Polites, Stephanie F.

ABSTRACT Introduction Children with hepatoblastoma (HB) and other solid tumors frequently undergo intraoperative blood transfusion (IBT) with unknown impact on oncologic outcomes and scant data to guide transfusion in this population. This study tested the hypothesis that IBT is associated with poorer survival in children with HB. Methods A multicenter retrospective observational study of patients aged <18 years with HB who underwent primary tumor resection, including liver transplantation, from 2010 to 2019 was performed at 19 institutions. The association of IBT with risk of recurrence and death were determined using propensity score reweighted (PSR) Cox proportional hazards regression analyses. Results There were 338 patients identified who met inclusion criteria and had sufficient data for inclusion. Of those, 257 (76%) received IBT, including 253 (98%) who received packed red blood cells (pRBC), 84 (33%) who received plasma, and 28 (11%) who received platelets. IBT was associated with higher pretreatment extent of disease ( p < 0.001), presence of annotation factors (+VPEFR: 50%, n = 129 vs. 37%, n = 30, p = 0.035), and complex resections (extended, meso‐, or total hepatectomy: 54%, n = 139 vs. 27%, n = 22, p < 0.001); these differences were mitigated after applying propensity score weighting. Patients who received IBT had greater postoperative hemoglobin (g/dL) (median 10 (I8–11) vs. 9 (8–10), p = 0.013) and required more postoperative plasma and platelet transfusions ( p < 0.05). Median follow‐up was 4.4 (2.0–8.3) years. Compared with non‐IBT patients, those with IBT had higher incidence of death (PSR HR 2.35, 95% CI 1.10–5.02). Recurrence did not significantly differ across groups (PSR HR = 0.82, 95% CI 0.45–1.48). Conclusion IBT was associated with greater hazard of death. Postoperative hemoglobin levels suggested that unnecessary transfusions occurred and a pRBC‐focused approach to IBT led to coagulopathy. Development of optimal transfusion strategies for HB are needed to minimize unnecessary transfusions.

Surgical outcomes and perioperative factors associated with pediatric cranioplasty remain incompletely understood. The authors queried the National Surgical Quality Improvement Program-Pediatric (NSQIP-P) database to evaluate outcomes and intraoperative/postoperative blood transfusions in patients undergoing cranioplasty between 2019 and 2023. A total of 249 patients (mean age 9.1 ± 5.4 y) were included. Most cranioplasties (70.7%) utilized alloplastic materials compared with autologous. Blood transfusion was required in 24.1% of cases, predominantly on the day of surgery (86.7%). Multivariable analysis identified age (OR: 0.86 per year, 95% CI: 0.80–0.93, P < 0.001) and cranioplasty size >5 cm (versus ≤5 cm) (OR: 2.10, 95% CI: 1.01–4.50, P = 0.049) as predictors of transfusion. The 30-day unplanned readmission rate was 5.3% at a mean of 14.6 ± 7.9 days from surgery, most commonly for a surgical site infection (33.0%). The 30-day unplanned reoperation rate was 2.9%, at a mean of 10.2 ± 5.6 days. Autologous cranioplasty was not associated with higher odds of readmission (OR: 0.79, 95% CI: 0.25–2.13, P = 0.66) or reoperation (OR: 5.04, 95% CI: 0.96–36.99, P = 0.07) compared with alloplastic cranioplasty. Rates of readmission and reoperation following pediatric cranioplasty are comparable to other complex craniofacial procedures. Younger age and larger cranioplasty size are associated with higher rates of blood transfusion. Our results show that autologous reconstruction is not associated with higher short-term complication rates compared with alloplastic reconstruction, although future studies are still needed to further the understanding of the optimal cranioplasty material.

Objective We sought to characterize the frequency of postoperative opioid prescriptions in patients undergoing cleft lip repair (CLR). Design The NSQIP-P 2023 database was queried to identify patients undergoing CLR using CPT codes. Univariable analysis was conducted to identify factors associated with postoperative opioid prescription. Setting A multicenter sample from 157 participating sites. Patients/participants A total of 1497 cases (1215 unilateral CLR and 282 bilateral CLR) were identified. Main outcomes/measures The postoperative outcomes of interest were whether an opioid was prescribed to a patient at discharge and what factors were independently associated with opioid prescribing at discharge. Results A total of 44.6% of patients with a mean age of 5.1 ± 1.9 months were prescribed opioids at discharge, most commonly oxycodone (76.3%). Univariable analysis identified an increase in weight (kg) (OR: 1.09, 95% CI: 1.02-1.17, P = .03), American Society of Anesthesiologists (ASA) classification (ordinal) (OR: 1.25, 95% CI: 1.06-1.47, P = .01), anesthesia time (10 min) (OR: 1.02, 95% CI: 1.01-1.04, P = .03), and age (months) (OR: 1.06, 95% CI: 1.00-1.12, P = .04) as being associated with opioid prescription at discharge. Bilateral versus unilateral CLR ( P = .49) and outpatient versus inpatient operation ( P = .38) were not associated with opioid prescribing. Conclusion Opioid stewardship is important in pediatric care, with our results showing age, weight, anesthesia time, and ASA classifications are predictors of opioid prescription. Further research examining contributors to patient pain following CLR is necessary to optimize opioid prescribing without negatively impacting the patient.

