Image-defined Risk Factors Research Articles

Технические аспекты лапароскопического удаления нейрогенных опухолей абдоминальной локализации у детей

Progress in a modern pediatric oncological surgery, including neuroblastoma surgery, is characterized by increased role of the minimally-invasive approach. Authors represent standardized technical aspects of abdominal neuroblastoma laparoscopic resection. The purpose of this research was to systematize the technical aspects of laparoscopic resection for pediatric abdominal neuroblastoma, also in cases with Image-Defined Risk Factors (IDRF). Methods used: pre- and intraoperative data of 181 patients (85 (47%) males/96 (53%) females) of the median age of 14 [5-51] (1-209) months old with abdominal neuroblastoma who had received laparoscopic tumor resection at the National Scientific and Practical Center for Pediatric Hematology, Oncology and Immunology named after Dmitry Rogachev (Moscow, Russia) in Jan. 2018-Jan. 2024 were analyzed. Technical features of laparoscopic surgery procedure, also in cases with IDRF have been systematized as follows: patient position on the operating table, trocars placement, visualization of surgical field, dissection plan. Results: maximal tumor length was 3,6 [2,6-5] (0,7-12,6) cm, maximal tumor volume was 16,8 [4-40] (0,5-557) ml. IDRF were identified in 53 (29%) cases, wherein two and more IDRFs were in 19 (10%) patients. The surgical intervention duration was 100 [70-140] (35-725) minutes, intraoperative blood loss was 5 [5-18] (5-3600) ml, intraoperative transfusion was 0 [0-0] (0-890) ml. Intraoperative complications were observed in 6 (3%) patients, conversion was required in 7 (4%) cases. Complete tumor resection (98% to 100%) was achieved in 163 (90%) cases. Conclusion: the accumulated experience of minimally invasive surgery for neuroblastoma indicates the demand for reviewing of the current indications for these surgical procedures as well as a precise investigation of the effect of each IDRF on the difficulty and risk of surgery. In this Article Authors have described standardized technical features of abdominal neuroblastoma laparoscopic resection. These technical features are used in prospective clinical trial whilst the purpose of this research was to design the difficulty scoring system for such surgery (ClinicalTrials.gov ID NCT06296732).

Quantitative MRI in Childhood Neuroblastoma: Beyond the Assessment of Image-defined Risk Factors.

Neuroblastoma commonly occurs in children. MRI is a radiation-free imaging modality and has played an important role in identifying image-defined risk factors of neuroblastoma, providing necessary guidance for surgical resection and treatment response evaluation. However, image-defined risk factors are limited to providing structural information about neuroblastoma. With the evolution of MRI technologies, quantitative MRI can not only help assess image-defined risk factors but can also quantitatively reflect the biologic features of neuroblastoma in a noninvasive manner. Therefore, compared with anatomic imaging, these emerging quantitative MRI technologies are expected to provide more imaging biomarkers for the management of neuroblastoma. This review article discusses the current applications of quantitative MRI technologies in evaluating childhood neuroblastoma. Keywords: Pediatrics, MR-Functional Imaging, Children, MRI, Neuroblastoma, Quantitative Imaging © RSNA, 2024.

Inter-observer variability in assessing image-defined risk factors: implications for risk stratification in locoregional abdominopelvic neuroblastoma.

