Abstract:
BACKGROUND:Rhabdoid tumors (RTs) of the liver are rare, aggressive and nonsecreting malignancies occurring mainly during the first year of life. Definition of RT relies on characteristic morphology and on the inactivation of the SMARCB1 tumor suppressor gene. The aim of this study was to analyze clinical data, treatments and outcomes in our patients. PATIENTS AND METHODS:6 cases of patients treated in our institution for RT of the liver between January 2007 and January 2015 are reported. Variables examined included age at diagnosis, tumor stage, treatment and long-term survival. RESULTS:Median age at diagnosis was 5months (range: 4-23). Normal for age serum AFP levels was observed in all patients. No patient presented with metastasis at diagnosis. The diagnosis of RT based on the loss of SMARCB1 was made early in 4 patients. The 2 others were initially diagnosed as nonsecreting hepatoblastomas. Median follow-up was 6years (range: 2-9). All patients received chemotherapy, with variable regimens depending on initial diagnosis, followed by surgical resection. Three patients (50%) died of disease. Two of them were mistaken for nonsecreting hepatoblastomas at diagnosis and had recurrence shortly after completion of treatment. The third one presented a cardiac right atrium thrombus. Three patients (50%) are long-term survivors; they received multimodal therapy including chemotherapy according to protocol EpSSG NRSTS consisting of doxorubicin and surgical removal of the tumor performed within 3months after diagnosis. One patient had adjuvant radiotherapy. CONCLUSION:According to our results, search of SMARCB1 mutation or alternatively immunohistochemical assay for SMARCB1 in nonsecreting hepatoblastomas is mandatory to exclude RT. Chemotherapy according to EpSSG NRSTS protocol together with a surgical treatment seems justified to improve long-term survival. TYPE OF STUDY:Retrospective study. LEVEL OF EVIDENCE:Level IV.
journal_name
J Pediatr Surgjournal_title
Journal of pediatric surgeryauthors
Cornet M,De Lambert G,Pariente D,Planchon JM,Guettier C,Martelli H,Guérin F,Branchereau Sdoi
10.1016/j.jpedsurg.2017.09.005subject
Has Abstractpub_date
2018-03-01 00:00:00pages
567-571issue
3eissn
0022-3468issn
1531-5037pii
S0022-3468(17)30572-9journal_volume
53pub_type
杂志文章abstract::Histologic studies of the human chorionic gonadotropin (hCG) producing hepatoblastoma were made for seven cases encountered in Japan. It was found in all cases that multinucleated tumor giant cells in several figures were admixed in usual hepatoblastoma tissue and one of them was confirmed to be hCG positive immunohis...
journal_title:Journal of pediatric surgery
pub_type: 杂志文章
doi:10.1016/s0022-3468(87)80258-0
更新日期:1987-05-01 00:00:00
abstract:BACKGROUND:Adult trauma centers (TCs) in the United States may be verified with an on-call operating room team if the performance improvement program shows no adverse outcome. Using queuing and simulation methodology, this study attempts to add a volume guideline for injured children. METHODS:Data from 63 verified TCs...
journal_title:Journal of pediatric surgery
pub_type: 杂志文章
doi:10.1016/j.jpedsurg.2010.08.048
更新日期:2010-12-01 00:00:00
abstract:BACKGROUND:Pyriform sinus fistula (PSF) is a rare congenital anomaly, and the preferred definitive treatment is yet to be verified. In this study, we investigated the treatment outcomes of PSF specifically comparing endoscopic-assisted surgery and endoscopic radiofrequency ablation (RA). METHODS:The medical records of...
journal_title:Journal of pediatric surgery
pub_type: 杂志文章
doi:10.1016/j.jpedsurg.2020.06.039
更新日期:2020-07-01 00:00:00
abstract:BACKGROUND:Standardization of surgical instrument trays and doctor preference cards (DPC) are known to reduce the cost of adult surgical cases. The practice in pediatric surgery may be more complex owing to a wide range of patient age, leading to difficulty with practice implementation and loss of potential financial s...