ABSTRACTBackgroundRenal medullary carcinoma (RMC) is an aggressive tumor associated with sickle cell trait. Despite treatment advances for other rare renal tumors, RMC survival remains poor. We aimed to describe the contemporary management and survival of children and adults with RMC.ProcedureIn this multicenter retrospective cohort study, Pediatric Surgical Oncology Research Collaborative sites searched their databases for patients diagnosed with RMC (2000–2022). Descriptive statistics were calculated and survival analyses performed using Kaplan–Meier and Cox regression.ResultsThirty‐four patients with RMC were identified. Median age was 19 years (IQR: 15–28; range: 7–52). Most were male (24/34; 71%), Black (27/32; 84%), had sickle cell trait or disease (30/33; 91%), presented with metastatic disease (27/34; 79%), and were symptomatic at presentation (32/34; 94%). Median overall survival (OS) was 24 months from diagnosis (16 months for children, 28 months for adults, p = 0.6). Receipt of platinum‐based chemotherapy (23/34; 68%) was associated with significantly higher OS than other regimens (35 vs. 5 months, p < 0.001). Nephrectomy (24/34; 71%) was associated with significantly improved OS compared with non‐operative management (34 vs. 7 months, p = 0.001). Immunotherapy, targeted therapy, or radiation therapy were not associated with significant differences in OS, nor were age, sex, race, sickle cell status, SMARCB1/INI‐1, stage, nephrectomy approach, retroperitoneal lymph node dissection, gross residual disease, margins, or tumor size.ConclusionsRMC survival remains poor despite newer therapies. Nephrectomy and platinum‐based chemotherapy should be considered in locally advanced and metastatic disease. Coordinated international cooperative group studies are needed to meaningfully improve RMC survival.

BACKGROUND: Children continue to undergo right hemicolectomy (RHC) for neuroendocrine tumors (NET) of the appendix based on adult guidelines. Appendectomy alone is recommended for the pediatric population; however, there are no data on safety of this approach in the setting of positive margins. This study aimed to determine the association between tumor characteristics and survival in a national cohort, hypothesizing that survival would be excellent despite high-risk features including positive margins on appendectomy. STUDY DESIGN: Patients 18 years of age or younger with NET of the appendix were identified in the National Cancer Database from 2004 to 2022 using ICD-O-3 codes. Characteristics of patients who underwent definitive appendectomy vs RHC were compared using chi-square tests. Five-year survival was determined for appendectomy patients with and without high-risk size, margin, and lymph node (LN) features. RESULTS: Of 1,339 patients, 1,156 (86%) underwent appendectomy and 183 (14%) RHC. Median age in both groups was 15 (13 to 17) years. Patients who underwent RHC had larger tumors (24% vs 4%, greater than 2 cm, p < 0.001), more lymphovascular invasion (31% vs 10%, p < 0.001), and were more likely to undergo LN assessment (82% vs 10%, p < 0.001) with a higher LN positivity rate (31% vs 16%, p = 0.006). There was no difference in margins status (p = 0.76). The 5- and 10-year survival was excellent regardless of tumor characteristics for patients overall (99.9% and 99.4%, respectively) and for those who underwent definitive appendectomy (99.9% and 99.2%). CONCLUSIONS: These data further support appendectomy as definitive management for children with appendiceal NET. Additional resection does not confer a survival benefit even in the setting of positive margins.

