Successful Laparoscopic Management of Zinner Syndrome in a Pediatric Patient.

Zinner syndrome, a rare congenital anomaly affecting males, is characterized by atresia of the ejaculatory duct, seminal vesicle cysts, and ipsilateral renal agenesis. This case report details a 2-year-old boy successfully treated with laparoscopic excision of a dilated vas deferens and seminal vesicle cyst. The rarity of Zinner syndrome in pediatric patients underscores the importance of understanding its diagnosis and minimally invasive surgical management.

Exosomal long non-coding RNA MALAT1: a candidate of liquid biopsy in monitoring of Wilms' tumor.

PURPOSE: Wilms' tumor (WT) is a rare kidney cancer that primarily affects children. Exosomes are extracellular vesicles that cargo nucleic acids, proteins,etc. for cellular communication. Long non-coding RNAs (lncRNAs) have utility as biomarkers for cancer diagnosis, prognosis, and disease monitoring. We hypothesize that expression of lncRNA, metastasis-associated lung adenocarcinoma transcript-1(MALAT1), is dysregulated and possibly trafficked within exosomes to influence the tissue microenvironment for metastasis and recurrence of WT. METHODS: We investigated the expression of MALAT1 in thirty WT samples by qPCR. Exosomes were isolated using a precipitated and affinity-binding-based kit, and characterized using TEM, NTA, and DLS. RESULTS: Mean number of exosomes was 9.01×108/mL in primary culture, 1.64×108/mL in urine, and 4.65×108/plasma:400µl. Average yield of total RNA was 1.28µg (primary-culture supernatant:1ml), 1.47µg (Urine:1ml), 1.65µg (Plasma:400 µL). We quantified MALAT1 in exosomes derived from these sources in patients of WT. Expression of MALAT1 was significantly downregulated (p=0.008) in WT samples. CONCLUSION: This is the first study that demonstrated the presence of lncRNA MALAT1 in various invasive and non-invasive samples of patients with WT(primary tissue culture, urine, and plasma samples).

Sex Bias in Treatment Abandonment of Childhood Cancer in India.

OBJECTIVES: To explore the magnitude of sex bias and determinants of treatment abandonment (TA) in childhood cancer in India. METHODS: Individual data of children (0-19 y) registered between January 1, 2017 and July 31, 2022, was compiled. TA was defined as defaulting curative intent treatment ≥4 wk. Defaulting treatment irrespective of intent ≥4 wk was defined as Treatment Default (TD). The primary outcome was the proportion of male-to-female children with TA. Secondary outcomes included the proportion of male-to-female children with upfront TA, TA at relapse, TD, TD-p (TD only in the palliative setting). The impact of clinico-demographic factors on TA was analysed using multivariable regression and propensity score matching (PSM). RESULTS: Three thousand two hundred eighty four patients were analysed. The overall male-to-female ratio (MFR) was 2.08 (95% CI 1.94-2.24). Of 2906 patients treated with curative intent, 415 (14·3%) abandoned treatment. TA was higher in females than males (16·4% vs. 13·3%; p = 0·022) with adjusted MFR of 0·81 (0·66-0·98). The adjusted MFR of TA for treatment-naïve and relapsed patients and TD were 0·73 (0·59-0·91), 1·13 (0·65-1·96) and 0·84 (0·71-1·00) respectively. Sex independently predicted TA on multivariable analysis. However, on PSM analysis including socio-economic variables, lower maternal education predicted higher TA in children with cancer (10·1% vs. 6%, p = 0·015). CONCLUSIONS: Child sex predicted TA in childhood cancer in India with more females abandoning treatment. Maternal education is a more crucial factor predicting TA over child sex, when socio-economic factors were considered. Hence, policies promoting female education and gender equality may mitigate sex-based gaps in childhood cancer care.

Perinatal Outcome in Congenital Diaphragmatic Hernia (CDH): A Single-Center Experience.

Objective: To study the perinatal outcome in fetuses diagnosed with congenital diaphragmatic hernia (CDH). Methods: Thirty-two pregnant women with antenatal diagnosis of CDH in fetus, who delivered between 2018 and 2021, were included in the study. Postnatally eventration of diaphragm was diagnosed in 3 neonates and were excluded. Results: The median gestational age at diagnosis was 23 weeks (IQR: 216-261 weeks). The mean O/E LHR was 34.88 ± 9.03%, and the O/E LHR was significantly lower in fetuses who did not survive (40.81 ± 4.25 vs 31.26 ± 9.33; p = 0.0037). On ROC analysis, at a cutoff of ≤ 32.93, O/E LHR had a specificity of 100% with a sensitivity of 72.22% in predicting mortality. Cases with liver herniation were not significantly different between survivors versus non-survivors. The overall survival rate was 37.93%, and the leading cause of death was severe persistent pulmonary hypertension. Conclusion: O/E LHR can predict mortality in neonates with antenatal diagnosis of CDH. The presence of pulmonary hypertension was the leading cause of death in these neonates.

