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1.
J Pediatr Surg ; 2024 Mar 05.
Artículo en Inglés | MEDLINE | ID: mdl-38548494

RESUMEN

BACKGROUND: Limb-sparing surgery is the standard of care for primary bone tumors. However, such procedures are associated with high rates of wound complications, specifically in lower-extremity surgeries. Therefore, identifying and implementing interventions to minimize the likelihood of wound complications after limb-sparing resection of the lower extremity is crucial. METHODS: Patients who underwent limb-sparing osteosarcoma or Ewing sarcoma resection during a 7-year period at a single institution were retrospectively reviewed. Data were collected on 39 patients who underwent limb-sparing resection of the femur. Patient demographics, tumor characteristics, and perioperative and postoperative data were extracted and analyzed. Patients who underwent resection before April 2017 received conventional postoperative incision dressings. Starting in April 2017, patients received vacuum-assisted closure (VAC) with the 3 M™ Prevena VAC system after surgical closure. Eighteen patients received conventional postoperative incision dressing, and 21 received incisional wound VAC. A wound complication was defined as any Clavien-Dindo classification greater than 0 within a 28-day postoperative period. RESULTS: Patients who received postoperative incisional wound VAC had lower rates of wound complications than those who received conventional incision dressings (14% vs. 50%; p = 0.035). Additionally, patients in whom wound complications developed had a longer average hospital stay than those without wound complications (5 days vs. 4 days; p = 0.029). CONCLUSIONS: Wound complications prolong the hospital stay and can delay adjuvant chemotherapy for bone tumors. The use of postoperative incisional wound VAC is associated with less likelihood of wound complications and should be considered in any high-risk surgical closure. LEVEL OF EVIDENCE: Level III Treatment Study.

2.
J Pediatr Surg ; 2024 Mar 01.
Artículo en Inglés | MEDLINE | ID: mdl-38519388

RESUMEN

BACKGROUND AND AIMS: As survival rates in childhood cancer progress significantly, health outcomes in adulthood are pivotal to quality of life (QoL). Female patients undergoing chemotherapy and radiation for childhood cancer may experience adverse effects such as gonadotoxicity-related ovarian insufficiency. Ovarian tissue cryopreservation (OTC) is well studied in adults, but has only recently started to be explored in an effort to preserve fertility in young patients with childhood cancer. This systematic review aims to critically highlight contemporary outcomes of cryopreservation in female pediatric cancer patients. METHODS: A systematic search was conducted in PubMed, Embase, and Web of Science databases to identify English-language full text articles and abstracts published between 2004 and 2022 describing cryopreservation among female children (0-21 years old) with cancer. Abstracts and full-text articles were screened for inclusion. Subsequently, data from eligible studies was extracted and analyzed. Descriptive statistics were utilized to estimate overall outcomes of cryopreservation. RESULTS: Of 104 abstracts and 34 full-text articles, 12 studies were included. Data was collected from 7 world countries and involved some 612 pediatric and adolescent patients with malignant disease. Most common cancers included hematological malignant disease (81%), CNS nervous system malignant tumors (56%), and sarcomas (39%). Of the 6 studies with full reporting, OTC was undertaken in 501 patients, and 5.9% (30/501) of these patients underwent ovarian tissue transplantation (OTT). After OTT, 27 patients desired pregnancy and 33% (9/27) became pregnant. Six of these 9 patients (67%) had live births. CONCLUSIONS: Preliminary analysis showed that OTC has been successfully performed but not yet studied thoroughly in pediatric cancer patients in a longitudinal manner. This study has further shown that cryopreservation outcomes are mainly reported among adult patients living in high income countries, demonstrating a crucial need for long-term outcome studies focused on pediatric and prepuberal OTC, subsequent OTT, and potential pregnancy. This work is considered critical to aid standardize recommendations of fertility preservation in childhood cancer patients and to better inform the efficacy of these procedures to benefit patients in world nations of all fiscal income levels. LEVEL OF EVIDENCE: Level III.