journal_title:Journal of pediatric surgery
pub_type: 杂志文章,评审
doi:10.1016/j.jpedsurg.2020.05.010
更新日期:2020-11-01 00:00:00
abstract:PURPOSE:An association between chronic renal failure (CRF) and gastroesophageal reflux (GER) is well known. The aim of this study was to pharmacologically characterize and investigate the possible contribution of smooth muscle reactivity pathways involving GER on the CRF rat model. MATERIAL AND METHODS:Chronic renal f...
journal_title:Journal of pediatric surgery
pub_type: 杂志文章
doi:10.1016/j.jpedsurg.2006.12.019
更新日期:2007-04-01 00:00:00
abstract:BACKGROUND/PURPOSE:Ischemic reperfusion injury (IR/I) should be minimum for the success of small bowel transplantation (SBTx). This study investigated whether preoperative administration of neuropeptide bombesin (BBS) had a protective effect against IR/I and subsequent acute rejection. METHODS:Allogenic SBTx was perfo...
journal_title:Journal of pediatric surgery
pub_type: 杂志文章
doi:10.1016/j.jpedsurg.2005.08.035
更新日期:2005-12-01 00:00:00
abstract:PURPOSE:Eighteen cases of pyogenic liver abscess (PLA) admitted at JIPMER hospital, South India, over a 6-year period were analyzed to document the clinical profile and to evaluate the management of PLA among children. METHODS:Records of all these patients were reviewed for presenting signs and symptoms, any associate...
journal_title:Journal of pediatric surgery
pub_type: 杂志文章
doi:10.1016/s0022-3468(98)90081-1
更新日期:1998-03-01 00:00:00
abstract::A newborn with right diaphragmatic hernia suffered myocardial stunning and suprasystemic pulmonary hypertension secondary to postpartal asphyxia. In addition to conventional therapy, norepinephrine, enoximone, and inhalational nitric oxide were successfully used. Norepinephrine improved myocardial perfusion pressure; ...
journal_title:Journal of pediatric surgery
pub_type: 杂志文章
doi:10.1016/0022-3468(95)90751-3
更新日期:1995-06-01 00:00:00
abstract:OBJECTIVE:To review and compare robotic ipsilateral uretero-ureterostomy (RIUU) and laparoscopic ipsilateral uretero-ureterostomy (LIUU) in terms of safety, efficacy, and outcomes. MATERIALS AND METHODS:A retrospective chart review, including all pediatric patients who underwent RIUU and LIUU at 5 different medical ce...
journal_title:Journal of pediatric surgery
pub_type: 杂志文章
doi:10.1016/j.jpedsurg.2020.12.022
更新日期:2021-01-07 00:00:00
abstract:BACKGROUND:The purpose of this study was to review surgical outcomes after elective placement of peritoneal dialysis (PD) catheters in children with end-stage renal disease. METHODS:Children with PD catheters placed between February 2002 and July 2014 were retrospectively reviewed. Outcomes were catheter life, late (>...
journal_title:Journal of pediatric surgery
pub_type: 杂志文章
doi:10.1016/j.jpedsurg.2016.02.011
更新日期:2016-05-01 00:00:00
abstract:PURPOSE:Total thyroidectomy (TT) is a safe and efficacious treatment of malignant thyroid disease in children. The role of TT in benign thyroid disease is less well-defined. The goal of this study was to compare the safety of TT performed for benign and malignant disease. METHODS:The medical records of 31 patients und...
journal_title:Journal of pediatric surgery
pub_type: 杂志文章
doi:10.1016/j.jpedsurg.2008.11.032
更新日期:2009-08-01 00:00:00
abstract:PURPOSE:Few studies have focused on the behavior of rhabdoid tumor (RT) in the fetus and neonate. The purpose of this review is to show that perinatal RTs are associated with unusual findings and a poor prognosis. METHODS:The author conducted a 40-year systematic review of the literature. Clinical presentation, pathol...
journal_title:Journal of pediatric surgery
pub_type: 杂志文章,评审
doi:10.1016/j.jpedsurg.2009.12.011
更新日期:2010-03-01 00:00:00
abstract::This report describes a case of thoracoscopic repair of esophageal atresia with a rare type D tracheoesophageal fistula in a child with complex congenital heart disease. We demonstrate the feasibility of thoracoscopic repair and anesthetic management in a child with complex congenital heart disease. ...