Utility of Ischemia-Modified Albumin as a Biomarker for Acute Appendicitis: A Systematic Review and Meta-Analysis.

BACKGROUND: Acute appendicitis is a frequently encountered surgical emergency. Despite several scoring systems, the possibility of delayed diagnosis persists. In addition, a delayed diagnosis leads to an increased risk of complicated appendicitis. Hence, there is a need to identify biological markers to help clinicians rapidly and accurately diagnose and prognosticate acute appendicitis with a high sensitivity and specificity. Although several markers have been evaluated, the pressing concern is still the low specificity of these markers. One such marker is serum ischemia-modified albumin (IMA), which can be a novel biomarker for accurately diagnosing and prognosticating acute appendicitis. METHODS: The authors conducted a systematic search of the PubMed, EMBASE, Web of Science, and Scopus databases through February 2023 as per the PRISMA guidelines. The difference in the levels of IMA between patients with acute appendicitis vs. healthy controls, and the difference in the levels of IMA between patients with complicated vs. non-complicated acute appendicitis were taken as the outcome measures. Statistical analysis was performed using a random effects model and mean difference (MD) was calculated. The methodological quality of the studies was assessed by utilizing the Newcastle-Ottawa scale. RESULTS: A total of six prospective comparative studies were included in the meta-analysis. The analysis revealed that the mean level of serum IMA was significantly raised in the acute appendicitis group (MD 0.21, 95% CI 0.05 to 0.37, p = 0.01). Similarly, the mean serum IMA levels were also raised in the complicated appendicitis group compared to the non-complicated appendicitis group (MD 0.05, 95% CI 0.01 to 0.10, p = 0.02). Three of the studies included were, however, of poor methodological quality. CONCLUSIONS: Serum IMA is a viable potential marker for diagnosing and prognosticating acute appendicitis. However, due to the limited methodological quality of available studies, further prospectively designed and adequately powered studies are needed.

Anthropometric Outcomes in Survivors of Pediatric Solid Tumors.

Nutritional status is an important aspect of childhood cancer, with a bearing on the disease and subsequent survivorship. We sought to evaluate the long-term anthropometric outcomes in a cohort of pediatric solid tumor survivors treated between 1994 and 2016. Anthropometry was noted at the time of presentation, after completion of therapy, and at the last follow-up. The z-scores for weight-for-age, height-for-age, and BMI-for-age were calculated using WHO growth charts for ages <5 years and the Indian Academy of Pediatrics growth charts for age >/= 5 years. BMI was calculated for adult survivors. We included 317 survivors, comprising 48, 81, and 188 survivors of Hepatoblastoma (HB), Malignant Germ cell Tumor (MGCT), and Wilms Tumor (WT) respectively. The median age at diagnosis was 24.5 (IQR 59-13.2) months, with a follow-up ranging from 5 to 19.54 years. The z-scores of the collective cohort and individual cohorts of HB, MGCT, and WT showed an improving trend from diagnosis to the last follow-up. The difference in the prevalence of malnutrition was found to be statistically significant when any two-time points were compared. Of the 28 adult survivors, 43% were noted to be underweight. Thus, anthropometric measures improve during follow-up, however, up to 15% of children persist in being malnourished.

Novel Scoring Systems and Age-Based Hepatic Shear Wave Stiffness Cut-Offs for Improving Sonographic Diagnosis of Biliary Atresia.