Risk stratification for locoregional neuroblastoma partially relies on image-defined risk factors (IDRFs). This study aimed to evaluate how inter-observer variability in assessing IDRFs impacts risk stratification in locoregional abdominopelvic neuroblastoma. A retrospective analysis was conducted on 123 patients who underwent upfront contrast-enhanced CT scans. Two radiologists independently assessed the presence of IDRFs. Patients were staged as either L1 (IDRF-negative) or L2 (IDRF-positive) according to the International Neuroblastoma Risk Group Staging System. Based on the radiologists' evaluations, 97 cases with sufficient clinical data were classified into risk groups using the revised Children's Oncology Group neuroblastoma risk classifier. The kappa values and 95% confidence intervals (CIs) were calculated to assess inter-radiologist agreement on IDRF evaluation and risk stratification. There was low agreement between radiologists in assessing L1/L2 status with a kappa value of 0.28 (95% CI: 0.14-0.42). However, agreement for evaluating the number of IDRFs was good, with an intraclass correlation coefficient of 0.73 (95% CI: 0.64-0.80). Based on the first radiologist's evaluation, 13 patients were classified as low-risk, 52 as intermediate-risk, and 32 as high-risk. Based on the second radiologist's evaluation, 37 patients were classified as low-risk, 37 as intermediate-risk, and 23 as high-risk. The kappa value for risk stratification between the two radiologists was 0.47 (95% CI: 0.33-0.62). Inter-observer variability in assessing IDRF presence may affect risk stratification in locoregional abdominopelvic neuroblastoma.

Tracking changes in image-defined risk factors during neoadjuvant chemotherapy and their predictive value for surgical outcomes based on the International Neuroblastoma Surgical Report Form.

The capacity of presurgical image-defined risk factors (IDRFs) to predict secondary surgical outcomes in patients with neuroblastoma is controversial. The International Neuroblastoma Surgical Report Form (INSRF) was employed to retrospectively collect the clinical data of 53 patients diagnosed with neuroblastoma at our hospital from April 2014 to April 2020. IDRFs were identified at the time of diagnosis and reassessed during the course of neoadjuvant chemotherapy. Various statistical tests were used to evaluate the correlation between IDRFs and secondary surgical outcomes. A total of 195 IDRFs were identified. Notably, by two courses of neoadjuvant chemotherapy, the number of "two body compartments," "intraspinal tumor extension," and "trachea-compressing" IDRFs decreased significantly (p=.001). The primary tumor volumes and the number of IDRFs decreased significantly by four courses of neoadjuvant chemotherapy, especially in "intraspinal tumor extension" IDRFs (p=.034). The median number of IDRF per patient was four (interquartile range [IQR]: 1-5) at diagnosis, which diminished to one (IQR: 1-3) subsequent to neoadjuvant chemotherapy. The presence of preoperative IDRFs was not associated with surgical complications (p=.286) or the extent of surgery (p=.188). However, the number of preoperative IDRFs linked to the extent of surgery (p=.002), not to operative complications (p=.669). Specifically, presurgery "renal vessel contact" IDRFs were predictive of surgical complications, while presurgery "infiltration of vital structures" IDRFs were associated with the extent of surgery. The number of IDRFs decreased significantly by four courses of neoadjuvant chemotherapy. The number and type of presurgery IDRFs may predict secondary surgical outcomes, surpassing the mere consideration of their presence or absence.

A subset of image-defined risk factors predict completeness of resection in children with high-risk neuroblastoma: An international multicenter study.

Image-defined risk factors (IDRFs) were promulgated for predicting the feasibility and safety of complete primary tumor resection in children with neuroblastoma (NB). There is limited understanding of the impact of individual IDRFs on resectability of the primary tumor or patient outcomes. A multicenter database of patients with high-risk NB was interrogated to answer this question. Patients with high-risk NB (age <20years) were eligible if cross-sectional imaging was performed at least twice prior to resection. IDRFs and primary tumor measurements were recorded for each imaging study. Extent of resection was determined from operative reports. There were 211 of 229 patients with IDRFs at diagnosis, and 171 patients with IDRFs present pre-surgery. A ≥90% resection was significantly more likely in the absence of tumor invading or encasing the porta hepatis, hepatoduodenal ligament, superior mesenteric artery (SMA), renal pedicles, abdominal aorta/inferior vena cava (IVC), iliac vessels, and/or diaphragm at diagnosis or an overlapping subset of IDRFs (except diaphragm) at pre-surgery. There were no significant differences in event-free survival (EFS) and overall survival (OS) when patients were stratified by the presence versus absence of any IDRF either at diagnosis or pre-surgery. Two distinct but overlapping subsets of IDRFs present either at diagnosis or after induction chemotherapy significantly influence the probability of a complete resection in children with high-risk NB. The presence of IDRFs was not associated with significant differences in OS or EFS in this cohort.

