Understanding the Value of Tumor Markers in Pediatric Ovarian Neoplasms

Purpose The purpose of this study was to determine the diagnostic accuracy of tumor markers for malignancy in girls with ovarian neoplasms. Methods A retrospective review of girls 2–21 years who presented for surgical management of an ovarian neoplasm across 10 children's hospitals between 2010 and 2016 was performed. Patients who had at least one concerning feature on imaging and had tumor marker testing were included in the study. Sensitivity, specificity, and negative and positive predictive values (PPV) of tumor markers were calculated. Results Our cohort included 401 patients; 22.4% had a malignancy. Testing for tumor markers was inconsistent. AFP had high specificity (98%) and low sensitivity (42%) with a PPV of 86%. The sensitivity, specificity, and PPV of beta-hCG was 44%, 76%, and 32%, respectively. LDH had high sensitivity (95%) and Inhibin A and Inhibin B had high specificity (97% and 92%, respectively). Conclusions Tumor marker testing is helpful in preoperative risk stratification of ovarian neoplasms for malignancy. Given the variety of potential tumor types, no single marker provides enough reliability, and therefore a panel of tumor marker testing is recommended if there is concern for malignancy. Prospective studies may help further elucidate the predictive value of tumor markers in a pediatric ovarian neoplasm population

Multi-institutional review of the preoperative diagnostic accuracy for pediatric ovarian mature cystic teratomas

Study objective: To assess the preoperative imaging impression and surgeon diagnostic accuracy for pediatric ovarian mature cystic teratomas (MCTs) DESIGN: Retrospective review SETTING: Eleven pediatric hospitals PARTICIPANTS: Patients ages 2 to 21 who underwent surgical management of an ovarian neoplasm or adnexal torsion with an associated ovarian lesion INTERVENTION: None MAIN OUTCOME MEASURES: Preoperative imaging impression, surgeon diagnosis, tumor markers, and pathology RESULTS: Our cohort included 946 females. Final pathology identified 422 (45%) MCTs, 405 (43%) other benign pathologies, and 119 (12%) malignancies. Preoperative imaging impression for MCTs had a 70% sensitivity, 92% specificity, 88% positive predictive value (PPV), and 79% negative predictive value (NPV). For the preoperative surgeon diagnosis, sensitivity was 59%, specificity 96%, PPV 92%, and NPV 74%. Some measures of diagnostic accuracy were affected by the presence of torsion, size of the lesion on imaging, imaging modality, and surgeon specialty. Of the 352 masses preoperatively thought to be MCTs, 14 were malignancies (4%). Eleven patients with inaccurately diagnosed malignancies had tumor markers evaluated and 82% had at least 1 elevated tumor marker, compared with 49% of those with MCTs. Conclusions: Diagnostic accuracy for the preoperative imaging impression and surgeon diagnosis is lower than expected for pediatric ovarian MCTs. For all ovarian neoplasms, preoperative risk assessment including a panel of tumor markers and a multidisciplinary review is recommended. This process could minimize the risk of misdiagnosis and improve operative planning to maximize the use of ovarian-sparing surgery for benign lesions and allow for appropriate resection and staging for lesions suspected to be malignant

Can Fecal Continence be Predicted in Patients Born with Anorectal Malformations?

Purpose The purpose of this study was to identify factors associated with attaining fecal continence in children with anorectal malformations (ARM). Methods We performed a multi-institutional cohort study of children born with ARM in 2007–2011 who had spinal and sacral imaging. Questions from the Baylor Social Continence Scale were used to assess fecal continence at the age of ≥ 4 years. Factors present at birth that predicted continence were identified using multivariable logistic regression. Results Among 144 ARM patients with a median age of 7 years (IQR 6–8), 58 (40%) were continent. The rate of fecal continence varied by ARM subtype (p = 0.002) with the highest rate of continence in patients with perineal fistula (60%). Spinal anomalies and the lateral sacral ratio were not associated with continence. On multivariable analysis, patients with less severe ARM subtypes (perineal fistula, recto-bulbar fistula, recto-vestibular fistula, no fistula, rectal stenosis) were more likely to be continent (OR = 7.4, p = 0.001). Conclusion Type of ARM was the only factor that predicted fecal continence in children with ARM. The high degree of incontinence, even in the least severe subtypes, highlights that predicting fecal continence is difficult at birth and supports the need for long-term follow-up and bowel management programs for children with ARM