OBJECTIVES: To make sonographic evaluation for biliary atresia (BA) more objective and reproducible using scoring systems, and evaluate hepatic shear wave elastography (SWE) as an adjunct in sonographic diagnosis of BA. METHODS: Sixty-four infants with cholestatic jaundice were enrolled between June 2016 and March 2018 in this prospective observational cohort study. Sonography and SWE was performed with SuperSonic Aixplorer system. Novel scoring systems were developed incorporating established sonographic parameters and hepatic stiffness values and analysed using SPSS software. RESULTS: Of the 18 patients confirmed as BA, 3 were misdiagnosed on conventional sonography (16.7%) as non-BA. Gall bladder (GB) wall irregularity and fasting GB length were the most accurate (93.8%) and most specific (97.8%) individual parameters, respectively. A significant difference was noted in the triangular cord (TC) thickness of BA and non-BA infants (p <0.001), with a high specificity of 95.6% for a 4 mm cut-off value for a positive TC sign. Comparison of hepatic SWE stiffness among age-matched groups of BA and non-BA showed significant differences (≤60 d: p = 0.003; >60 d: p <0.001) but with a reduced accuracy (93.8%). Diagnostic accuracy of greyscale scoring system (96.9%), greyscale + elastography scoring system in ≤60 d (94.4%) and >60 d (97.8%) were better than that of conventional sonographic diagnosis (93.8%). CONCLUSIONS: Grey scale scoring system improves the accuracy of sonographic diagnosis of BA without any additional cost or time penalty along with making it universally reproducible. SWE has only an adjunctive role, if any, in the sonographic diagnosis of BA.

Preoperative Triple-Phase Three-Dimensional-Multi-Detector Computed Tomography Imaging of the Hepatic Vascular Tree: An Accurate Road Map Prior to Tumor Resection in Hepatoblastoma.

Objective: To evaluate the hepatic vasculature/tumor relations in hepatoblastoma patients with three-dimensional (3D) reformatted images after triple-phase multi-detector computed tomography (MDCT) and to compare these with the surgical findings to judge the accuracy of this investigation. Materials and Methods: The study was carried out in hepatoblastoma patients after appropriate neo-adjuvant chemotherapy, prior to resection. Images were postprocessed at a dedicated workstation for multi-planar reformations, maximum intensity projection, curved planar reformations, and volume-rendered technique reconstructions. The reporting was done as per a specific protocol by both the radiologist and surgeon (per-operative findings) and the accuracy of MDCT ascertained as per concordance between the surgical and imaging findings. Results: Fourteen children (13 boys, 1 girl) underwent surgery. Clinically, relevant information regarding vascular, tumor involvement, and interface with vessels was provided by the study in all cases. Although all tumors were deemed resectable on preoperative imaging, one procedure was abandoned due to an unanticipated portal cavernoma. While a few anatomical variations were unexpectedly encountered during surgery, there was good concordance overall between findings on imaging and surgical exploration. Conclusions: MDCT with 3D reformatting provides accurate virtual representations of the hepatic tumor. This allows simulation of surgical resection with decreased risk of vascular injury and postoperative liver failure.

Cytomorphology of paediatric hepatocellular carcinoma: A useful diagnostic adjunct.

INTRODUCTION: Hepatocellular carcinoma (HCC) is a common primary malignancy of the liver but is rare in the paediatric age group; thus, it may be misdiagnosed as the more common tumour, hepatoblastoma. Management varies in both these tumours, and pathological diagnosis thus plays an important role for definitive therapy. Only a few case reports available in the literature have described the cytological characteristics of paediatric HCC. The present study was thus planned to evaluate the cytomorphological features of paediatric HCC. METHODS: Cases diagnosed with HCC on ultrasound-guided fine needle aspiration cytology over a period of 14 years were retrieved. The cases were evaluated for detailed cytological features including cellularity, architecture, sinusoidal wrapping, trabecular thickness, necrosis, anisonucleosis, chromatin, nucleoli, nuclear contours, bi- or multinucleation, intranuclear and intracytoplasmic inclusions, naked nuclei, extra-medullary haematopoiesis, monomorphism, and nuclear overlapping. RESULTS: Twelve cases of HCC were included in the study. The median age at diagnosis was 10 years. Serum alpha-fetoprotein level was raised in most of them. Five of the 12 cases were characterised as moderately differentiated, three as poorly differentiated, two as well differentiated, and two as the fibrolamellar type of HCC. Cytohistological correlation was performed in seven cases. CONCLUSIONS: Ultrasound-guided fine needle aspiration serves as a useful tool to diagnose paediatric HCC and differentiate it from other primary hepatic malignancies, especially hepatoblastoma which closely mimics HCC in this age group, as serum alpha protein levels and imaging findings are unable to distinguish these two tumours.

Pediatric Adrenocortical Neoplasms: A Study Comparing Three Histopathological Scoring Systems.