Evaluation of Image-Defined Risk Factors Efficacy on Neuroblastoma Surgical Resectability

Abstract Background Neuroblastoma (NB) is one of the most prevalent extracranial solid cancers in children that commonly occur in the adrenal glands (49%). Thus, monitoring the diagnosis, staging, treatment planning, response evaluation, and updating and discovering new International Neuroblastoma Risk Groups (INRGs) is an important method to facilitate the treatment process. Patients and Methods The clinical, radiological, pathological, and surgical data of 30 neuroblastoma patients were analyzed, preoperative image-defined risk factors (IDRFs) were present in 23 cases with the most two frequent IDRFs were encasement of aorta then invasion of the renal pedicles. Results The statistical analysis data represented 30 cases showed that there was significant correlation with preoperative IDRFs and the type of surgery as well as significant correlation between IDRF and primary tumor location. The results affirm that IDRFs remain a useful prognostic tool for neuroblastoma patients both for short and long-term outcomes. Conclusion Hence, the efficiency of using the image defined risk factor was predictable of surgical resectability and complication.

Clinical Profile and Outcomes of Childhood Wilms Tumors Treated in a Tertiary Cancer Center from North India

In India and other lower-middle-income countries, the progress in the management of Wilms tumor (WT) has lagged behind when compared to the developed countries. In the current study, we highlight the outcomes of 61 children with WT treated at an oncological center in northern India. Our study found that the patient demographics, including age and gender distribution, closely paralleled other Indian studies. Notably, 24% of patients had distant metastasis at diagnosis. An upfront biopsy was performed in majority of the patient with a remarkable concordance rate of over 90%, with no significant complications associated with the procedure. Our surgical approach, guided by image-defined risk factors, resulted in 84% of patients undergoing delayed surgery after chemotherapy. Notably, the incidence of intraoperative complications, including tumor spill, was minimal. Treatment abandonment and toxic death due to sepsis emerged as significant challenges, affecting 22% and 7% of our patient cohort, respectively. The 3-year event-free survival (EFS) and overall survival were 70% and 84%, respectively. The age group of 6 to 8 years and stage V disease emerged as adverse factors influencing EFS. In summary, our study highlights the multifaceted nature of WT management in resource-constrained settings with survival rates promising but still lagging behind high-income countries. Addressing challenges such as treatment abandonment and reducing toxic death is imperative to enhance outcomes of WT in India.

Evaluation of Image-Defined Risk Factor (IDRF) Assessment in Patients With Intermediate-risk Neuroblastoma: A Report From the Children's Oncology Group Study ANBL0531

BackgroundThe International Neuroblastoma Risk Group (INRG) classifier utilizes a staging system based on pretreatment imaging criteria in which image-defined risk factors (IDRFs) are used to evaluate the extent of locoregional disease. Children's Oncology Group (COG) study ANBL0531 prospectively examined institutional determination of IDRF status and compared that to a standardized central review. MethodsBetween 9/2009-6/2011, patients with intermediate-risk neuroblastoma were enrolled on ANBL0531 and had IDRF assessment at treating institutions. Paired COG pediatric surgeons and radiologists performed blinded central review of diagnostic imaging for the presence or absence of IDRFs. Second blinded review was performed in cases of discordance. Comparison of local and central review was performed using the Kappa coefficient to determine concordance in IDRF assessment. Results211 patients enrolled in ANBL0531 underwent IDRF assessment; 3 patients were excluded due to poor image quality. Central reviewer pairs agreed on the presence or absence of any IDRF in 170/208 (81.7%; κ = 0.48) cases. Thirteen (6.3%) cases could not be adjudicated after second blinded review. Radiologists were more likely to identify IRDFs as present than surgeons (p < 0.001). Local and central reviewers agreed on the presence or absence of any IDRF in only108/208 (51.9%; κ = 0.06) cases. ConclusionsAmong experienced pediatric surgeons and radiologists participating in central review, concordance was moderate, with agreement in 81.7% of cases. On comparison of local and central assessment of IDRFs, concordance was poor. These data indicate that greater standardization, education, technology, and training are needed to improve the assessment of IDRFs in children with neuroblastoma. Level of EvidenceTreatment Study, Level III.