3.
Lancet Glob Health ; 12(2): e331-e340, 2024 Feb.
Artículo en Inglés | MEDLINE | ID: mdl-38190831

RESUMEN

The true global burden of paediatric critical illness remains unknown. Studies on children with life-threatening conditions are hindered by the absence of a common definition for acute paediatric critical illness (DEFCRIT) that outlines components and attributes of critical illness and does not depend on local capacity to provide critical care. We present an evidence-informed consensus definition and framework for acute paediatric critical illness. DEFCRIT was developed following a scoping review of 29 studies and key concepts identified by an interdisciplinary, international core expert panel (n=24). A modified Delphi process was then done with a panel of multidisciplinary health-care global experts (n=109) until consensus was reached on eight essential attributes and 28 statements as the basis of DEFCRIT. Consensus was reached in two Delphi rounds with an expert retention rate of 89%. The final consensus definition for acute paediatric critical illness is: an infant, child, or adolescent with an illness, injury, or post-operative state that increases the risk for or results in acute physiological instability (abnormal physiological parameters or vital organ dysfunction or failure) or a clinical support requirement (such as frequent or continuous monitoring or time-sensitive interventions) to prevent further deterioration or death. The proposed definition and framework provide the conceptual clarity needed for a unified approach for global research across resource-variable settings. Future work will centre on validating DEFCRIT and determining high priority measures and guidelines for data collection and analysis that will promote its use in research.


Asunto(s)
Cuidados Críticos , Enfermedad Crítica , Humanos , Niño , Adolescente , Consenso , Enfermedad Crítica/terapia , Técnica Delphi , Recolección de Datos
4.
Ann Surg ; 279(3): 536-541, 2024 Mar 01.
Artículo en Inglés | MEDLINE | ID: mdl-37487006

RESUMEN

OBJECTIVE: To determine the impact of nodal basin ultrasound (US) surveillance versus completion lymph node dissection (CLND) in children and adolescents with sentinel lymph node (SLN) positive melanoma. BACKGROUND: Treatment for children and adolescents with melanoma are extrapolated from adult trials. However, there is increasing evidence that important clinical and biological differences exist between pediatric and adult melanoma. METHODS: Patients ≤18 years diagnosed with cutaneous melanoma between 2010 and 2020 from 14 pediatric hospitals were included. Data extracted included demographics, histopathology, nodal basin strategies, surveillance intervals, and survival information. RESULTS: Of 252 patients, 90.1% (n=227) underwent SLN biopsy (SLNB), 50.9% (n=115) had at least 1 positive node. A total of 67 patients underwent CLND with 97.0% (n=65/67) performed after a positive SLNB. In contrast, 46 total patients underwent US observation of nodal basins with 78.3% (n=36/46) of these occurring after positive SLNB. Younger patients were more likely to undergo US surveillance (median age 8.5 y) than CLND (median age 11.3 y; P =0.0103). Overall, 8.9% (n=21/235) experienced disease recurrence: 6 primary, 6 nodal, and 9 distant. There was no difference in recurrence (11.1% vs 18.8%; P =0.28) or death from disease (2.2% vs 9.7%; P =0.36) for those who underwent US versus CLND, respectively. CONCLUSIONS: Children and adolescents with cutaneous melanoma frequently have nodal metastases identified by SLN. Recurrence was more common among patients with thicker primary lesions and positive SLN. No significant differences in oncologic outcomes were observed with US surveillance and CLND following the identification of a positive SLN.


Asunto(s)
Melanoma , Ganglio Linfático Centinela , Neoplasias Cutáneas , Adulto , Humanos , Adolescente , Niño , Melanoma/diagnóstico por imagen , Melanoma/cirugía , Melanoma/patología , Neoplasias Cutáneas/diagnóstico por imagen , Neoplasias Cutáneas/cirugía , Ganglio Linfático Centinela/patología , Recurrencia Local de Neoplasia/patología , Escisión del Ganglio Linfático , Biopsia del Ganglio Linfático Centinela , Estudios Retrospectivos
5.
Pediatr Blood Cancer ; 71(2): e30789, 2024 Feb.
Artículo en Inglés | MEDLINE | ID: mdl-38015091