Adrenocortical neoplasms are rare in childhood. Their histopathological categorization into benign and malignant is often challenging, impacting further management. While the AFIP/Wieneke scoring system is widely used for the prognostic classification of these tumors, it has limitations. Few other tumor scoring systems have evolved over the past few years. These have been validated in adults but not yet in pediatric patients. We evaluated a cohort of pediatric adrenocortical neoplasms to assess the applicability of AFIP/Wieneke criteria and the recently introduced Helsinki score and reticulin algorithm in predicting clinical outcomes. A tumor was considered 'clinically aggressive' in the presence of any of the following: metastases, recurrence, progressive disease, or death due to disease. Cases without any such event were considered 'clinically good'. Event-free survival time was the duration from the date of clinical presentation to any post-operative adverse event. For overall survival analysis, the endpoint was either the last follow-up or death due to disease.Using ROC curve analysis, the obtained cut-off Helsinki score of 24 could stratify the cases into two prognostically relevant groups. Survival analysis showed significant differences in the event-free and overall survival of these two groups of patients, validating the proposed cut-off. None of the three histopathological scoring systems could predict an unfavorable outcome with 100% accuracy. All showed a sensitivity of ≥ 80%, with the reticulin algorithm achieving 100% sensitivity. The specificity and accuracy of the AFIP/Wieneke criteria were the lowest (62.5% and 73.08%, respectively). While the Helsinki score (at the cut-off score of 24) and the reticulin algorithm had similar accuracy rates (80.77%, and 80%, respectively), the specificity of the former was higher (81.25%) than the latter (68.75%). A separate analysis revealed that the Ki-67 index at a cut-off of 18% had a sensitivity of 80% and a specificity of 81.25% for predicting an unfavorable outcome.

Assessing the Feasibility of Predicting Intermediate Outcomes of Esophageal Atresia Using Okamoto Classification: A Prospective Study.

Purpose: Several risk classifications based on various preoperative factors have been proposed to prognosticate the immediate survival of children operated for esophageal atresia. A major drawback of these classifications is that they only focus on immediate survival while ignoring the long-term morbidity and mortality in these children. Our study aims to bridge this gap in knowledge by studying the impact of one such classification (Okamoto's classification) on mortality and morbidity during a period of 1 year after discharge from the hospital in operated cases of esophageal atresia. Materials and Methods: After institutes ethical clearance, 106 children operated for esophageal atresia-tracheoesophageal fistula between 2012 and 2015 were studied prospectively for a period of 1 year after their discharge. The children were graded as per Okamoto classification. The primary objective was to determine the efficacy of this classification in predicting the survival rates in infancy and the secondary objective was to compare the complication rates in these children based on the classification. Results: Sixty-nine children met the inclusion criteria. There were 40, 15, 10, and 4 children in Okamoto Classes I, II, III, and IV, respectively. Twenty-one patients (30%) died during the follow-up period with the maximum number of deaths occurring in Okamoto Class IV (75%) and the minimum in Okamoto Class I (17.5%) (P = 0.003). There was a significant correlation between the Okamoto classes with the incidence of poor weight gain (P = 0.001), lower respiratory tract infection (P = 0.007), and failure to thrive (P = 0.01) higher in Okamoto IV and III as compared to I and II. Conclusion: Okamoto prognostic classification during the initial hospitalization is relevant even at 1 year follow-up with increased mortality and morbidity in Okamoto Class IV as compared to Class I.

Role of Procalcitonin as a Biomarker in Early Identification of Adverse Events Following Esophageal Atresia Surgery.