Robot-assisted laparoscopic adrenalectomy: Extended application in children

BackgroundMinimally invasive surgery for paediatric adrenal tumours has evolved, but robot-assisted laparoscopic adrenalectomy (RALA) in children remains poorly studied. The current prospective study aims to demonstrate the safety and efficacy of RALA in treating children with adrenal tumours. MethodsA prospective institutional analysis of children presenting with neuroblastic and endocrine tumours treated with RALA was undertaken over a six year-period. For each child, clinical parameters were collected relating to diagnosis, surgery and outcomes. ResultsA total 50 RALA were performed; 23 for unilateral neuroblastic tumours (87 % neuroblastomas) and 27 for endocrine tumours.Eight neuroblastic tumours (35 %) had image-defined risk factors (all due to tumour invading the renal pedicle). Median length of stay was two days. Resection margins were macroscopically clear in all cases. After median follow-up of 2.9 years (1.6–3.9), two children are under treatment for metastatic relapse (high-risk disease) and three died due to refractory disease.Sixteen children had endocrine tumours: pheochromocytoma (n = 13), or bilateral nodular adrenocortical hyperplasia with Cushing's syndrome (n = 14). One child required non-emergent conversion, and one complication occurred (grade IIIb) after median follow-up of 3.3 years (1.0–5.7). ConclusionsThe current study is the largest reported experience in the literature and confirms the safety and effectiveness of RALA in carefully selected children with adrenal tumours. Through an iterative process and in the setting of a dedicated paediatric robotic surgical team indications have been clarified and extended. The current study confirms RALA has particularly utility in patients with severe disease (IDRF + metastatic neuroblastomas) or genetic predisposition syndromes.

A non-inferiority study of MRI versus CT for staging and image-defined risk factor assessment in the preoperative work-up of abdominopelvic neuroblastoma

BackgroundNeuroblastoma accounts for 15 % of cancer deaths in children. Complete surgical resection is associated with a higher overall survival rate but also a higher morbidity rate. An international group of experts has defined a nomenclature of image-defined risk factors (IDRFs) for the determination of operability and the anticipation of reasonably foreseeable complications of surgery. However, there is no consensus on the optimal imaging modality (CT or MRI) for the assessment of IDRFs. The objective of the present study was to determine the non-inferiority of MRI vs. CT in the preoperative assessment of abdominopelvic neuroblastoma. The secondary objective was to assess the contribution of gadolinium contrast enhancement. MethodsAll children diagnosed with abdominopelvic neuroblastoma and whose preoperative work-up included a contrast-enhanced CT or MRI scan of the abdomen and pelvis between January 2014 and January 2023 were included. To evaluate the IDRFs, all the images were reviewed in three steps: (i) non-contrast MRI scans, (ii) both non-contrast and contrast-enhanced MRI scans, and (iii) contrast-enhanced CT scans. ResultsTwenty-five patients were found to be eligible, and fifteen were included. The mean time interval between MRI and preoperative CT was 23 days. In all patients, the identified IDRFs were similar for all three imaging modalities. Fourteen patients underwent full resection of the tumour. The surgical reports were fully consistent with the IDRFs described on CT and/or MRI. ConclusionA high-resolution three-dimensional T2 MRI sequence agreed fully with contrast-enhanced CT for the detection of IDRFs. Contrast-enhanced MRI did not add value. However, surgeons will need time to adapt to this MRI-based approach and learn how to interpret the results with confidence.