RESUMEN

BACKGROUND: Image-guided core-needle biopsy (IGCNB) is a widely used and valuable clinical tool for tissue diagnosis of pediatric neuroblastoma. However, open surgical biopsy remains common practice even if children undergo more invasive and painful procedures. This review aims to determine the diagnostic accuracy and safety of IGCNBs in pediatric patients with neuroblastoma. METHODS: We conducted a systematic review of peer-reviewed original articles published between 1980 and 2023, by searching "pediatric oncology," "biopsy," "interventional radiology," and "neuroblastoma." Exclusion criteria were patients older than 18 years, studies concerning non-neurogenic tumors, case reports, and language other than English. Both the systematic review and meta-analysis were conducted according to the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) statement. RESULTS: A total of 533 abstracts articles were analyzed. Of these, eight retrospective studies met inclusion criteria (490 infants, 270 surgical biopsies [SB], 220 image-guided biopsies). Tissue adequacy for primary diagnosis (SB: n = 265, 98%; IGCNB: n = 199, 90%; p = .1) and biological characterization (SB: n = 186, 95%; IGCNB: n = 109, 89%; p = .15) was similar with both biopsy techniques, while intraoperative transfusion rate (SB: n = 51, 22%; IGCNB: n = 12, 6%; p = .0002) and complications (%) (SB: n = 58, 21%; IGCNB: n = 14, 6%; p = .005) were higher with surgical biopsy. Length of stay was similar in both groups; however, no additional data about concurrent diagnostic or treatment procedures were available in the analyzed studies. CONCLUSIONS: IGCNB is a safe and effective strategic approach for diagnostic workup of NB and should be considered in preferance to SB wherever possible.


Asunto(s)
Neuroblastoma , Oncología Quirúrgica , Lactante , Niño , Humanos , Estudios Retrospectivos , Neuroblastoma/diagnóstico , Neuroblastoma/cirugía , Neuroblastoma/patología , Biopsia Guiada por Imagen
7.
Ann Surg Oncol ; 30(12): 7789-7798, 2023 Nov.
Artículo en Inglés | MEDLINE | ID: mdl-37543553

RESUMEN

BACKGROUND: Fluorescence-guided surgery (FGS) with indocyanine green (ICG) is increasingly applied in pediatric surgical oncology. However, FGS has been mostly reported in case studies of liver or renal tumors. Applying novel technologies in pediatric surgical oncology is more challenging than in adult surgical oncology due to differences in tumor histology, biology, and fewer cases. No consensus exists on ICG-guided FGS for surgically managing pediatric solid tumors. Therefore, we reviewed the literature and discuss the limitations and prospects of FGS. METHODS: Using PRISMA guidelines, we analyzed articles on ICG-guided FGS for childhood solid tumors. Case reports, opinion articles, and narrative reviews were excluded. RESULTS: Of the 108 articles analyzed, 17 (14 retrospective and 3 prospective) met the inclusion criteria. Most (70.6%) studies used ICG to identify liver tumors, but the timing and dose of ICG administered varied. Intraoperative outcomes, sensitivity and specificity, were reported in 23.5% of studies. Fluorescence-guided liver resections resulted in negative margins in 90-100% of cases; lung metastasis was detected in 33% of the studies. In otolaryngologic malignancies, positive margins without fluorescence signal were reported in 25% of cases. Overall, ICG appeared effective and safe for lymph node sampling and nephron-sparing procedures. CONCLUSIONS: Despite promising results from FGS, ICG use varies across the international pediatric surgical oncology community. Underreported intraoperative imaging outcomes and the diversity and rarity of childhood solid tumors hinder conclusive scientific evidence supporting adoption of ICG in pediatric surgical oncology. Further international collaborations are needed to study the applications and limitations of ICG in pediatric surgical oncology.