Introduction: Surgical complication following esophageal atresia repair is one of the several factors known to influence the final outcomes. Early identification of such complications may help in timely institution of therapeutic measures and translate into improved prognosis. Objective: The objective of this study was to evaluate the role of procalcitonin in early prediction of the adverse events after surgery in patients of esophageal atresia and the temporal relationship with clinical manifestations and other inflammatory biomarkers such as C-reactive protein (CRP). Materials and Methods: This was a prospective study on consecutive patients of esophageal atresia (n = 23). Serum procalcitonin and CRP levels were assessed at baseline (prior to surgery) and on postoperative days (POD) 1, 3, 5, 7, and 14. The trends in the biomarker values and temporal relationships of deviation in trend with the clinical and conventional laboratory parameters and patient outcomes were analyzed. Results: Baseline serum procalcitonin was elevated (n = 23; 1.7 ng/ml: min: 0.07 ng/ml-max: 24.36 ng/ml) in 18/23 (78.3%) patients. Procalcitonin nearly doubled on POD-1 (n = 22; 3.28 ng/ml: min: 0.64 ng/ml-max: 16.51 ng/ml) followed by a gradual decline. CRP was also elevated on POD-1 (three times the baseline) and depicted a delayed peak at POD-3. POD-1 procalcitonin and CRP levels correlated with survival. POD-1 procalcitonin cutoff at 3.28 ng/ml predicted mortality with a sensitivity and specificity of 100% and 57.9% (P = 0.05). Serum procalcitonin and CRP were higher for patients who sustained complications, so was the time required for hemodynamic stabilization. Procalcitonin (baseline and POD-5) and CRP (POD-3 and POD-5) values correlated with the clinical course after surgery. Baseline procalcitonin cutoff at 2.91 ng/ml predicted the possibility of a major complication with a sensitivity of 71.4% and a specificity of 93.3%. POD-5 procalcitonin cutoff at 1.38 ng/ml predicted the possibility of a major complication with a sensitivity of 83.3% and a specificity of 93.3%. Patients who sustained major complications depicted a change in serum procalcitonin trend 24-48 h ahead of clinical manifestation of an adverse event. Conclusions: Procalcitonin is a good indicator to identify the adverse events in neonates after surgery for esophageal atresia. The procalcitonin levels in patients who sustained a major complication depicted a reversal in trend 24-48 h of clinical manifestation. POD-1 procalcitonin correlated with survival while the baseline and POD-5 serum procalcitonin predicted the clinical course.

Unjumbling the TWIST score for testicular torsion: systematic review and meta-analysis.

OBJECTIVE: A systematic review and meta-analysis of the studies evaluating the utility of the Testicular Work-up for Ischemia and Suspected Torsion (TWIST) score in establishing or excluding the diagnosis of testicular torsion (TT) is herewith presented in an attempt to quantify the available evidence. METHODS: The study protocol was outlined in advance. The review has been conducted in accordance with Preferred Reporting Items for Systematic Reviews and Meta-analysis (PRISMA). The PubMed, PUBMED Central, PMC databases & Scopus followed by Google (Scholar & search engine) were systematically interrogated with the keywords TWIST score, testis and testicular torsion. Fourteen sets of data (n = 1940) from 13 studies were included; data from 7 studies (giving a detailed score-wise break-up) (n = 1285) were dis-integrated and re-integrated to tweak the cut-offs for low and high risk. RESULTS: For every 4 patients presenting to the Emergency Department (ED) with acute scrotum, one patient will eventually be diagnosed with TT. The mean TWIST score was higher in patients with testicular torsion (5.13 ± 1.53 vs 1.50 ± 1.40 for those without TT). TWIST score can be used to predict testicular torsion at cut-off of 5 with a sensitivity, specificity, PPV, NPV, and accuracy of 0.71 (0.66, 0.75; 95%CI), 0.97 (0.97, 0.98; 95%CI), 90.2%, 91.0%, and 90.9% respectively. While the slider for cut-off was shifted from 4 to 7, there was a rise in specificity and PPV of the test with a corresponding decline in sensitivity, NPV, and accuracy. The sensitivity witnessed a sharp decline from 0.86 (0.81-0.90; 95%CI) @ cut-off 4 to 0.18 (0.14-0.23; 95%CI) @ cut-off 7. The area under the SROC curve for cut-off 5 was more than that for cut-offs 4, 6 & 7. TWIST cut-off of 2 may be used to predict the absence of testicular torsion with a sensitivity, specificity, PPV, NPV, and accuracy of 0.76 (0.74, 0.78; 95%CI), 0.95 (0.93, 0.97; 95%CI), 97.9%, 56.5%, and 80.7%, respectively. While the cut-off is lowered from 3 to 0, there is a corresponding rise in the specificity and PPV, while the sensitivity, NPV, and accuracy are compromised. The sensitivity witnesses a sharp decline from 91 to 35%. The area under the SROC curve for cut-off 2 was more than that for cut-off @ 0, 1 or 3. The sum of sensitivity and specificity of TWIST scoring system to ascertain the diagnosis of TT is more than 1.5 for cut-off values 4 & 5 only. The sum of sensitivity and specificity of TWIST scoring system to confirm the absence of TT is more than 1.5 for cut-off values 3 & 2 only. CONCLUSION: TWIST is a relatively simple, flexible, and objective tool which may be swiftly administered even by the para-medical personnel in the ED. The overlapping clinical presentation of diseases originating from the same organ may prevent TWIST from absolutely establishing or refuting the diagnosis of TT in all the patients with acute scrotum. The proposed cut-offs are a trade-off between sensitivity and specificity. Yet, the TWIST scoring system is immensely helpful in the clinical decision-making process and saves time-lag associated with investigations in a significant majority of patients.

