Abstract:
:Desmoplastic small round cell tumour (DSRCT) is an extremely rare neoplasm. Adolescent males and young adults are most frequently affected. It is highly malignant, with only 29% of patients surviving up to 3 years. This paper documents two cases, one of which, at 4 years old, is the second youngest case documented. Case 1, a 10-year old boy, presented with a 20-day history of choluria, acholia, asthenia, anorexia, and right abdominal pain. Laboratory values were altered, and imaging showed multiples masses in the liver and retroperitoneum. A minilaparotomy was carried out, and a biopsy showed a stage III DSRCT. He was treated with chemotherapy but died of hepatic failure. Case 2, a 4-year-old boy, presented with a 2-month history of abdominal distension. Several hard masses were palpated in the abdomen, and a right inguinal mass that compressed the right testis was observed. Biopsy of the inguinal tumour showed a DSRCT. After treatment with chemotherapy, two operations were carried out to resect different intraabdominal masses. The patient died with peritoneal carcinomatosis 2 months after the last operation. The first patient died due to the advanced stage of the disease, and the second died after chemotherapy, peripheral blood stem transplantation, and multiple operations. The occurrence of this type of tumour in the paediatric age group as well as its high malignancy is noteworthy. Until more effective forms of treatment are found, we recommend treatment with chemotherapy, surgery, and radiotherapy, with close monitoring of the patient.
journal_name
Pediatr Surg Intjournal_title
Pediatric surgery internationalauthors
Mingo L,Seguel F,Rollán Vdoi
10.1007/s00383-005-1401-1subject
Has Abstractpub_date
2005-04-01 00:00:00pages
279-81issue
4eissn
0179-0358issn
1437-9813journal_volume
21pub_type
杂志文章abstract::A 6-year-old girl presented with intractable constipation. On investigation, she was found to have an association of anorectal stenosis, anterior sacral defect, and anterior meningocoele (Currarino's triad). The meningocoele was excised and a Duhamel pull-through procedure performed after resection of a massively dist...
journal_title:Pediatric surgery international
pub_type: 杂志文章,评审
doi:10.1007/s003830050362
更新日期:1998-07-01 00:00:00
abstract::The aspiration of a bronchial foreign body (FB) remains a common pediatric problem with serious and sometimes fatal sequelae. The diagnosis is often delayed or overlooked. With the aim of determining a reliable clinical and/or radiologic finding to indicate the requirement for bronchoscopy, 100 patients admitted to ou...
journal_title:Pediatric surgery international
pub_type: 杂志文章
doi:10.1007/s003830050080
更新日期:1997-03-21 00:00:00
abstract:PURPOSE:The aim of the German Network for Congenital Uro-REctal malformations (CURE-Net) is to collect data of affected patients with anorectal malformation (ARM) to investigate molecular causes, clinical implications and psychosocial outcome. The current issue was to examine the transition to adulthood in adults with ...
journal_title:Pediatric surgery international
pub_type: 杂志文章
doi:10.1007/s00383-012-3120-8
更新日期:2012-08-01 00:00:00
abstract:AIM OF THE STUDY:Liver retraction during laparoscopic fundoplication is important for obtaining an optimal space. Several methods have been developed, but the risks and benefits are unclear. We compared three different approaches and evaluated their safety and utility. METHODS:Forty-three neurologically impaired patie...
journal_title:Pediatric surgery international
pub_type: 杂志文章
doi:10.1007/s00383-020-04646-8
更新日期:2020-05-01 00:00:00
abstract:PURPOSE:Haemolytic uraemic syndrome (HUS) is the commonest childhood cause of acute renal failure. Peritoneal dialysis peritonitis (PDP) is a well-recognised complication, with some children requiring surgical intervention (SI). The aim of this study is to determine whether the presence of enteric organisms in cases of...
journal_title:Pediatric surgery international
pub_type: 杂志文章
doi:10.1007/s00383-015-3833-6
更新日期:2016-03-01 00:00:00
abstract::An infant born in the 34th week of gestation weighing 5,355 g with a massive sacrococcygeal (SC) tumor was delivered by elective cesarean section. An ultrasonographic examination showed solid and cystic components in the tumor. Resection was successfully undertaken with insertion of a Nélaton catheter into the rectum ...
journal_title:Pediatric surgery international
pub_type: 杂志文章
doi:10.1007/s003830000451
更新日期:2001-03-01 00:00:00
abstract:PURPOSE:Surgery of the inguinal canal is very common in infancy. Occasionally, injury of the bladder may occur with severe consequences. The aim of this paper was to warn young surgeons of this serious complication. METHODS:During the last 2 years, we observed four cases of bladder injury following surgery on inguinal...
journal_title:Pediatric surgery international
pub_type: 杂志文章
doi:10.1007/s00383-010-2707-1
更新日期:2010-12-01 00:00:00
abstract::Pyloric atresia (PA) is an uncommon anomaly that may be associated with many other congenital anomalies, the commonest of which is junctional epidermolysis bullosa (JEB). Most of the cases of PA associated with JEB (Herlitz syndrome) reported have been fatal. A case of PA associated with JEB, malrotation, and a high a...