9.
J Pediatr Surg ; 58(11): 2135-2140, 2023 Nov.
Artículo en Inglés | MEDLINE | ID: mdl-37385908

RESUMEN

BACKGROUND: Minimally invasive surgery is increasingly utilized for resection of neurogenic tumors in children. The minimally invasive retroperitoneoscopic approach was recently reported in children, but transperitoneal laparoscopy still remains the most common technique. The aim of this study is to compare a novel single-port retroperitoneoscopy (SPR) approach for pediatric neurogenic tumor resection with transperitoneal laparoscopic (TPL). METHODS: Patients undergoing minimally invasive resection of abdominal neurogenic tumors over 5 years at a single institution (from 2018 to 2022) were retrospectively reviewed. Tumor volume, stage, presence of image-defined risk factors (IDRFs), neoadjuvant chemotherapy, operative time, estimated blood loss (EBL), length of stay (LOS), complications, oral morphine equivalents per kilogram (OME/Kg), and time to chemotherapy were assessed and compared with SPR and TPL approaches. RESULTS: Eighteen and fifteen patients underwent TPL and SPR, respectively. No significant differences were found between the TPL and SPR approaches in terms of tumor characteristics and IDRFs. Patients who underwent SPR had a significantly faster recovery (p = 0.008) and less postoperative opioid use compared to those in TPL (p = 0.02), thus allowing an enhanced recovery after surgery (ERAS) protocol application. TPL and SPR approaches were performed in presence of IDRFs, respectively in 2 (11%) and 4 patients (27%), with a IDRFs-related conversion in one TPL procedure. Both approaches had one < Grade 3 Clavien Dindo complication, but not requiring further surgery. DISCUSSION: SPR approach can be considered as a safe and feasible minimally invasive approach for the resection of pediatric primary adrenal and neurogenic tumors. The retroperitoneoscopic approach performed using a single port technique represents a promising new frontier of ERAS application in pediatric surgical oncology. CONCLUSION: SPR is a viable surgical alternative in selected neurogenic abdominal tumors with limited IDRFs, thus allowing for the application of ERAS protocols in these patients. LEVEL OF EVIDENCE: Level III.

10.
Pediatr Blood Cancer ; 70(10): e30437, 2023 10.
Artículo en Inglés | MEDLINE | ID: mdl-37194488

RESUMEN

BACKGROUND: Clearing all pulmonary metastases is essential for curing pediatric solid tumors. However, intraoperative localization of such pulmonary nodules can be challenging. Therefore, an intraoperative tool that localizes pulmonary metastases is needed to improve diagnostic and therapeutic resections. Indocyanine green (ICG) real-time fluorescence imaging is used for this purpose in adult solid tumors, but its utility in pediatric solid tumors has not been determined. METHODS: A single-center, open-label, nonrandomized, prospective clinical trial (NCT04084067) was conducted to assess the ability of ICG to localize pulmonary metastases of pediatric solid tumors. Patients with pulmonary lesions who required resection, either for therapeutic or diagnostic intent, were included. Patients received a 15-minute intravenous infusion of ICG (1.5 mg/kg), and pulmonary metastasectomy was performed the following day. A near-infrared spectroscopy iridium system was optimized to detect ICG, and all procedures were photo-documented and recorded. RESULTS: ICG-guided pulmonary metastasectomies were performed in 12 patients (median age: 10.5 years). A total of 79 nodules were visualized, 13 of which were not detected by preoperative imaging. Histologic examination confirmed the following histologies: hepatoblastoma (n = 3), osteosarcoma (n = 2), and one each of rhabdomyosarcoma, Ewing sarcoma, inflammatory myofibroblastic tumor, atypical cartilaginous tumor, neuroblastoma, adrenocortical carcinoma, and papillary thyroid carcinoma. ICG guidance failed to localize pulmonary metastases in five (42%) patients who had inflammatory myofibroblastic tumor, atypical cartilaginous tumor, neuroblastoma, adrenocortical carcinoma, or papillary thyroid carcinoma. CONCLUSIONS: ICG-guided identification of pulmonary nodules is not feasible for all pediatric solid tumors. However, it may localize most metastatic hepatic tumors and high-grade sarcomas in children.


Asunto(s)
Neoplasias de la Corteza Suprarrenal , Carcinoma Corticosuprarrenal , Neoplasias Pulmonares , Nódulos Pulmonares Múltiples , Neuroblastoma , Neoplasias de la Tiroides , Adulto , Humanos , Niño , Verde de Indocianina , Estudios Prospectivos , Cáncer Papilar Tiroideo , Estudios de Factibilidad , Nódulos Pulmonares Múltiples/diagnóstico por imagen , Nódulos Pulmonares Múltiples/cirugía , Nódulos Pulmonares Múltiples/patología , Neoplasias Pulmonares/diagnóstico por imagen , Neoplasias Pulmonares/cirugía , Espectroscopía Infrarroja Corta
11.
J Pediatr Urol ; 19(4): 491-492, 2023 08.
Artículo en Inglés | MEDLINE | ID: mdl-37179199