Quality of life in paediatric solid tumours: a randomised study of metronomic chemotherapy versus placebo.

OBJECTIVE: Health-related quality of life (HRQoL) is an important outcome for paediatric cancer studies. We compared the HRQoL between patients of progressive paediatric solid tumours randomised to metronomic chemotherapy versus placebo. METHODS: In this double-blinded, placebo-controlled randomised study of 108 children with progressive malignancies, HRQoL was evaluated using the PedsQOL Cancer module V.3 at baseline (A1), A2 (9 weeks or earlier if progressed) or A3 (18 weeks or earlier if progressed). RESULTS: There was no statistically significant difference in the change in quality of life produced by each arm from A1 to A2 in either mean total scores or individual domain scores, reported by children or their parents. On analysing the response according to the minimal clinically important difference, defined as an improvement by 4.5 points, we found no significant differences, be it among bone-sarcomas, other tumours, responders (those who received ≥9 weeks of treatment) or non-responders. CONCLUSIONS: The present study concludes that there was no significant difference in HRQoL, between the patients in the two arms at second and later assessments. This is consistent with the other survival endpoints in the study. TRIAL REGISTRATION NUMBER: Clinical trial registration: clinicaltrials.gov Identifier: NCT01858571.

Management and Outcomes of Children with Stage 4S (MS) Neuroblastoma: A Single-Center Experience from a Resource-Challenged Nation.

OBJECTIVES: To demonstrate the clinical profile, management options, and outcomes of children with stage 4S neuroblastoma (NB 4S) diagnosed at a tertiary care center in a resource-challenged nation. The authors also intend to highlight the factors associated with an unfavorable prognosis in this series of patients. METHODS: The archives for children with NB 4S, diagnosed over a 24-y period (January 1996-December 2019), were retrospectively retrieved. Data on patient characteristics, management protocols, oncologic outcomes, and overall survival (OS) were reviewed. Multivariate logistic- regression analysis was performed to identify the factors independently predicting unfavorable outcomes. RESULTS: A total of 22 children (59% males) were included. Adrenal was the most common (82%) primary site. Liver involvement (100%), bone marrow infiltration (23%), and subcutaneous nodules (9%) were observed upon evaluation. Management involved supportive treatment (22%), chemotherapy only (41%), chemotherapy and tumor excision (28%). Ventral hernia was created in two children (9%) due to abdominal compartment syndrome (ACS). Four children died (4/22; 18%) due to ACS (n = 2) and refractory coagulopathy (n = 2). There were no recurrences and all survivors were disease-free. The 5-y OS was 81.8% with a median follow-up duration of 31 mo (range 9 mo-22 y). Age < 2 mo (p = 0.002), respiratory distress at presentation (p < 0.001), and chemotherapy nonresponsiveness (p < 0.001) were significantly associated with mortality. All three factors were independent predictors of mortality. CONCLUSION: Children with NB 4S have a favorable outcome with 5-y OS of 81.8%. Age < 2 mo, respiratory distress at presentation, and chemotherapy nonresponsiveness are independent predictors of a poor outcome.

Pediatric Primary Yolk Sac Tumour of the Kidney: Recommendations for Pretreatment Diagnosis.

Introduction Although nephroblastomas are frequently treated without prior biopsy, there are the occasional other pediatric renal tumors that require different management. In the literature, there are around 30 primary renal germ cell tumors (GCT), including four cases of Yolk sac tumor (YST). We present another primary renal YST.Case report: A five-year-old boy was diagnosed as Wilms tumor on radiology and needle biopsy. He received chemotherapy, with no response. The post-chemotherapy resection specimen revealed a YST.Conclusion: Renal YST may be indistinguishable from Wilms tumor clinically and radiologically. For pre-biopsy chemotherapy management protocols, serum tumor markers such as AFP may be recommended to identify the occasional GCT, including YST. Pre-chemotherapy needle biopsies may lead to misdiagnosis, and may require confirmation by an experienced pathologist or central review.

Response to Neoadjuvant Chemotherapy and Outcomes in Children With Wilms Tumor With Caval Thrombus: A Single Center Experience.

