G. Suren Arul

Revised Risk Classification for Pediatric Extracranial Germ Cell Tumors Based on 25 Years of Clinical Trial Data From the United Kingdom and United States

PURPOSE To risk stratify malignant extracranial pediatric germ cell tumors (GCTs). PATIENTS AND METHODS Data from seven GCT trials conducted by the Childrens Oncology Group (United States) or the Childrens Cancer and Leukemia Group (United Kingdom) between 1985 and 2009 were merged to create a data set of patients with stage II to IV disease treated with platinum-based therapy. A parametric cure model was used to evaluate the prognostic importance of age, tumor site, stage, histology, tumor markers, and treatment regimen and estimate the percentage of patients who achieved long-term disease-free (LTDF) survival in each subgroup of the final model. Validation of the model was conducted using the bootstrap method. RESULTS In multivariable analysis of 519 patients with GCTs, stage IV disease (P = .001), age ≥ 11 years (P < .001), and tumor site (P < .001) were significant predictors of worse LTDF survival. Elevated alpha-fetoprotein (AFP) ≥ 10,000 ng/mL was associated with worse outcome, whereas pure yolk sac tumor (YST) was associated with better outcome, although neither met criteria for statistical significance. The analysis identified a group of patients age > 11 years with either stage III to IV extragonadal tumors or stage IV ovarian tumors with predicted LTDF survival < 70%. A bootstrap procedure showed retention of age, tumor site, and stage in > 94%, AFP in 12%, and YST in 27% of the replications. CONCLUSION Clinical trial data from two large national pediatric clinical trial organizations have produced a new evidence-based risk stratification of malignant pediatric GCTs that identifies a poor-risk group warranting intensified therapy.

Pediatric and Adolescent Extracranial Germ Cell Tumors: The Road to Collaboration

During the past 35 years, survival rates for children with extracranial malignant germ cell tumors (GCTs) have increased significantly. Success has been achieved primarily through the application of platinum-based chemotherapy regimens; however, clinical challenges in GCTs remain. Excellent outcomes are not distributed uniformly across the heterogeneous distribution of age, histologic features, and primary tumor site. Despite good outcomes overall, the likelihood of a cure for certain sites and histologic conditions is less than 50%. In addition, there are considerable long-term treatment-related effects for survivors. Even modest cisplatin dosing can cause significant long-term morbidities. A particular challenge in designing new therapies for GCT is that a variety of specialists use different risk stratifications, staging systems, and treatment approaches for three distinct age groups (childhood, adolescence, and young adulthood). Traditionally, pediatric cancer patients younger than 15 years have been treated by pediatric oncologists in collaboration with their surgical specialty colleagues. Adolescents and young adults with GCTs often are treated by medical oncologists, urologists, or gynecologic oncologists. The therapeutic dilemma for all is how to best define disease risk so that therapy and toxicity can be appropriately reduced for some patients and intensified for others. Further clinical and biologic insights can only be achieved through collaborations that do not set limitations by age, sex, and primary tumor site. Therefore, international collaborations, spanning different cooperative groups and disciplines, have been developed to address these challenges.

Is adjuvant chemotherapy indicated in ovarian immature teratomas? A combined data analysis from the Malignant Germ Cell Tumor International Collaborative

There is a debate regarding the management of ovarian immature teratomas (ITs). In adult women, postoperative chemotherapy is standard except for stage I, grade 1 disease, whereas surgery alone is standard in pediatric patients. To determine the role of chemotherapy, a pooled analysis of pediatric and adult clinical trials was conducted.

The transversus abdominis plane (TAP) block in neonates and infants - results of an audit.

Neither author has any involvement, financial or otherwise, with the manufacturers of sugammadex. No funding or sponsorship was sought or provided from any source. Written parental consent was obtained having shown the parents the full text of the report. Cameron C.R. Buchanan Aidan M. O’Donnell Department of Anaesthetics, Waikato Hospital, Hamilton, New Zealand Email: cam.buchanan@waikatodhb.health.nz

Fifteen-minute consultation: The infant with a tongue tie

Tongue tie is an increasingly common cause for referral of infants to our general paediatric surgery service. In this article, we will explore the indications for tongue tie division in the newborn child, the practicalities of the procedure and the supporting evidence.