RESUMEN

BACKGROUND: Retroperitoneoscopic lymphadenectomy is an established surgical approach in adult urology, but rarely described in pediatric population. METHODS: We develop retroperitoneoscopic surgical oncology in children, combining new technology innovations in pediatric surgery such as single site port retroperitoneoscopic in supine position and indocyanine green (ICG). RESULTS: The video describes a step-by-step approach from the ICG injection technique to the lymph-node retroperitoneoscopic harvesting. The video highlights anatomical landmarks and ICG intraoperative lymph nodes findings. Four consecutive surgical procedures were performed in children with paratesticular rhabdomyosarcoma who required staging template retroperitoneal lymph node dissection (RPLND) for staging. All patients were discharged the same day without 30-days postoperative complications. CONCLUSION: Retroperitoneoscopic approach with single port and indocyanine guided lymphatic mapping for template retroperitoneal lymph node dissection (RPLND) is a feasible minimally invasive procedure in children. Combining different technology innovations allows an effective lymph node harvesting with the possibility to offer an enhanced recovery after surgery in pediatric oncology population.


Asunto(s)
Verde de Indocianina , Rabdomiosarcoma , Adulto , Humanos , Niño , Estadificación de Neoplasias , Escisión del Ganglio Linfático/métodos , Ganglios Linfáticos/patología , Rabdomiosarcoma/patología
12.
Pediatr Blood Cancer ; 69(12): e29906, 2022 12.
Artículo en Inglés | MEDLINE | ID: mdl-35929184

RESUMEN

BACKGROUND: Survival of Wilms tumor (WT) is > 90% in high-resource settings but < 30% in low-resource settings. Adapting a standardized surgical approach to WT is challenging in low-resource settings, but a local control strategy is crucial to improving outcomes. OBJECTIVE: Provide resource-sensitive recommendations for the surgical management of WT. METHODS: We performed a systematic review of PubMed and EMBASE through July 7, 2020, and used the GRADE approach to assess evidence and recommendations. RECOMMENDATIONS: Initiation of treatment should be expedited, and surgery should be done in a high-volume setting. Cross-sectional imaging should be done to optimize preoperative planning. For patients with typical clinical features of WT, biopsy should not be done before chemotherapy, and neoadjuvant chemotherapy should precede surgical resection. Also, resection should include a large transperitoneal laparotomy, adequate lymph node sampling, and documentation of staging findings. For WT with tumor thrombus in the inferior vena cava, neoadjuvant chemotherapy should be given before en bloc resection of the tumor and thrombus and evaluation for viable tumor thrombus. For those with bilateral WT, neoadjuvant chemotherapy should be given for 6-12 weeks. Neither routine use of complex hilar control techniques during nephron-sparing surgery nor nephron-sparing resection for unilateral WT with a normal contralateral kidney is recommended. When indicated, postoperative radiotherapy should be administered within 14 days of surgery. Post-chemotherapy pulmonary oligometastasis should be resected when feasible, if local protocols allow omission of whole-lung irradiation in patients with nonanaplastic histology stage IV WT with pulmonary metastasis without evidence of extrapulmonary metastasis. CONCLUSION: We provide evidence-based recommendations for the surgical management of WT, considering the benefits/risks associated with limited-resource settings.


Asunto(s)
Neoplasias Renales , Trombosis , Tumor de Wilms , Niño , Humanos , Neoplasias Renales/cirugía , Neoplasias Renales/tratamiento farmacológico , Tumor de Wilms/cirugía , Tumor de Wilms/patología , Nefrectomía/métodos , Vena Cava Inferior/patología , Estudios Retrospectivos
13.
Pediatr Surg Int ; 38(9): 1335-1340, 2022 Sep.
Artículo en Inglés | MEDLINE | ID: mdl-35849175

RESUMEN

PURPOSE: Placement of a central venous catheter (CVC) is the most commonly performed pediatric procedure. This study aims to develop simple formulas to calculate intravascular length of CVCs prior to insertion to minimize reliance on fluoroscopic and radiographic imaging, which may not be uniformly available. METHODS: We performed a single-institution, retrospective review of 115 pediatric patients who received both CVC placement and computed tomography (CT) imaging of the chest within 3 months of the procedure. Using measurements from the CT imaging, formulas calculating the length of the intravascular component of the CVC based on height and insertion laterality were developed and compared to previously published formulas. These formulas were then trialed prospectively to validate reliability and application. RESULTS: Formulas were developed for right-sided and left subclavian insertion. The right-side formula accurately predicted CVC length in 52.6% of patients, compared to 47.4% by the Andropoulos formula. The left subclavian formula accurately estimated 62.5%, compared to 34.5% by the Stroud formula. CONCLUSIONS: The optimal intravascular length of central venous catheters may be determined by simple formulas based on patient height and insertion site. LEVEL OF EVIDENCE: III.