journal_title:Pediatric surgery international
pub_type: 杂志文章
doi:10.1007/s003830050572
更新日期:1999-01-01 00:00:00
abstract:PURPOSE:The goal of this study was to determine the degree of consensus in the management of spinal cord tethering (TC) in patients with anorectal malformation (ARM) in a large cohort of European pediatric centers. METHODS:A survey was sent to pediatric surgeons (one per center) members of the ARM-Net Consortium. RES...
journal_title:Pediatric surgery international
pub_type: 杂志文章,多中心研究
doi:10.1007/s00383-017-4105-4
更新日期:2017-08-01 00:00:00
abstract::Minimally invasive surgery (MIS) for cancer patients has become widely accepted in general surgery, however, it has not completely replaced the standard open operative procedures in pediatric oncology. The aim of this study was to evaluate the host relationship following MIS in a murine model of retroperitoneal neurob...
journal_title:Pediatric surgery international
pub_type: 杂志文章
doi:10.1007/s003830050271
更新日期:1998-03-01 00:00:00
abstract:PURPOSE:Pediatric patients with intestinal failure need long-term parenteral nutrition (PN), but this nutritional support causes liver dysfunction, such as intestinal failure-associated liver disease (IFALD). Several studies have shown that the lipid emulsion produced by soybean oil (SO) is associated with the occurren...
journal_title:Pediatric surgery international
pub_type: 杂志文章
doi:10.1007/s00383-017-4190-4
更新日期:2018-02-01 00:00:00
abstract:PURPOSE:Determining the integrity of the pancreatic duct is important in high-grade pancreatic trauma to guide decision making for operative vs non-operative management. Computed tomography (CT) is generally an inadequate study for this purpose, and magnetic resonance cholangiopancreatography (MRCP) is sometimes obtain...
journal_title:Pediatric surgery international
pub_type: 杂志文章,多中心研究
doi:10.1007/s00383-018-4309-2
更新日期:2018-09-01 00:00:00
abstract::Hypospadias surgery is one of the most difficult areas in pediatric urology and has been characterized by constant evolution. Some of the surgical techniques proposed in the past are now considered inadequate because of an unacceptable complication rate or poor functional and aesthetic results. The key for assessing a...
journal_title:Pediatric surgery international
pub_type: 杂志文章
doi:10.1007/s00383-004-1147-1
更新日期:2004-03-01 00:00:00
abstract::Incarcerated inguinal hernias in infants are commonly encountered. Reduction with intravenous sedation using morphine and related drugs carries risks of apnoea and/or respiratory arrest, especially in small, ex-premature babies who have an increased incidence of incarceration and thus opiate use is best avoided. Cauda...
journal_title:Pediatric surgery international
pub_type: 杂志文章
doi:10.1007/s00383-005-1534-2
更新日期:2005-09-01 00:00:00
abstract::The Thomsen-Friedenreich cryptantigen (TCA) is located on the surface of all red cells, but is concealed by a layer of neuraminic acid. When bacteria that produce neuraminidase disrupt this coating, the TCA can be exposed and activated. If blood products containing antibody to the TCA are subsequently administered, ha...
journal_title:Pediatric surgery international
pub_type: 杂志文章
doi:10.1007/s003830050488
更新日期:1998-12-01 00:00:00
abstract:PURPOSE:To describe the association of anorectal malformation (ARM) and spinal dysraphism (SD) in terms of impact on the management of SD and embryogenetic implications. METHODS:Patients with SD associated with (A) or without (B) ARM were included. The two groups were further divided into operated on (A1/B1) or not (A...
journal_title:Pediatric surgery international
pub_type: 杂志文章
doi:10.1007/s00383-017-4104-5
更新日期:2017-08-01 00:00:00
abstract::Port-site recurrence (PSR) following laparoscopic procedures has been an unpredictable complication in adult cancer patients; however, no data exist about this phenomenon in the pediatric field. The aim of this study was to determine whether PSR, following endosurgical procedure for malignancies, is a typical complica...
journal_title:Pediatric surgery international
pub_type: 杂志文章
doi:10.1007/s00383-002-0918-9
更新日期:2003-05-01 00:00:00
abstract::Two patients who presented with a history of right inguinal hernia and a third with an impalpable left testis were found to have transverse testicular ectopia. Both testes were fixed in their own scrotum, through a modified Ombredanne operation in two patients and a subdartos pouch in one. ...
journal_title:Pediatric surgery international
pub_type: 杂志文章
doi:
更新日期:1997-02-01 00:00:00
abstract::Migration of particulate matter from plastic tubing and solid plastic implants has been documented in a number of studies, including some with the use of cardiac bypass, haemodialysis, and pump-assisted intravenous infusions. In order to ascertain whether silicone embolisation occurs when children have an Ivac 560 pum...
