Neonatal Sacrococcygeal Fetiform Teratoma Containing Bowel: A Case Report.

A fetiform sacrococcygeal teratoma (homunculus) is a highly differentiated subgroup of mature cystic teratoma that resembles a malformed fetus. These tumors originate at the base of the coccyx and may vary in their intrapelvic and extrapelvic extent and location. It is important to differentiate this anomaly from fetus-in-fetu which has a higher degree of structural organization. A 5-day-old neonate presented with a type II sacrococcygeal fetiform teratoma. The mass contained both cystic and solid components. Upon surgical excision and coccygectomy, fully formed bowel was found inside the mass, as well as bones and other well-defined structures. The tumor was confirmed to be fully excised and no malignant or immature features were found on histopathological examination. The patient was last seen growing well with an alpha-fetoprotein of 3.5 µg/L, 14 months after resection.

Development of a template for operative reporting of pediatric cancer surgery in limited-resource settings by using a modified Delphi method.

PURPOSE: A comprehensive operative report for cancer surgery is crucial for accurate disease staging, risk stratification, and therapy escalation/de-escalation, which affects the outcome. Narrative operative reports may fail to include some critical findings. Furthermore, standardized operative reports can form the basis of a local registry, which is often lacking in limited-resource settings (LRSs). In adult literature, synoptic operative reports (SOR) contain more key findings than narrative operative reports. In the LRSs, where the capacity of diagnostic pathology services is typically suboptimal, the value of a thorough operative report is even greater. The aim of this study was to develop a SOR template to help standardize childhood cancer surgery reporting in LRSs. METHODS: Twenty-three experts in pediatric cancer with extensive experience practicing in LRSs were invited to participate in a modified Delphi procedure. SOR domains for pediatric oncology surgery were drafted based on a literature search and then modified based on experts' opinions. The experts anonymously answered multiple rounds of online questionnaires until all domains and subdomains reached a consensus, which was predefined as 70% agreement. RESULTS: Sixteen experts participated in the study, and two rounds of the survey were completed. Twenty-one domains were considered relevant, including demographics, diagnosis, primary site, preoperative disease stage, previous tumor biopsy or surgery, preoperative tumor rupture, neoadjuvant therapy, surgical access, type of resection, completeness of resection, tumor margin assessment, locoregional tumor extension, organ resection, intraoperative tumor spillage, vascular involvement, lymph node sampling, estimated blood loss, intraoperative complications and interventions to address them, specimen names, and specimen orientation. CONCLUSION: We developed a SOR template for pediatric oncology surgery in LRSs. Consensus for all 21 domains and associated subdomains was achieved using a modified Delphi procedure.

The pre-surgical factors that determine the decision to proceed to resection in children diagnosed with high-risk neuroblastoma in a resource limited setting.

Surgical control has prognostic value in neuroblastoma (NB). Advanced NB is common at diagnosis in South Africa. We investigated the pre-surgery factors that influenced decisions to perform surgical resections. We included 204 patients with high-risk NB from a national retrospective study, who completed induction chemotherapy between 2000 and 2016.The median age was 32.4 months (IQR 15.1 - 53.5 months). Primary tumor resection was achieved in 76.9% of patients between 0-18 months of age, 51.8% between 18-60 months and 51.7% older than 60 months (p < 0.001). Only 43.2% of patients with distant metastatic disease had surgery done (p < 0.001). LDH was >750 U/L in 46.8% and ferritin >120 g/dL in 53.1% of those who had surgery (p = 0.005). The majority (80.4%), who had achieved post-induction metastatic complete remission (mCR), were operated, while 28.7% without mCR had surgery (p < 0.001). The long-term overall survival in patients with mCR and primary tumor resection was 36.5% compared to those with mCR without primary tumor resection (25.4%) and without mCR (≤3.0%)(p < 0.001). Age (p < 0.001), stage (p < 0.001), mCR (p < 0.001) and treatment setting (p < 0.001) were of prognostic significance. The tumor site and MYCN-amplification did not significantly predict resection rates. Post-induction mCR and stage were associated with surgical resection and five-year OS (p < 0.001) on multivariate analysis.Patients with high-risk NB who achieved mCR and had primary tumor resections are curable in limited resourced settings. Stage and post-induction mCR were significant variables that led to surgery. These variables should be included as indications in the management of metastatic NB in resource limited settings.

High-risk neuroblastoma that achieved post-induction chemotherapy metastatic remission and have undergone resection, is curable, even in limited resource settings.Achieving metastatic complete remission was the only factor that significantly predicated if surgery was done.The age at diagnosis, stage and hospitals with expertise in neuroblastoma surgery were of prognostic significance in South Africa.If a patient with high-risk neuroblastoma achieves metastatic complete remission in a resource limited setting, it should be an indication for resection of the primary tumor.