Management of gastroesfophageal reflux associated with malrotation in children

AIM Children being investigated for gastroesophageal reflux (GOR) have a high incidence of malrotation. Current literature suggests these patients should be managed with a combined antireflux and Ladds procedure. We review our experience, the largest series to date, of performing an elective Ladds procedure as the first-line intervention. METHOD Retrospective case note review of 20 children with significant symptoms of GOR and an incidental finding of malrotation. Children presenting immediately with bilious vomiting were excluded. All patients underwent a Ladds procedure as their sole primary operative intervention. RESULTS Median age at operation was 7 months (21 days-12 years). Fifteen patients (75%) had evidence of reflux on barium contrast study. All children were followed up for at least 6 months. Eighteen (90%) had resolution or significant improvement of their symptoms postsurgery. Only 3 have not managed to tolerate a full oral diet, all unrelated to GOR. None of our series required an antireflux procedure. CONCLUSION In children with debilitating vomiting necessitating surgical management, a contrast study is imperative in the work up. The high incidence of GOR and the significant improvement after correction of malrotation show the relationship between delayed gastric emptying and GOR. We suggest that when an abnormally placed duodenojejunal flexure is found, a Ladds procedure alone is sufficient and may obviate the need for a more invasive antireflux procedure.

Intra-abdominal Kaposiform hemangioendothelioma and the benefits of laparoscopic surveillance.

We report a case of a neonate presenting with the rare vascular tumor, Kaposiform hemangioendothelioma. She had a lesion arising from the left ovary with multiple intraperitoneal metastases causing small bowel obstruction. We managed this case with primary surgical resection followed by laparoscopic surveillance of the peritoneal cavity and metastectomy. The child is fit and well and free of tumor after 32 months. Neither this presentation of this tumor nor this management approach has been previously described in the literature. Pediatr Blood Cancer 2012; 58: 992–993.

Characterization of the upper pouch tracheo–oesophageal fistula in oesophageal atresia

AIM A small proportion of infants with oesophageal atresia (OA) are thought to have a proximal tracheoesophageal fistula (TOF). Failure to recognize these can hamper mobilization of the upper pouch and lead to life-threatening episodes of aspiration once oral feeding starts. We reviewed our experience of upper pouch fistulae to identify characteristic features of proximal TOF. METHODS A retrospective review of TOF/OA patient notes and bronchoscopy photographs and videos, identified from our database from 01/01/2006 to 12/31/2015, was performed. RESULTS Eight (6.1%) infants were identified (M:F 5:3) from a total population of 131 newly diagnosed TOF/OA infants during the period. Their median gestational age was 33 (range 28-39) weeks, and median birth weight was 1647g (range 1100-3400g). Five were initially diagnosed with pure OA and 3 with a distal TOF. All patients underwent rigid bronchoscopy at the initial surgery but only one proximal fistula was identified. The 7 missed proximal fistulae were subsequently found either during on-table oesophagograms for gap assessment (n=2), at the time of thoracotomy when mobilizing the upper pouch (n=3), or during subsequent bronchoscopy for symptoms post OA repair (n=2). Two patients needed a further operation to divide the fistula. Review of the bronchoscopy videos identified four characteristic differences between upper and lower pouch fistulae. Proximal fistulae are found just distal to the vocal cords, are very small, often no more than a pit, do not open and close with ventilation, and are best identified by insufflation of the esophagus. CONCLUSION Upper pouch fistulae are relatively easy to miss because of different characteristics compared with H-type or distal fistulae that have not previously been mentioned in the literature. LEVEL OF EVIDENCE level IV.

Surgical Complications of Childhood Tumors

Most childhood tumors will first present to a physician; some tumors will present in an atypical manner and may mimic a surgical condition. The diagnosis may be missed if the surgeon is not aware of the possibility of cancer. A very great number of rare presentations of childhood cancer have been described in the literature. It is important that the surgeon who is not experienced in the management of childhood cancer is aware that an apparently benign condition could be a manifestation of an underlying malignancy.

Is Adjuvant Chemotherapy necessary in Ovarian Immature Teratoma? A Combined Data Analysis from The Malignant Germ Cell Tumors International Collaborative (MaGIC)

This is the author accepted manuscript. The final version is available from Wiley at http://dx.doi.org/10.1002/cncr.29732.