Asunto(s)
Cateterismo Venoso Central , Catéteres Venosos Centrales , Cateterismo Venoso Central/métodos , Niño , Humanos , Reproducibilidad de los Resultados , Estudios Retrospectivos , Tomografía Computarizada por Rayos X
14.
J Pediatr Surg ; 57(12): 920-925, 2022 Dec.
Artículo en Inglés | MEDLINE | ID: mdl-35794043

RESUMEN

BACKGROUND: Lymph node sampling is critical to surgical staging in Wilms tumor; failure to sample lymph nodes is associated with under-staging and an increased incidence of local relapse. However, no standard lymphatic mapping method is currently being utilized for Wilms tumor to aid identification of regional draining lymph nodes. Herein, we describe the use of fluorescence-guided lymphatic mapping for Wilms tumor. MATERIALS AND METHODS: Two tertiary level referral centers independently began indocyanine green (ICG) fluorescence-guided nodal mapping. In one center, this was achieved with ipsilateral intra-parenchymal (IP) injection of ICG during minimally invasive tumor nephrectomy (MIN) following neoadjuvant chemotherapy and in the other, with Peri­Hilar (PH) injection during upfront, open tumor nephrectomy (ON). Successful lymph node mapping was defined as the presence of fluorescence signal in draining lymph nodes. RESULTS: Eight patients (median age of 2.5 years) underwent fluorescence-guided lymphatic mapping (four IP and four PH injection). Lymphatic mapping was successful in seven patients (88%) including each of the four patients with IP injection. CONCLUSIONS: Fluorescence-guided lymphatic mapping of Wilms tumor drainage is feasible by both IP injection and PH injection techniques. However, whether lymphatic mapping improves the precision of lymph node sampling is unknown and should be studied in prospective trials.


Asunto(s)
Recurrencia Local de Neoplasia , Tumor de Wilms , Humanos , Preescolar , Estudios Prospectivos , Recurrencia Local de Neoplasia/patología , Verde de Indocianina , Ganglios Linfáticos/patología , Nefrectomía , Tumor de Wilms/cirugía , Tumor de Wilms/patología , Biopsia del Ganglio Linfático Centinela/métodos , Colorantes
15.
Ecancermedicalscience ; 16: 1356, 2022.
Artículo en Inglés | MEDLINE | ID: mdl-35510137

RESUMEN

Most children with tumors will require one or more surgical interventions as part of the care and treatment, including making a diagnosis, obtaining adequate venous access, performing a surgical resection for solid tumors (with staging and reconstruction), performing procedures for cancer prevention and its late effects, and managing complications of treatment; all with the goal of improving survival and quality of life. It is important for surgeons to adhere to sound pediatric surgical oncology principles, as they are closely associated with improved local control and survival. Unfortunately, there is a significant disparity in survival rates in low and middle income countries, when compared to those from high income countries. The International Society of Paediatric Surgical Oncology (IPSO) is the leading organization that deals with pediatric surgical oncology worldwide. This organization allows experts in the field from around the globe to gather and address the surgical needs of children with cancer. IPSO has been invited to contribute surgical guidance as part of the World Health Organization Initiative for Childhood Cancer. One of our goals is to provide surgical guidance for different scenarios, including those experienced in High- (HICs) and Low- and Middle-Income Countries (LMICs). With this in mind, the following guidelines have been developed by authors from both HICs and LMICs. These have been further validated by experts with the aim of providing evidence-based information for surgeons who care for children with cancer. We hope that this initiative will benefit children worldwide in the best way possible. Simone Abib, IPSO President Justin T Gerstle, IPSO Education Committee Chair Chan Hon Chui, IPSO Secretary.

