lactente , Laparoscopia/métodos , Masculino , Doenças do Sistema Nervoso/epidemiologia , Doenças do Sistema Nervoso/cirurgia , Complicações Pós-Operatórias/etiologia , Estudos Retrospectivos
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