Impact of an objective scoring system on initiation of therapy in patients with International Neuroblastoma Risk Group Staging System (INRGSS) stage MS neuroblastoma: A report from the Children’s Oncology Group.

10003 Background: A subset of children with INRGSS Stage MS neuroblastoma (NBL) may be observed without treatment, while others have rapidly evolving symptoms that can be life-threatening. ANBL1232 adapted a previously developed semi-quantitative objective scoring system (OSS) to assign a numeric value to symptoms and laboratory abnormalities. The goal was to standardize monitoring, therapy initiation, and treatment duration in this cohort. Methods: Patients with newly diagnosed Stage MS NBL were eligible for this prospective trial. An OSS score (OSSS) was assigned at enrollment; the total number was based on scoring in 5 systems: gastrointestinal (GI), respiratory, circulatory, renal, and hepatic. Within the 5 systems, individual clinical and laboratory parameters were scored as not present = 0, mild/moderate = 1 [Grade 2 CTCAEv4.0 adverse event (AE)] or severe = 2 (Grade ≥3 AE). The OSSS was the sum of highest score within each organ system (maximum score: 2 per system, 10 total). Asymptomatic MS patients with an OSSS &lt; 2 who were either &lt; 3 months (mo) of age without hepatomegaly or 3-18 mo of age with favorable biology tumors were eligible for observation without initial treatment. Observed patients underwent monthly physical examinations and laboratory assessments for the first 6 mo following diagnosis. Tumor imaging was performed every 3 mo for 1 year, then every 6-12 mo through 36 mo. An OSSS ≥2 or a protocol-defined increase in primary tumor size prompted initiation of therapy. Results: From July 2014 to February 2021, 89 eligible and evaluable patients with newly diagnosed stage MS NBL enrolled. Among these, 18 (20.2%; 5 male and 13 female) were eligible for observation. Observed patients were older at diagnosis (median age: 2.87 vs. 1.81 mo, p = 0.23) and more likely to have primary tumors without image-defined risk factors (62.5% vs. 30.0%, p = 0.0166) than those assigned to therapy up front. Nearly all observed patients (17/18) had abdominal/adrenal primaries. The initial OSSS was 0 in all observed patients. Median reported observation time was 36 mo (range: 1-36 mo); 13 patients completed all required monthly assessments for the first 6 mo. No patients in the observation cohort required initiation of therapy for an increase in OSSS. One patient with OSSS = 1 at mo 3 had complete GI symptom resolution by mo 5. Conclusions: An OSSS of 0 at diagnosis can aid in identifying a favorable group of patients with stage MS NBL who can be safely observed. No patients in the observation cohort developed evidence of organ dysfunction or OSSS &gt; 1 despite frequent physical examination and comprehensive laboratory testing, suggesting that follow up may be safely liberalized in this population over time. Clinical and laboratory criteria implemented at diagnosis could be used to identify patients requiring prompt treatment. Clinical trial information: NCT02176967 .

Staging and Restaging Pediatric Abdominal and Pelvic Tumors: A Practical Guide.

The most common abdominal malignancies diagnosed in the pediatric population include neuroblastoma, Wilms tumor, hepatoblastoma, lymphoma, germ cell tumor, and rhabdomyosarcoma. There are distinctive imaging findings and patterns of spread for each of these tumors that radiologists must know for diagnosis and staging and for monitoring the patient's response to treatment. The multidisciplinary treatment group that includes oncologists, surgeons, and radiation oncologists relies heavily on imaging evaluation to identify the best treatment course and prognostication of imaging findings, such as the image-defined risk factors for neuroblastomas, the PRETreatment EXtent of Disease staging system for hepatoblastoma, and the Ann Arbor staging system for lymphomas. It is imperative for radiologists to be able to correctly indicate the best imaging methods for diagnosis, staging, and restaging of each of these most prevalent tumors to avoid inconclusive or unnecessary examinations. The authors review in a practical manner the most updated key points in diagnosing and staging disease and assessing response to treatment of the most common pediatric abdominal tumors. ©RSNA, 2024 Supplemental material is available for this article.