journal_title:Journal of pediatric surgery
pub_type: 杂志文章
doi:10.1016/j.jpedsurg.2007.05.013
更新日期:2007-09-01 00:00:00
abstract::Presently, descriptions of rural trauma are complications of national sample statistics and local data from states projected to rural areas. This study reviews all hospital discharges (36,866) for children (aged 0 to 18 years) from January 1985 through December 1990 in an entirely rural state. Fourteen percent of admi...
journal_title:Journal of pediatric surgery
pub_type: 杂志文章
doi:10.1016/s0022-3468(05)80316-1
更新日期:1993-10-01 00:00:00
abstract:AIM:An enormous amount of information about pediatric surgical conditions is available on the World Wide Web (www). Our aim was to ascertain how many parents accessed the www and how useful they found the exercise. METHOD:Over a 2-month period, all parents attending the surgical outpatient clinics were asked to comple...
journal_title:Journal of pediatric surgery
pub_type: 杂志文章
doi:10.1016/j.jpedsurg.2006.10.003
更新日期:2007-02-01 00:00:00
abstract:PURPOSE:The purpose of this study was 2-fold: to study the complications of neuroblastoma treatment and to establish surgical guidelines. MATERIALS AND METHODS:The medical records of 92 patients with neuroblastoma (stage 1 or 2, n = 33; stage 3, n = 21; stage 4, n = 31; stage 4S, n = 7) who had undergone surgery in ou...
journal_title:Journal of pediatric surgery
pub_type: 杂志文章
doi:10.1016/j.jpedsurg.2006.08.003
更新日期:2006-12-01 00:00:00
abstract:BACKGROUND/PURPOSE:American pediatric surgical education has more than a 65-year history of formalizing the organization and the curriculum of the training process. However, never before have so many simultaneous internal and external forces appeared on the horizon that have the collective potential of influencing the ...
journal_title:Journal of pediatric surgery
pub_type: 历史文章,杂志文章
doi:10.1016/j.jpedsurg.2004.04.002
更新日期:2004-08-01 00:00:00
abstract::A newborn boy was brought to us, 2 hours after birth, with a mucosal-lined left hemiperineal lesion associated with classical bladder exstrophy and an anterolaterally displaced anus. Perineal anatomy was restored by excising the mucosa lined lesion. The bladder closure for classical bladder exstrophy was done at the s...
journal_title:Journal of pediatric surgery
pub_type: 杂志文章
doi:10.1016/j.jpedsurg.2010.03.012
更新日期:2010-07-01 00:00:00
abstract:BACKGROUND:Many children evaluated for child abuse have non-inflicted injuries due to supervisory neglect. Despite its prevalence, supervisory neglect has received minimal attention. METHODS:We performed a retrospective review of patients maintained in a hospital forensic registry. Text analytics software was used to ...
journal_title:Journal of pediatric surgery
pub_type: 杂志文章
doi:10.1016/j.jpedsurg.2019.10.010
更新日期:2020-02-01 00:00:00
abstract::The etiology of aganglionosis of the bowel remains controversial. Initial embryological studies in chicks, mice, and humans suggested the defect was in the migratory capacity of the vagal neural crest cells. This traditional theory has recently been challenged by the demonstration of a defect in the local microenviron...
journal_title:Journal of pediatric surgery
pub_type: 杂志文章
doi:10.1016/0022-3468(92)90860-a
更新日期:1992-03-01 00:00:00
abstract::Small intestinal myoelectric activity has been studied extensively in adult humans and in many animal models. However, little is known about gut myoelectrical activity in newborns, a population susceptible to primary and secondary motility disorders. We report the development of a chronic neonatal piglet model for ass...
journal_title:Journal of pediatric surgery
pub_type: 杂志文章
doi:10.1016/0022-3468(90)90075-k
更新日期:1990-03-01 00:00:00
abstract:BACKGROUND/PURPOSE:Our approach to full-thickness anorectal prolapse has transitioned to laparoscopic suture rectopexy (LSRP). The purpose of this study was to describe the indications, technique, and postoperative outcomes for LSRP. METHODS:Rectopexy was performed using 3 or 4 laparoscopic ports. Redundant rectum was...