journal_title:Pediatric surgery international
pub_type: 杂志文章
doi:10.1007/BF01194802
更新日期:1997-01-01 00:00:00
abstract:PURPOSE:To report the authors' experience in the management of perforated choledochal cyst with bile peritonitis (PCC) in children. METHODS:Medical records of all children undergoing surgery for PCC at our hospital from May, 2005 to January, 2013 were reviewed. RESULTS:Twenty seven patients were identified, with a me...
journal_title:Pediatric surgery international
pub_type: 杂志文章
doi:10.1007/s00383-014-3461-6
更新日期:2014-03-01 00:00:00
abstract::To categorize variant persistent müllerian-duct derivatives (MDD) in males and determine their related clinical presentations for further management, five male patients with retained MDDs from abnormal müllerian remnants were studied. They comprised one patient with persistent müllerian-duct syndrome, one with transve...
journal_title:Pediatric surgery international
pub_type: 杂志文章
doi:10.1007/s00383-002-0718-2
更新日期:2002-09-01 00:00:00
abstract::The embryogenesis of a previously unreported mesothelial pouch containing a Meckel's diverticulum is presented. ...
journal_title:Pediatric surgery international
pub_type: 杂志文章
doi:10.1007/BF01076960
更新日期:1997-07-01 00:00:00
abstract::The introduction of posterior sagittal anorectoplasty (PSARP) by deVries and Peña in the early 80s has impacted to the treatment of patients with anorectal malformations (ARM). It gained great recognition worldwide in a very short time, and since then, surgeons dealing with the treatment of this complex malformation c...
journal_title:Pediatric surgery international
pub_type: 杂志文章,评审
doi:10.1007/s00383-020-04629-9
更新日期:2020-04-01 00:00:00
abstract::Between 1993-2002 we used both diagnostic and operative laparoscopy in the management of five cases of persistent müllerian duct syndrome (BMDS). Two siblings from two different families accounted for four of the cases. They presented with cryptorchidism and inguinal hernias. The diagnosis was established during diagn...
journal_title:Pediatric surgery international
pub_type: 杂志文章
doi:10.1007/s00383-003-0984-7
更新日期:2003-09-01 00:00:00
abstract::Gastroschisis closure is performed either primarily or after staged reduction of the prolapsed bowel. A technique for surgical closure of the abdominal wall defect is described which allows for an almost scarless appearance of the abdominal surface with preservation of a midline umbilicus. ...
journal_title:Pediatric surgery international
pub_type: 杂志文章
doi:10.1007/s00383-008-2215-8
更新日期:2008-09-01 00:00:00
abstract::Congenital diaphragmatic hernia (CDH) is accompanied by pulmonary hypoplasia and pulmonary hypertension. Fetal lung growth is dependent on the secretion of lung liquid, which normally is absorbed at partus. The ion channel NKCC-1 is involved in this secretory process, but has recently also been reported to be implicat...
journal_title:Pediatric surgery international
pub_type: 杂志文章
doi:10.1007/s00383-008-2209-6
更新日期:2008-09-01 00:00:00
abstract:PURPOSE:Cervicothoracic and apical thoracic neuroblastoma pose unique surgical challenges. We report our experience with the trapdoor anterior thoracotomy (TAT) approach to overcome these difficulties. METHODS:Retrospective review of our centre's neuroblastoma database was conducted. Patients who underwent TAT at our ...
journal_title:Pediatric surgery international
pub_type: 杂志文章
doi:10.1007/s00383-020-04692-2
更新日期:2020-08-01 00:00:00
abstract::Minor degrees of exomphalos have been known to be associated with vitellointestinal duct anomalies. The most frequent association is Meckel's diverticulum. It is unusual for a fistula to be communicating directly with the exomphalos sac. We report five neonates that presented with a fistula on the exomphalos sac over ...
journal_title:Pediatric surgery international
pub_type: 杂志文章,评审
doi:10.1007/s00383-006-1699-3
更新日期:2006-07-01 00:00:00
abstract::We present the case of a 10-year-old girl with biliary atresia in whom focal nodular hyperplasia (FNH) of the liver occurred following successful "Kasai" hepatic portoenterostomy at the age of 2 months. Her jaundice completely disappeared a few weeks after the operation. A 4-cm diameter liver tumor in segment IV was f...
journal_title:Pediatric surgery international
pub_type: 杂志文章
doi:10.1007/s00383-007-2090-8
更新日期:2008-05-01 00:00:00
abstract::A 7-year-old female with a type I choledochal cyst underwent an exploratory laparotomy. A Roux-en-Y choledochojejunostomy was done following cyst excision. The patient was re-explored 8 days later for an anastomotic bile leak and high fever. A round-worm was found obstructing the jejunal lumen at the porta hepatis, re...
journal_title:Pediatric surgery international
pub_type: 杂志文章
doi:10.1007/s003830050394
更新日期:1998-09-01 00:00:00