Globalization in Pediatric Surgical Training: The Benefit of an International Fellowship in a Low-to-Middle-Income Country Academic Hospital.

OBJECTIVE: A relative oversupply of pediatric surgeons led to increasing difficulties in surgical training in high-income countries (HIC), popularizing international fellowships in low-to-middle-income countries (LMIC). The aim of this study was to evaluate the benefit of an international fellowship in an LMIC for the training of pediatric surgery trainees from HICs. METHODS: We retrospectively reviewed and compared the prospectively maintained surgical logbooks of international pediatric surgical trainees who completed a fellowship at Chris Hani Baragwanath Academic Hospital in the last 10 years. We analyzed the number of surgeries, type of involvement, and level of supervision in the operations. Data are provided in mean differences between South Africa and the respective home country. RESULTS: Seven fellows were included. Operative experience was higher in South Africa in general (Δx̅ = 381; 95% confidence interval [CI]: 236-656; p < 0.0001) and index cases (Δx̅ = 178; 95% CI: 109-279; p < 0.0001). In South Africa, fellows performed more index cases unsupervised (Δx̅ = 71; 95% CI: 42-111; p < 0.0001), but a similar number under supervision (Δx̅ = -1; 95% CI: -25-24; p = 0.901). Fellows were exposed to more surgical procedures in each pediatric surgical subspecialty. CONCLUSION: An international fellowship in a high-volume subspecialized unit in an LMIC can be highly beneficial for HIC trainees, allowing exposure to higher caseload, opportunity to operate independently, and to receive a wider exposure to the different fields of pediatric surgery. The associated benefit for the local trainees is some reduction in their clinical responsibilities due to the additional workforce, providing them with the opportunity for protected academic and research time.

The implementation of a national paediatric oncology protocol for neuroblastoma in South Africa.

PURPOSE: The aim of the World Health Organization-International Paediatric Oncology Society is to improve childhood cancer survival in low- and middle-income countries to 60% by 2030. This can be achieved using standardised evidence-based national treatment protocols for common childhood cancers. The aim of the study was to describe the development and implementation of the SACCSG NB-2017 neuroblastoma (NB) treatment protocol as part of the treatment harmonisation process of the South African Children's Cancer Study Group. METHODS: The Consolidated Framework for Implementation Research was used to identify factors that could influence the implementation of the national NB protocol as a health care intervention. The evaluation was done according to five interactive domains for implementation: intervention characteristics, inner setting, outer setting, individual or team characteristics and the implementation process. RESULTS: The protocol was developed over 26 months by 26 physicians involved in childhood cancer management. The process included an organisational phase, a resource identification phase, a development phase and a research ethics approval phase. Challenges included nationalised inertia, variable research ethical approval procedures with delays and uncoordinated clinical trial implementation. CONCLUSION: The implementation of the national NB protocol demonstrated the complexity of the implementation of a national childhood cancer treatment protocol. However, standardised paediatric cancer treatment protocols based on local expertise and resources in limited settings are feasible.

Long-term memory for elementary visual percepts: memory psychophysics of context and acquisition effects.

In the first phase of each of two experiments, participants learned to associate a set of labels (i.e., consonant-vowel-consonant [CVC]) with a set of line lengths by using a paired-associate learning procedure. In the second phase of each experiment, these learned labels were used as memorial standards in the method of constant stimuli. Psychometric functions and the associated indices of discriminative performance (i.e., Weber fractions [WFs], just noticeable difference, and point of subjective equality) were then obtained for the remembered standards. In Experiment 1, WFs (i.e., the indices of memory precision) obtained with remembered standards were found to be higher (i.e., had poorer discriminability) than were WFs obtained with perceptual standards. In addition, WFs obtained with the remembered standards exhibited serial position effects (i.e., poorer discriminability for central items in the memory ensemble) and systematically varied with set size (i.e., the number of standards in the memory set), but WFs obtained with perceptual standards did not depend on serial position or set size. In Experiment 2, increasing the number of acquisition trials reduced WFs and diminished serial position effects. In addition, WFs did not vary systematically with the "physical" spacing between the standards in memory, but they did with the ordinal spacing. The results are consistent with a noisy analogue representation of remembered magnitudes, whereby central items in a memory ensemble are subject to lateral inhibition and thus reduced discriminability. Finally, presentation order effects, as defined by the classic time-order error, were observed with purely perceptual comparisons but not with comparisons involving a remembered standard. This latter finding is inconsistent with a strong form of the functional equivalence view of perception and memory.