Abstract Disclosure: K. Leopold: None. S. Polites: None. L. Binkovitz: None. L. Casas: None. A.N. Lteif: None. Title: An Unexpected Cause of Ga-68 Dotatate PET/CT Somatostatin Receptor Avidity in a Pediatric Patient with Hereditary Paraganglioma-Pheochromocytoma Syndrome due to an SDHA Mutation. Introduction: Individuals with Succinate Dehydrogenase (SDHx) gene mutations have an increased risk of paragangliomas and pheochromocytomas beginning in childhood.Clinical Case: A 9 year old male with Cornelia de Lange syndrome and a paternally inherited SDHA pathogenic gene variant (c1534C&gt;T) presented with worsening migraine headaches and skin pallor with activity. 24 hour urine fractionated metanephrines were mildly elevated, with metanephrine 122 mcg/24h (29-92 mcg/24 h), normetanephrine 187 mcg/24 h (34-169 mcg/24 h), and total metanephrine 309 mcg/24 h (47-223 mcg/24 h). Ga-68 DOTATATE PET/CT showed a focus of moderately increased radiotracer activity in the anterior aspect of the distal gastric antrum with a max SUV 8.2 corresponding to a 9x7 mm nodule on CT in the same area, suggestive of underlying somatostatin receptor positive lesion such as a paraganglioma. MRI with contrast completed to further characterize the lesion showed no visible mass corresponding to the focal area of radiotracer uptake on DOTATATE PET/CT. Repeat 24 h urine metanephrines continued to show mild elevation of metanephrine (138 mcg/24h), normetanephrine (200 mcg/24h), and total metanephrines (338 mcg/24h). 24 hour urine catecholamines, 5-Hydroxyindoleacetic acid, and homovanillic acid were normal. An upper endoscopic ultrasound was completed which confirmed the presence of a lesion near the stomach but not arising from the stomach wall and not consistent with a gastrointestinal stromal tumor. CT enterography with IV contrast showed a 6x6 mm enhancing mass within the distal right lateral gastric wall. Due to ongoing symptoms, mild elevations in urine fractionated metanephrines, and a clear mass on US and CT, the patient underwent laparoscopic abdominal exploration where a 14x14x13 mm mass with surrounding gastric and duodenal serosa was excised with primary closure of the bowel. Pathology showed ectopic pancreatic tissue in the wall of the small bowel and stomach. Repeat Ga-68 DOTATATE PET/CT 5 months following surgery showed resolution of the distal gastric somatostatin receptor avidity. Clinical Lesson: This is the first case of ectopic pancreatic tissue mimicking a paraganglioma in a pediatric patient with hereditary paraganglioma-pheochromocytoma syndrome. False positive imaging should be considered in patients with SDHx mutations when the uptake is atypical in location and when the elevation in urine metanephrines is mild. Presentation: 6/3/2024

AbstractA new generation of disease‐specific molecular imaging agents is poised to revolutionize fluorescence‐guided surgery. Pafolacianine has been approved for adult lung and ovarian cancers. We demonstrate a proof of concept for pediatric surgeons treating young adults with pulmonary metastatic sarcomas. Five successful fluorescence‐guided pulmonary metastasectomy operations were performed in young adult patients with metastatic osteosarcoma or Ewing sarcoma following administration of pafolacianine. All osteosarcoma lesions identified using standard techniques were also markedly fluorescent in patients. Novel fluorescent molecular agents targeted to tumor‐specific receptors have promise of increased sensitivity and specificity for detecting metastatic nodules and enhancing surgical clearance of disease.

AbstractWith an annual cumulative occurrence of approximately 15,000 in North America, all childhood cancers are rare. Very rare cancers as defined by both the European Cooperative Study Group for Rare Pediatric Cancers and the Children's Oncology Group fall into two principal categories: those so uncommon (fewer than 2 cases/million) that their study is challenging even through cooperative group efforts (e.g., pleuropulmonary blastoma and desmoplastic small round cell tumor) and those that are far more common in adults and therefore rarely studied in children (e.g., thyroid, melanoma, and gastrointestinal stromal tumor). Treatment strategies for these latter tumors are typically based on adult guidelines, although the pediatric variants of these tumors may harbor different genetic signatures and demonstrate different behavior. If melanoma and differentiated thyroid cancer are excluded, other rare cancer types account for only 2% of the cancers in children aged 0 to 14. This article highlights several of the most common rare tumor types.

AbstractSynoptic operative notes for pediatric surgical oncology provide standardized and structured documentation of surgical procedures performed on pediatric patients with cancer. These reports capture essential details such as preoperative diagnosis, intraoperative findings, surgical technique, and tumor characteristics in a concise and uniform format. By promoting consistency, accuracy, and completeness in reporting, synoptic operative notes facilitate effective communication among multidisciplinary healthcare teams, enhance quality assurance efforts, and streamline data extraction for research purposes. The integration of synoptic reporting within electronic medical record systems further enhances accessibility and usability, ensuring efficient documentation practices and improved patient care outcomes in pediatric surgical oncology.

Neuroblastoma is the most common extracranial solid tumor diagnosed in children. This inaugural version of the NCCN Guidelines for Neuroblastoma provides recommendations for the diagnosis, risk classification, and treatment of neuroblastoma. The information in these guidelines was developed by the NCCN Neuroblastoma Panel, a multidisciplinary group of representatives with expertise in neuroblastoma, consisting of pediatric oncologists, radiologists, pathologists, surgeons, and radiation oncologists from NCCN Member Institutions. The evidence-based and consensus recommendations contained in the NCCN Guidelines are intended to guide clinicians in selecting the most appropriate treatments for their patients with this clinically heterogeneous disease.