Enhancing surgical planning for abdominal tumors in children through advanced 3D visualization techniques: a systematic review of future prospects.

Preoperative three-dimensional (3D) reconstruction using sectional imaging is increasingly used in challenging pediatric cases to aid in surgical planning. Many case series have described various teams' experiences, discussing feasibility and realism, while emphasizing the technological potential for children. Nonetheless, general knowledge on this topic remains limited compared to the broader research landscape. The aim of this review was to explore the current devices and new opportunities provided by preoperative Computed Tomography (CT) scans or Magnetic Resonance Imaging (MRI). A systematic review was conducted to screen pediatric cases of abdominal and pelvic tumors with preoperative 3D reconstruction published between 2000 and 2023. Surgical planning was facilitated through virtual reconstruction or 3D printing. Virtual reconstruction of complex tumors enables precise delineation of solid masses, formulation of dissection plans, and suggests dedicated vessel ligation, optimizing tissue preservation. Vascular mapping is particularly relevant for liver surgery, large neuroblastoma with imaging-defined risk factors (IDRFs), and tumors encasing major vessels, such as complex median retroperitoneal malignant masses. 3D printing can facilitate specific tissue preservation, now accessible with minimally invasive procedures like partial nephrectomy. The latest advancements enable neural plexus reconstruction to guide surgical nerve sparing, for example, hypogastric nerve modelling, typically adjacent to large pelvic tumors. New insights will soon incorporate nerve plexus images into anatomical segmentation reconstructions, facilitated by non-irradiating imaging modalities like MRI. Although not yet published in pediatric surgical procedures, the next anticipated advancement is augmented reality, enhancing real-time intraoperative guidance: the surgeon will use a robotic console overlaying functional and anatomical data onto a magnified surgical field, enhancing robotic precision in confined spaces.

Image-defined risk factors associated with MYCN oncogene amplification in neuroblastoma and their association with overall survival.

The MYCN oncogene is a critical factor in the development and progression of neuroblastoma, and image-defined risk factors (IDRFs) are radiological findings used for the preoperative staging of neuroblastoma. This study aimed to investigate the specific categories of IDRFs associated with MYCN amplification in neuroblastoma and their association with overall survival. A retrospective analysis was conducted on a cohort of 280 pediatric patients diagnosed with neuroblastoma, utilizing a combination of clinical and radiological data. MYCN amplification status was ascertained through molecular testing, and the assessment of IDRFs was conducted using either contrast-enhanced computed tomography or magnetic resonance imaging. The specific categories of IDRFs associated with MYCN amplification and their association with overall survival were analyzed. MYCN amplification was identified in 19.6% (55/280) of patients, with the majority of primary lesions located in the abdomen (53/55, 96.4%). Lesions accompanied by MYCN amplification exhibited significantly larger tumor volume and a greater number of IDRFs compared with those without MYCN amplification (P < 0.001). Both univariate and multivariate analyses revealed that coeliac axis/superior mesenteric artery encasement and infiltration of adjacent organs/structures were independently associated with MYCN amplification in abdominal neuroblastoma (P < 0.05). Patients presenting with more than four IDRFs experienced a worse prognosis (P = 0.017), and infiltration of adjacent organs/structures independently correlated with overall survival in abdominal neuroblastoma (P = 0.009). The IDRFs are closely correlated with the MYCN amplification status and overall survival in neuroblastoma.

Successful endoscopic approach for peripheral neuroblastic tumors in children.