AIM: The aim of our study is to present our experience in the management and outcome of Wilms tumor with intracaval thrombus. MATERIALS AND METHODS: All children with Wilms tumor with intracaval thrombus who presented to us from July 2000 to December 2017 were reviewed retrospectively. We evaluated the tumor stage, management, and outcomes in these patients. RESULTS: Thirty-four patients were included in the study. The median age of presentation was 48 months (11 to 84 mo). Preoperative chemotherapy was given in 32 (94%), with a median duration of 8 weeks. Intracaval thrombus completely resolved in 9 (26%) children after neoadjuvant chemotherapy. Surgical intervention for residual inferior vena cava (IVC) thrombus was performed in 32 patients. The median follow-up was 30 months (5 to 150 mo). At the last follow-up, 24 patients (70%) were alive and disease free. The 5-year overall survival (OS) and event-free survival were 67% (95% confidence interval, 50% to 84%) and 59% (95% confidence interval, 42% to 76%). The OS in children with nonmetastatic disease (94%) was significantly higher than those with metastases (29%; P <0.01). The OS in children with complete resolution of IVC thrombus (100%) was significantly higher than those with persistent thrombus (48%; P =0.025). Analysis of survival outcomes in children with nonmetastatic disease (stage III) revealed no significant difference on comparison with cohort with stage III disease with absence of IVC thrombus. The P -value was 0.224 and 0.53 for 5-year OS and event-free survival, respectively. CONCLUSION: The management of Wilms tumor can be complicated by the presence of caval thrombus. Patients with metastasis have a significantly poor outcome. Patients in whom, there is complete resolution of intracaval thrombus on neoadjuvant chemotherapy have a significantly higher OS.

Sex disparity in childhood cancer in India: a multi-centre, individual patient data analysis.

BACKGROUND: Sex disparity and its determinants in childhood cancer in India remain unexplored, with scarce information available through summary statistics of cancer registries. This study analysed the degree of sex bias in childhood cancer in India and its clinical and demographical associations. METHODS: In this retrospective, multicentre cohort study, we collected individual data of children (aged 0-19 years) with cancer extracted from the hospital-based records of three cancer centres in India between Jan 1, 2005, and Dec 31, 2019, and two population-based cancer registries (PBCRs; Delhi [between Jan 1, 2005, and Dec 31, 2014] and Madras Metropolitan Tumour Registry [between Jan 1, 2005, and Dec 31, 2017]). We extracted data on age, sex, and confirmed diagnosis of malignancy (according to the International Classification of Diseases-10 coding),and excluded participants if they were without a recorded diagnosis, had a benign diagnosis, had missing sex information, resided outside of India, or were a donor for haematopoietic stem cell transplantation (HSCT). The primary outcome was the male-to-female incidence rate ratio (MF-IRR) in the two PBCRs and the male-to-female ratios (MFR) from the hospital-based and the HSCT data. For PBCR data, MF-IRR was estimated by dividing the MFR by the total population at risk. MFR was analysed for patients seeking treatment at the cancer centres and for those undergoing HSCT. Logistic regression analyses were done to explore the association of clinical and demographical variables with sex of the patients seeking treatment and those undergoing HSCT in hospital-based data and multivariable analyses were done to determine independent sociodemographic predictors of sex bias. Annual time trends of MFR and MF-IRR during the 15-year study period were ascertained by time series regression analyses. FINDINGS: We included 11 375 children from PBCRs in the study. 26 891 children from hospital-based records were screened, and data from 22 893 (85·1%) were included (including 514 who underwent HSCT). Residence details were missing for 257 (1·1%) of 22 893 patients from hospital-based records. The crude MFR of children at diagnosis was in favour of boys: 2·00 (95% CI 1·92-2·09) in the Delhi PBCR and 1·44 (1·32-1·57) in Madras Metropolitan Tumour Registry. The MF-IRRs for cancer diagnosis were also skewed in favour of boys in both PBCRs (Delhi 1·69 [95% CI 1·61-1·76]; Madras Metropolitan Tumour Registry 1·37 [1·26-1·49]). The MFR for children seeking treatment from hospital-based records was 2·06 (95% CI 2·00-2·12) in favour of boys. In subgroup analyses, the proportion of boys seeking treatment was higher in northern India than southern India (p<0·0001); in private centres than in centres providing subsidised treatment (p<0·0001); in patients with haematological malignancies than those with solid malignancies (p<0·0001); in those residing 100 km or further from the hospital than those within 100 km of a hospital (p<0·0001); and those living in rural areas than those living in urban areas (p=0·0006). The MFR of 514 children who underwent HSCT was 2·81 (95% CI 2·32-3·43) in favour of boys. Time trend analysis showed that MFR did not show any significant annual change in either the overall cohort or in any of the individual centres for hospital-based data; however, the analysis did show a declining MF-IRR in the Delhi PBCR from 2005 to 2014 (p=0·031). INTERPRETATION: The sex ratio for childhood cancer in India has a bias towards boys at the level of diagnosis, which is more pronounced in northern India and in situations demanding greater financial commitment. Addressing societal sex bias and enhancing affordable health care for girls should be pursued simultaneously in India. FUNDING: None. TRANSLATION: For the Hindi translation of the abstract see Supplementary Materials section.

