No abstract

¹²³I-metaiodobenzylguanidine (¹²³I-MIBG) scintigraphy is widely used for the detection and staging of neuroblastoma. Risk-adapted treatment in patients with neuroblastic tumors is based on many clinical and genetic factors including histopathology. Purpose: non-invasive prediction of an unfavorable histological variant in patients with neuroblastic tumors using quantitative assessment of ¹²³I-MIBG uptake. This study was approved by the Independent Ethical Committee and the Academic Council of Dmitriy Rogachev National Medical Research Center of Pediatric Hematology, Oncology, Immunology Ministry of Healthcare of Russian Federation. 96 patients were included in this retrospective study. ¹²³I-MIBG-scintigraphy including whole body planner images and SPECT/CT were performed for all patients before any type of treatment. Semiquantitative and quantitative assessment of ¹²³I-MIBG uptake were calculated and analyzed. Out of 96 patients: 54 with neuroblastoma (NB), 28 with ganglioneuroblastoma (GNB) and 14 with ganglioneuroma (GN). The average values of TLCRR and SUVmax for NB were 5.67 and 7.5, for GNB – 2.58 and 3.1 and for GN – 1.48 and 1.85, respectively. A centile analysis was carried out for all groups. SPECT/CT in combination with modern software allows semiquantitative and quantitative assessment of ¹²³I-MIBG uptake in neuroblastic tumor. The TLCRR and SUVmax can be used separately as well as in combination with NSE for prediction of histological variant.

Early prediction of disease severity helps clinicians prevent adverse events and/or minimize losses in the event of a life-threatening complication. This provision fully applies to refractory septic shock, in which norepinephrine administration at a dose exceeding 0.5 μg/kg-1/min-1 is needed to maintain mean arterial pressure.The objective: to determine predictors of lethal outcomes in patients with refractory septic shock.Subjects and methods. A retrospective study included 79 patients with refractory septic shock aged from 42 to 74 years (59.7 ± 7.8), with severity of the condition as per SOFA varying from 8 to 16 scores. The predictive value of indicators was assessed using univariate and multivariate analyses.Results. In multivariate analysis, the only significant predictor of an unfavorable outcome was the SOFA score (adj. OR: 1.626 [95% CI: 1.313; 2.014], p < 0.001). Other putative indicators (age, oxygenation index, lactate and procalcitonin concentrations, and norepinephrine dose) had no predictive value.Conclusion: The SOFA score is an independent predictor of lethality in adult patients with refractory septic shock (аdj. OR: 1.26 [95% CI: 1.313; 2.014], p < 0.001). According to the results of the ROC analysis, along with SOFA, norepinephrine dose was also a significant predictor (AUC 0.989 [95% CI 0.934; 1.000], p < 0.001).

The aim of this study was the development and implementation of algorithms for computer analysis of medical images to quantify the asphericity of the area of radiopharmaceutical accumulation in a primary tumor, as well as to study the prognostic significance of asphericity as a predictor of an adverse outcome in patients with neuroblastoma. This retrospective study included 142 primary patients (69 boys, 73 girls, median age 22.5 months, range 1-198 months) with newly diagnosed neuroblastoma. All patients underwent full-body scintigraphy with 123I. Cox regression analysis, ROC analysis, and Kaplan-Meier analysis with a log-rank test in assessing event-free survival were used to assess the predictive value of asphericity. The median follow-up period was 18 months (range 7 to 78 months; 17 patients had disease progression / relapse, 10 patients died). The asphericity of the accumulation area in univariate Cox regression analysis is a significant pre- dictor of poor outcome (p = 0.035; hazard ratio (HR) = 1.106 [1.007; 1.215] for an increase of one unit). The optimal cut-off point for the asphericity parameter in the ROC analysis was chosen equal to 28.4%, predictor sensitivity: 77.8% (95% CI: 65%; 92%), specificity: 65.1% (95% CI: 52%; 75%). According to the results of the log-rank test, statistically significant differences were obtained: in patients with high asphericity (more than 28.4%) and low asphericity, the mean event-free survival rate was 42.9 (95% CI: 38.9%; 46.8%) and 56.6 (95% CI: 48.3%; 64.8%) months, respectively (p = 0.044). For the first time in the Russian Federation, a method for assessing the asphericity of neuroblastoma has been developed and implemented in software. In this study, the asphericity of the radiopharmaceutical accumulation area in the primary tumor, assessed prior to treatment initiation, identified children with a high and low risk of progression / relapse and death, with a sensitivity of 77.8% and a specificity of 65.1%.

Bilateral adrenal neuroblastoma (BANB) is a very rare entity. It is more common in young children and has certain clinical features. Patients with BANB have a more favorable prognosis than patients with unilateral adrenal neuroblastoma (NB). The article presents an analysis of 29 cases of BANB in children who underwent treatment at the Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology, and Immunology. This study is supported by the Independent Ethics Committee and approved by the Academic Council of the Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology, and Immunology. The analysis was performed for the period from April 2012 to December 2018. Bilateral adrenal involvement was diagnosed on the basis of analysis/assessment of the results of all relevant imaging and instrumental tests. Additionally, we performed a retrospective analysis of the findings of computed tomography, magnetic resonance imagining, and scintigraphy with 123I-metaiodobenzylguanidine (123I-MIBG). In view of 123I-MIBG scintigraphy sensitivity threshold, and in order to confirm the diagnosis, imaging findings were reviewed by an experienced radiologist. The patients received therapy in accordance with the risk-adapted NB-2004 protocol. Here, we analyzed the details of clinical presentation of the disease, age at the time of diagnosis, time from the disease onset to diagnosis, the presence or absence of life-threatening symptoms (LTS) and adrenal insufficiency, the aspects of patient management (including special aspects of surgical treatment), and treatment results. The median age at the time of diagnosis was 1.9 months. The patients were divided into 3 groups: children with localized BANB; children with true stage 4 disease; children with stage 4S disease. Most patients had metastatic NB, whereby stage 4S cases prevailed (55%, n = 16). Given the low sensitivity of MIBG scintigraphy for the detection of small tumours (less than 2 cm), one should use a comprehensive approach to the assessment of the results of imaging and instrumental tests in patients with BANB in order to confirm bilateral involvement. At diagnosis, about half of the patients with BANB (48%) had clinical symptoms associated with the presence of a retroperitoneal tumour mass and massive hepatomegaly because of liver metastases. Thirty-four percent of patients developed LTS. One of the typical features of BANB was the frequent development of adrenal insufficiency (in 41% of cases) caused by bilateral adrenal involvement, and this should be taken into account when performing diagnostic investigations and providing treatment to patients. Since bilateral radical resection is associated with the inevitable development of adrenal insufficiency, surgical treatment should be determined on an individual basis. Bilateral adrenalectomy is not generally recommended and can be undertaken after an interdisciplinary discussion in case of unfavourable course of the disease (e.g. in case of recurrence). Even if patients with BANB develop certain complications or events (progression) their prognosis is still good. The three- and five-year event-free survival rates in patients with BANB were 86 ± 6.4%. The three- and five-year overall survival rates were 100%. The analysis of patients with BANB showed that they have a more favourable course of the disease, and this may serve as grounds for assigning such patients into a special group. In view of the degree of tumour extension, the specifics of staging, and frequent development of adrenal insufficiency, standard check-ups should be complemented by endocrine monitoring, and surgical approaches should be chosen on an individual basis.