Recently, reports of endoscopic approaches for neuroblastoma, ganglioneuroblastoma, and ganglioneuroma (peripheral neuroblastic tumor; PNTs) have been increasing. This study aimed to clarify the indications for endoscopic surgery for PNTs. Pediatric patients who underwent endoscopic surgery for PNTs at our institution were included in this study. Image-defined risk factors (IDRFs) were analyzed using preoperative computed tomography (CT). Twenty-four patients underwent endoscopic surgery for PNTs. The diagnoses included neuroblastoma (n = 11), ganglioneuroma (n = 10), and ganglioneuroblastoma (n = 3). Regarding the tumor site, there were 18 cases of adrenal tumors, five cases of mediastinal tumors, and one case of retroperitoneal tumors. Image-defined risk factors were positive in eight cases (contacted with a renal vessel, n = 6; compression of principal bronchi, n = 2). Complete resection was accomplished in 21 cases (14 of 16 IDRF-negative cases and seven of eight IDRF-positive cases). All patients survived without recurrence during the follow-up period. The CT findings of contact with renal vessels and compression of principal bronchi do not seem to be indicators of incomplete resection. An endoscopic approach to PNTs in pediatric patients is feasible with a good prognosis if patients are selected strictly.

Prediction of Surgical Outcomes by Image Defined Risk Factors in Children with Wilms' Tumor

Prediction of Surgical Outcomes by Image Defined Risk Factors in Children with Wilms' Tumor

MR Imaging of Pediatric Neuroblastoma: Is Gadolinium Enhancement Necessary for Evaluation of Image-Defined-Risk Factors?

Background: Pre-treatment stratification and outcomes of neuroblastoma patients often depend on the assessment of image-defined risk factors (IDRFs) on MR Imaging, usually using Gadolinium-contrast materials which are cautioned in pediatrics. We aimed to address whether gadolinium contrast-enhanced sequences are necessary to identify the presence/absence of IDRFs. Methods: Patients with neuroblastoma with MR imaging were retrospectively identified from 2005 to 2021. Ninety confirmed IDRFs were evaluated in 23 patients. Corresponding MR studies were anonymized, randomized, and independently evaluated by 3 fellowship-trained pediatric radiologists. Each radiologist assessed the studies twice. At the first reading, all enhanced sequences were omitted, while in the second reading, the full study with enhanced sequences were included. Consensus reading was obtained among readers. Inter- and intra-rater agreements using Kappa statistics (κ) as well as the sensitivity, specificity, positive predictive value (PPV), negative predictive value (NPV), and accuracy of non-enhanced MR in assessing IDRFs with respect to enhanced MR were calculated. Results: There were substantial (ĸ: 0.64-0.73) intra-reader agreements, and moderate to substantial (ĸ: 0.57-0.62) inter-reader agreements among radiologists in identifying IDRFs using non-enhanced MR. Non-enhanced MR had a sensitivity of 87.8% (95% CI [79-94]), specificity of 93% (89-96), PPV of 82.3 (73-89), NPV of 95.4 (92-98), and accuracy of 91.6 (88-94) in identifying IDRFs. However, 5/23 patients (21.7%) had a change in staging with the inclusion of contrast sequences. Conclusion: Although contrast sequences have a role in IDRF assessment, the majority can be adequately assessed on MR without gadolinium-contrast enhancement. Validation in a larger cohort is an important next step.

Perioperative complication incidence and risk factors for retroperitoneal neuroblastoma in children: analysis of 571 patients