Multi-time point imaging in 99m Tc-pertechnetate scintigraphy of ectopic gastric mucosa in duplication cysts: value-added services.

OBJECTIVES: The aim of our study was to describe the scintigraphic patterns of 99m Tc-pertechnetate uptake in patients who were referred to the department of nuclear medicine for evaluating and diagnosing ectopic gastric mucosa in foregut and midgut duplication cysts. MATERIALS AND METHODS: This hospital-based, retrospective cum prospective research spans a period of 8 years from April 2014 to January 2022. Previous hospital medical records were analyzed and subsequently, a database was prepared which included the age, sex, clinical indication of a 99m Tc-pertechnetate scan, and the planar and SPECT-computed tomography (CT) imaging findings. Postoperative histopathological reports were available for 21 patients. Dynamic and planar static imaging was performed. We included SPECT-CT in suspected duplication cysts to increase the sensitivity and specificity which is a tradeoff for a small amount of additional radiation exposure. A total of 69 patients were subjected to a 99m Tc-pertechnetate scan for suspected foregut or midgut duplication cysts. All were subjected to dynamic planar and delayed static images up to 24 h or until focal uptake of radiotracer was noted which corroborated the anatomical findings, whichever was earlier. SPECT-CT was performed along with the planar study in 31 patients which confirmed the findings. Previously performed CT scans were used for anatomical correlation in the remaining ones. RESULTS: Duplication cysts were localized in a total of 28 patients (19 foregut duplication cysts and 12 small bowel duplications - 3 patients had dual duplication cysts, both foregut, and midgut). Forty-one patients had no scintigraphic evidence of ectopic gastric mucosa. Of these 69 patients, histopathological diagnosis was available for 21 patients (22 lesions). The report was concordant with the scan findings in 15 patients (16 lesions) and 6 patients showed discordance in histopathological diagnosis and scan findings. CONCLUSION: In conclusion, multi-time point imaging is the key to diagnosing ectopic gastric mucosa of various sizes and in various locations. An abnormal radiotracer uptake in dynamic sequences, even before the appearance of the stomach in the region of the small bowel is indicative of intestinal duplication, and delayed radiotracer visualization in the region of the thorax is characteristic of intrathoracic foregut duplication cyst.

Determinants and impact of diagnostic interval in bone sarcomas: A retrospective cohort study.

BACKGROUND: Diagnostic delays in cancers are frequent in developing countries due to poor health infrastructure. Existing literature from developed countries suggests that diagnostic interval in bone sarcomas is primarily dictated by tumour biology with no impact on survival. This study evaluates the social and biological determinants of the diagnostic interval in bone sarcomas in a resource-challenged setting and assesses its impact on treatment outcomes. METHODS: A retrospective single-institutional study was conducted on patients with high-grade bone sarcomas recorded in the sarcoma clinic database between 2003 and 2018. Baseline clinical characteristics and treatment outcomes were recorded. Logistic regression was performed to assess the impact of baseline clinical and social characteristics (distance from treating centre and rural vs. urban residence) on the diagnostic interval. Further, the impact of diagnostic interval on histologic response to neoadjuvant chemotherapy, amputation requirement in extremity sarcomas and survival was evaluated. RESULTS: A total of 1227 patients were included for analysis. The median diagnostic interval was 4 months (3-7 months). Age above 18 years, Ewing sarcoma (ES) diagnosis, absence of fever at presentation and tumour size above 7.5 cm were predictors of a longer diagnostic interval (>4 months). The length of the diagnostic interval did not impact amputation requirement or survival outcomes. However, the proportion of patients with good necrosis post-neoadjuvant chemotherapy was lower among patients with longer diagnostic intervals (25% vs. 34·16%; p-value = .04). CONCLUSION: Tumour characteristics rather than social factors determined the diagnostic interval. Diagnostic interval did not impact survival outcomes even in a resource-constrained setting.