Laparoscopy versus laparotomy for pediatric ovarian dermoids

Background: With increased surgeon comfort using laparoscopy, we hypothesized resection of pediatric ovarian dermoids using laparoscopy would yield a shorter length of stay and no increase in morbidity or recurrence compared to laparotomy. Methods: A retrospective review was performed amongst eleven pediatric hospitals. Patients aged 2 to 21 who underwent resection of an ovarian dermoid from 2010 to 2020 were included. Patient characteristics, operative details, and outcomes by approach were evaluated using Chi-squared and Wilcoxon-Mann tests. Results: 466 patients were included, with a median age of 14.4 and median follow-up of 4.0 months. 279 patients underwent laparoscopy (60%), 139 laparotomy (30%), and 48 laparoscopy converted to laparotomy (10%). There were no differences in rates of tumor spillage by approach (p = 0.15). 65% underwent ovarian-sparing surgery and 35% underwent oophorectomy. Length of stay was significantly shorter amongst patients who underwent laparoscopy (1 day versus 2 days for laparotomy and converted, p\u3c0.0001). There were no differences in rates of suspected recurrence or reoperation (p = 0.19 and p = 0.57, respectively). Conclusion: Patients who underwent laparoscopy experienced no differences in the rates of tumor spillage, recurrence, or reoperation and had a shorter length of stay compared to laparotomy. Laparoscopy is an acceptable approach for resection of pediatric ovarian dermoids

Development of a multi-institutional registry for children with operative congenital lung malformations

Introduction The purpose of this study was to develop a multi-institutional registry to characterize the demographics, management, and outcomes of a contemporary cohort of children undergoing congenital lung malformation (CLM) resection. Methods After central reliance IRB approval, a web-based, secure database was created to capture retrospective cohort data on pathologically-confirmed CLMs performed between 2009 and 2015 within a multi-institutional research collaborative. Results Eleven children's hospitals contributed 506 patients. Among 344 prenatally diagnosed lesions, the congenital pulmonary airway malformation volume ratio was measured in 49.1%, and fetal MRI was performed in 34.3%. One hundred thirty-four (26.7%) children had respiratory symptoms at birth. Fifty-eight (11.6%) underwent neonatal resection, 322 (64.1%) had surgery at 1–12 months, and 122 (24.3%) had operations after 12 months. The median age at resection was 6.7 months (interquartile range, 3.6–11.4). Among 230 elective lobectomies performed in asymptomatic patients, thoracoscopy was successfully utilized in 102 (44.3%), but there was substantial variation across centers. The most common lesions were congenital pulmonary airway malformation (n = 234, 47.3%) and intralobar bronchopulmonary sequestration (n = 106, 21.4%). Conclusion This multicenter cohort study on operative CLMs highlights marked disease heterogeneity and substantial practice variation in preoperative evaluation and operative management. Future registry studies are planned to help establish evidence-based guidelines to optimize the care of these patients

Current operative management of congenital lobar emphysema in children: A report from the Midwest Pediatric Surgery Consortium

Purpose The purpose of this study was to evaluate the clinical presentation and operative outcomes of patients with congenital lobar emphysema (CLE) within a large multicenter research consortium. Methods After central reliance IRB-approval, a retrospective cohort study was performed on all operatively managed lung malformations at eleven participating children's hospitals (2009–2015). Results Fifty-three (10.5%) children with pathology-confirmed CLE were identified among 506 lung malformations. A lung mass was detected prenatally in 13 (24.5%) compared to 331 (73.1%) in non-CLE cases (p < 0.0001). Thirty-two (60.4%) CLE patients presented with respiratory symptoms at birth compared to 102 (22.7%) in non-CLE (p < 0.0001). The most common locations for CLE were the left upper (n = 24, 45.3%), right middle (n = 16, 30.2%), and right upper (n = 10, 18.9%) lobes. Eighteen (34.0%) had resection as neonates, 30 (56.6%) had surgery at 1–12 months of age, and five (9.4%) had resections after 12 months. Six (11.3%) underwent thoracoscopic excision. Median hospital length of stay was 5.0 days (interquartile range, 4.0–13.0). Conclusions Among lung malformations, CLE is associated with several unique features, including a low prenatal detection rate, a predilection for the upper/middle lobes, and infrequent utilization of thoracoscopy. Although respiratory distress at birth is common, CLE often presents clinically in a delayed and more insidious fashion