BackgroundSurgery plays an important role in the treatment of neuroblastoma. Perioperative complications may impact the course of neuroblastoma treatment. To date, comprehensive analyses of complications and risk factors have been lacking.MethodsPatients with retroperitoneal neuroblastoma undergoing tumor resection were retrospectively analyzed between 2014 and 2021. The data collected included clinical characteristics, operative details, operative complications and postoperative outcomes. Risk factors for perioperative complications of retroperitoneal neuroblastoma were analyzed.ResultsA total of 571 patients were enrolled in this study. Perioperative complications were observed in 255 (44.7%) patients. Lymphatic leakage (28.4%), diarrhea (13.5%), and injury (vascular, nerve and organ; 7.5%) were the most frequent complications. There were three operation-related deaths (0.53%): massive hemorrhage (n = 1), biliary tract perforation (n = 1) and intestinal necrosis (n = 1). The presence of image-defined risk factors (IDRFs) [odds ratio (OR) = 2.09, P < 0.01], high stage of the International Neuroblastoma Risk Group staging system (INRGSS) (OR = 0.454, P = 0.04), retroperitoneal lymph node metastasis (OR = 2.433, P = 0.026), superior mesenteric artery encasement (OR = 3.346, P = 0.003), and inferior mesenteric artery encasement (OR = 2.218, P = 0.019) were identified as independent risk factors for perioperative complications.ConclusionsDespite the high incidence of perioperative complications, the associated mortality rate was quite low. Perioperative complications of retroperitoneal neuroblastoma were associated with IDRFs, INRGSS, retroperitoneal lymph node metastasis and vascular encasement. Patients with high-risk factors should receive more serious attention during surgery but should not discourage the determination to pursue total resection of neuroblastoma.8e95DYrdivwNpNwDkEtfUxVideo (MP4 94289 KB)

Thoracoscopy for Pediatric Thoracic Neurogenic Tumors-A European Multi-Center Study.

To assess the efficacy of thoracoscopy and the outcome for children with thoracic neurogenic tumors. We performed a retrospective review of 15 European centers between 2000 and 2020 with patients who underwent thoracoscopy for a neurogenic mediastinal tumor. We assessed preoperative data, complications, and outcomes. Results were expressed with the median and range values. We identified 119 patients with a median age of 4 years old (3 months-17 years). The diameter was 5.7 cm (1.1-15). INRG stage was L1 n = 46, L2 n = 56, MS n = 5, M n = 12. Of 69 patients with image-defined risk factors (IDRF), 29 had only (T9-T12) locations. Twenty-three out of 34 patients with preoperative chemotherapy had an 18 mm (7-24) decrease in diameter. Seven out of 31 patients lost their IDRF after chemotherapy. Fourteen had a conversion to thoracotomy. The length of the hospital stay was 4 days (0-46). The main complications included chylothorax (n = 7) and pneumothorax (n = 5). Long-term complications included Horner's syndrome (n = 5), back pain, and scoliosis (n = 5). Pathology was 53 neuroblastomas, 36 ganglioneuromas, and 30 ganglioneuroblastomas. Fourteen had a postoperative residue. With a median follow-up of 21 months (4-195), 9 patients had a recurrence, and 5 died of disease. Relapses were associated with tumor biology, histology, and the need for chemotherapy (p = 0.034, <0.001, and 0.015, respectively). Residues were associated with preoperative IDRF (excluding T9-T12 only) and the need for preoperative chemotherapy (p = 0.04 and 0.020). Our results show that thoracoscopy is safe, with good outcomes for thoracic neurogenic tumors in selected cases. Surgical outcomes are related to the IDRFs, whereas oncologic outcomes are related to tumor histology and biology.

Minimally invasive approach of paediatric neuroblastoma with thoracic vascular encasement.

Neuroblastoma (NB) is the most frequent paediatric extracranial solid tumour. The surgical management of these tumours in newborns changed recently, performing resections in cases with tumour size increase after birth. Minimally invasive procedures were mostly reported in cases without pre-operative image-defined risk factors (IDRFs), defined by vascular and organ involvement. Thoracoscopic resection represents a minority of the overall surgical procedures for neuroblastic tumour management, as the posterior mediastinum is one of the least frequent locations of NB. A thoracoscopic resection was performed on a 22-month-old child with a NB encasing the aorta and a 6-month-old child with the encasement of the left subclavian and vertebral artery. A step-by-step minimally invasive procedure was described, highlighting anatomical landmarks and dissection techniques. The described technique was performed in 130 min. Thoracoscopic resection provided a macroscopic resection without surgical complications and patient was discharged on the 3 rd post-operative day. The study shows a feasible and safe thoracoscopic approach for paediatric thoracic NB with IDRFs.