16.
Children (Basel) ; 9(1)2022 Jan 01.
Artículo en Inglés | MEDLINE | ID: mdl-35053663

RESUMEN

Malignant peripheral nerve sheath tumors (MPNSTs) are aggressive soft tissue sarcomas (STS) with nerve sheath differentiation and a tendency to metastasize. Although occurring at an incidence of 0.001% in the general population, they are relatively common in individuals with neurofibromatosis type 1 (NF1), for whom the lifetime risk approaches 10%. The staging of MPNSTs is complicated and requires close multi-disciplinary collaboration. Their primary management is most often surgical in nature, with non-surgical modalities playing a supportive, necessary role, particularly in metastatic, invasive, or widespread disease. We, therefore, sought to provide a comprehensive review of the relevant literature describing the characteristics of these tumors, their pathophysiology and risk factors, their diagnosis, and their multi-disciplinary treatment. A close partnership between surgical and medical oncologists is therefore necessary. Advances in the molecular characterization of these tumors have also begun to allow the integration of targeted RAS/RAF/MEK/ERK pathway inhibitors into MPNST management.

17.
J Pediatr Surg ; 57(9): 229-233, 2022 Sep.
Artículo en Inglés | MEDLINE | ID: mdl-34456040

RESUMEN

PURPOSE: We sought to identify clinical features associated with difficult subcutaneous port removals in children. METHODS: Ports placed between April 2014 and September 2017 at our institution were prospectively tracked for difficult removals. A case-control analysis was performed. Patients with ports that were difficult to remove (stuck; cases) were compared to biological sex and age-matched controls in a ratio of 1:3. Logistic regression determined the association between case/control status and clinical features adjusting for biological sex and age as covariates. A multivariable analysis was performed to identify independent associations. RESULTS: 57 stuck ports (28 extreme [10 endovascular intervention] and 29 moderate) and 171 controls were analyzed. Stuck ports were associated with a diagnosis of acute lymphoblastic leukemia (86% cases versus 22.2% controls; p < 0.001) and a longer placement duration (median 2.6 years [interquartile range (IQR) 2.5-2.6] versus 0.8 years [IQR 0.5-1.4]; p < 0.001). On univariate analysis, procedural and device features associated with stuck ports included subclavian access (71.9% cases versus 48.5% controls; p = 0.0126), a polyurethane versus silicone catheter (96.5% cases versus 79.9% controls; p = 0.001), and a rough catheter appearance at removal (92.6% cases versus 9.4% controls; p < 0.0001). A diagnosis of ALL and duration of line placement were associated with having a stuck port on multivariate analysis. CONCLUSION: Polyurethane central venous catheters placed for the two-year treatment of acute lymphoblastic leukemia may become difficult to remove. This constellation of factors warrants more extensive preoperative discussion of risk, endovascular backup availability, and scheduling for longer operating room time.


Asunto(s)
Cateterismo Venoso Central , Catéteres Venosos Centrales , Leucemia-Linfoma Linfoblástico de Células Precursoras , Estudios de Casos y Controles , Catéteres de Permanencia , Niño , Humanos , Poliuretanos , Estudios Retrospectivos
18.
J Pediatr Surg ; 57(9): 174-178, 2022 Sep.
Artículo en Inglés | MEDLINE | ID: mdl-34518021

RESUMEN

BACKGROUND: Indocyanine green (ICG), a water-soluble tricarbocyanine fluorophore, is being increasingly used for tumor localization based on its passive intra-tumoral accumulation due to enhanced permeability and retention in tumor tissue. Therefore, we hypothesized that ICG can provide contrast to facilitate accurate, real-time recognition of renal tumors at the time of nephron-sparing surgery in children. METHODS: This retrospective study examined the feasibility of ICG in guiding nephron-sparing surgery for pediatric renal tumors. RESULTS: We reviewed the medical records of 8 pediatric patients with renal tumors in 12 kidneys. Intraoperative localization of tumor with near infrared guidance was successful in all 12 kidneys. However, we consistently found an inverse pattern of near infrared signal in which the normal kidney demonstrated increased fluorescent signal relative to the kidney tumor. CONCLUSIONS: Fluorescence-guided renal tumor delineation is unique because it has an inverse pattern of near infrared signal in which the normal kidney demonstrates increased signal relative to the adjacent tumor. Nevertheless fluorescence-guided distinguishing of renal tumor from surrounding normal kidney is feasible.