journal_title:Journal of pediatric surgery
pub_type: 杂志文章
doi:10.1016/j.jpedsurg.2010.06.006
更新日期:2010-10-01 00:00:00
abstract:BACKGROUND/PURPOSE:Trauma centers (TC) are certified based on widely accepted criteria. These specific criteria rarely are scrutinized individually. The purpose of this study was to analyze the individual components of a pediatric trauma center for their effect on outcome. METHODS:Members of the National Pediatric Tra...
journal_title:Journal of pediatric surgery
pub_type: 杂志文章,多中心研究
doi:10.1016/s0022-3468(99)90392-5
更新日期:1999-05-01 00:00:00
abstract:BACKGROUND:A large cohort of Hodgkin lymphoma (HL) survivors exist. With patients transitioning from pediatric to adult care, practitioners should be aware of potential complications. The aim of this study was to describe the long-term complications of patients who had staging laparotomy for the treatment of HL. METHO...
journal_title:Journal of pediatric surgery
pub_type: 杂志文章
doi:10.1016/j.jpedsurg.2016.11.047
更新日期:2017-09-01 00:00:00
abstract::Defect of the intestinal musculature is a rare condition. It may cause intestinal perforation or obstruction. It manifests itself mainly in the neonatal period and usually affects preterm infants. We describe one such case, which was first diagnosed as a spontaneous isolated intestinal perforation. Emergency laparotom...
journal_title:Journal of pediatric surgery
pub_type: 杂志文章
doi:10.1016/j.jpedsurg.2010.05.029
更新日期:2010-08-01 00:00:00
abstract:BACKGROUND:Renal insufficiency is a significant complication of Wilms tumor treatment in the 5% with bilateral disease. Nephron-sparing surgery (NSS) is recommended after neoadjuvant chemotherapy initially. However, the role of NSS in recurrent disease is unknown. We reviewed our experience to assess the feasibility an...
journal_title:Journal of pediatric surgery
pub_type: 杂志文章
doi:10.1016/j.jpedsurg.2013.09.048
更新日期:2014-01-01 00:00:00
abstract::Splenectomy is a recognized factor predisposing to the late complication of serious sepsis. The meningococcus has been listed as an important organism in postsplenectomy infection. A survey of the literature, however, revealed a total of only 13 documented case reports over a 31-year period, including ten pediatric ca...
journal_title:Journal of pediatric surgery
pub_type: 杂志文章
doi:10.1016/s0022-3468(86)80195-6
更新日期:1986-04-01 00:00:00
abstract::"Conservative" management of splenic rupture in patients with mononucleosis has not been adequately evaluated because of its infrequent occurrence. Splenic rupture can be lethal in this setting and is the most common cause of death from mononucleosis. We report a case of spontaneous splenic rupture in a young girl wit...
journal_title:Journal of pediatric surgery
pub_type: 杂志文章
doi:10.1016/s0022-3468(87)80512-2
更新日期:1987-11-01 00:00:00
abstract::There are few reports regarding the presence of retained fixed fragments after removal of indwelling central venous catheters in children. We conducted a retrospective study of 355 patients who underwent removal of central venous catheter from 1996 to 2008. Six patients (1.6%) had a failed attempt of removal, resultin...
journal_title:Journal of pediatric surgery
pub_type: 杂志文章
doi:10.1016/j.jpedsurg.2010.02.001
更新日期:2010-07-01 00:00:00
abstract:PURPOSE:To present the clinical and histological features of prepubertal testicular tumors (PTTs), the long-term experience of a single institution was reviewed. MATERIALS AND METHODS:A total of 62 prepubertal children who were treated for testicular tumors at Kanagawa Children's Medical Center from 1971 to 2014 were ...
journal_title:Journal of pediatric surgery
pub_type: 杂志文章
doi:10.1016/j.jpedsurg.2016.02.042
更新日期:2016-08-01 00:00:00