Asunto(s)
Verde de Indocianina , Neoplasias Renales , Niño , Humanos , Neoplasias Renales/diagnóstico por imagen , Neoplasias Renales/patología , Neoplasias Renales/cirugía , Nefrectomía , Nefronas/cirugía , Estudios Retrospectivos
19.
Ecancermedicalscience ; 15: 1187, 2021.
Artículo en Inglés | MEDLINE | ID: mdl-33777180

RESUMEN

The COVID-19 pandemic poses an unprecedented health crisis in all socio-economic regions across the globe. While the pandemic has had a profound impact on access to and delivery of health care by all services, it has been particularly disruptive for the care of patients with life-threatening noncommunicable diseases (NCDs) such as the treatment of children and young people with cancer. The reduction in child mortality from preventable causes over the last 50 years has seen childhood cancer emerge as a major unmet health care need. Whilst survival rates of 85% have been achieved in high income countries, this has not yet been translated into similar outcomes for children with cancer in resource-limited settings where survival averages 30%. Launched in 2018, by the World Health Organization (WHO), the Global Initiative for Childhood Cancer (GICC) is a pivotal effort by the international community to achieve at least 60% survival for children with cancer by 2030. The WHO GICC is already making an impact in many countries but the disruption of cancer care during the COVID-19 pandemic threatens to set back this global effort to improve the outcome for children with cancer, wherever they may live. As representatives of the global community committed to fostering the goals of the GICC, we applaud the WHO response to the COVID-19 pandemic, in particular we support the WHO's call to ensure the needs of patients with life threatening NCDs including cancer are not compromised during the pandemic. Here, as collaborative partners in the GICC, we highlight specific areas of focus that need to be addressed to ensure the immediate care of children and adolescents with cancer is not disrupted during the pandemic; and measures to sustain the development of cancer care so the long-term goals of the GICC are not lost during this global health crisis.

20.
J Laparoendosc Adv Surg Tech A ; 31(5): 589-593, 2021 May.
Artículo en Inglés | MEDLINE | ID: mdl-33599543

RESUMEN

Background: Posterolateral thoracotomy provides limited access to the thoracic apex that can result in poor visualization of subclavian vessels, their branches, and the brachial plexus. A thoracoscopic approach may overcome these limitations. Purpose: We report a thoracoscopic approach and associated technical challenges in resecting apical thoracic neuroblastoma encasing the subclavian artery. Methods: A single-institution retrospective chart review was performed (2018-2020) for patients undergoing thoracoscopic resection of apical neuroblastoma encasing the subclavian artery. Patient demographics, imaging, and hospital course were reviewed. Operative video recordings were assessed for exposure quality, technical challenges, and percentage of tumor resection. Patients were placed laterally, with three 5-mm ports triangulated to the apex. Dissection started at the tumor edge and followed along the vessel and branches. Results: Four patients (median age 2.7 years) underwent thoracoscopic apical neuroblastoma resection. Median length of stay was 2.5 days. One low-risk patient underwent resection for tumor growth during observation. One intermediate and 2 high-risk patients received neoadjuvant chemotherapy. Two patients continued having persistent vascular encasement, whereas in 1 patient the mass decreased in size and only abutted the subclavian and vertebral arteries. In 1 patient, tumor involved the brachial plexus, which was freed and preserved thoracoscopically. All cases had substantial tumor-feeding vessels branching from the subclavian artery. There was one conversion to open thoracotomy due to dense tumor adherence to the subclavian artery and vein. More than 95% resection was achieved in all cases. All patients had baseline Horner syndrome. No complications were reported. Conclusion: The thoracoscopic approach for resecting apical neuroblastoma provides optimal exposure and safe access in selected patients.


Asunto(s)
Neuroblastoma/cirugía , Arteria Subclavia/cirugía , Neoplasias Torácicas/cirugía , Toracoscopía/métodos , Preescolar , Femenino , Humanos , Lactante , Masculino , Invasividad Neoplásica , Neuroblastoma/diagnóstico por imagen , Neuroblastoma/patología , Estudios Retrospectivos , Arteria Subclavia/diagnóstico por imagen , Neoplasias Torácicas/diagnóstico por imagen , Neoplasias Torácicas/patología , Tomografía Computarizada por Rayos X , Resultado del Tratamiento
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