Surgical management, staging, and outcomes of Wilms tumours with intravascular extension: Results of the IMPORT study

PURPOSE: To review surgical management, tumour stage and clinical outcomes in children with intravascular extension of Wilms tumour (WT) registered in a national clinical study (2012-19). METHODS: WTs with presence/suspicion of tumour thrombus in the renal vein (RV) or beyond on radiology, surgery or pathology case report forms were identified. Only cases where thrombus was confirmed by surgeon and/or reference pathologist were included. Surgical management, disease stage, overall (OS) and event free survival (EFS) were investigated. RESULTS: 69/583 (11.8%) patients met the inclusion criteria. Forty-six (67%) had abdominal stage III due to thrombus-related reasons: 11 had macroscopically incomplete resection, including 8 cases where cavotomy was not performed; 20 had piecemeal complete resection of thrombus; 15 had microscopically positive resection margins at the RV. 66% of tumour thrombi contained viable tumour. There were eight relapses and five deaths. EFS, but not OS, was significantly associated with completeness of surgical resection (P<0.05). OS and EFS were also significantly associated with histological risk group (P<0.05) but not with viability of tumour thrombus (P=0.19; P=0.59). CONCLUSIONS: WTs with intravascular extension have a high risk of local stage III due to thrombus-related reasons. Controlled complete removal of the thrombus should be the aim of surgery. LEVEL OF EVIDENCE: Level II

Characteristics and outcome of pediatric renal cell carcinoma patients registered in the International Society of Pediatric Oncology (SIOP) 93‐01, 2001 and UK‐IMPORT database: A report of the SIOP‐Renal Tumor Study Group

In children, renal cell carcinoma (RCC) is rare. This study is the first report of pediatric patients with RCC registered by the International Society of Pediatric Oncology‐Renal Tumor Study Group (SIOP‐RTSG). Pediatric patients with histologically confirmed RCC, registered in SIOP 93‐01, 2001 and UK‐IMPORT databases, were included. Event‐free survival (EFS) and overall survival (OS) were analyzed using the Kaplan‐Meier method. Between 1993 and 2019, 122 pediatric patients with RCC were registered. Available detailed data (n = 111) revealed 56 localized, 30 regionally advanced, 25 metastatic and no bilateral cases. Histological classification according to World Health Organization 2004, including immunohistochemical and molecular testing for transcription factor E3 (TFE3) and/or EB (TFEB) translocation, was available for 65/122 patients. In this group, the most common histological subtypes were translocation type RCC (MiT‐RCC) (36/64, 56.3%), papillary type (19/64, 29.7%) and clear cell type (4/64, 6.3%). One histological subtype was not reported. In the remaining 57 patients, translocation testing could not be performed, or TFE‐cytogenetics and/or immunohistochemistry results were missing. In this group, the most common RCC histological subtypes were papillary type (21/47, 44.7%) and clear cell type (11/47, 23.4%). Ten histological subtypes were not reported. Estimated 5‐year (5y) EFS and 5y OS of the total group was 70.5% (95% CI = 61.7%‐80.6%) and 84.5% (95% CI = 77.5%‐92.2%), respectively. Estimated 5y OS for localized, regionally advanced, and metastatic disease was 96.8%, 92.3%, and 45.6%, respectively. In conclusion, the registered pediatric patients with RCC showed a reasonable outcome. Survival was substantially lower for patients with metastatic disease. This descriptive study stresses the importance of full, prospective registration including TFE‐testing

Вплив різних типів портокавального шунтування на стан імунної системи у дітей з допечінковою формою портальної гіпертензії

Objective. The paper is focused on the features and grade of changes in the immune system under the extrahepatic portal hypertension and after splenorenal and mesocaval shunting operations.Materials and methods. In the study were included 100 children, in whom the cell, humoral and unspecific parts of immune system were evaluated. The leading pathological factors were defined.Results. It was established that there is significant tensity in the humoral part (IgМ, IgA) and phagocytosis, secondary cell immune deficiency with decreasing of general number of lymphocytes and subpopulations of lymphocytes (СD3+, СD4+, СD8+). Immunological parameters after splenorenal shunting were statisticily significant approached to the age norms (p&lt;0,05) comparing to those after mesocaval shunting. However, the operative treatment essentially doesn’t improve the condition of the immune system.Conclusion. The parameters of immunogramme should be considered while monitoring such category of patients, selection of the immunocorrective and detoxication therapy for prevention of postoperative complications and for the further management under extrahepatic portal hypertension.Цель: изучить характер и степень нарушений со стороны иммунной системы у детей с допеченочной формой портальной гипертензии (ДфПГ) после различных типов портокавального шунтирования.Материалы и методы. В исследование было включено 100 детей, у которых оценивали клеточное и гуморальное звено иммунной системы, факторы неспецифической защиты, определяли основные иммунопатологические синдромы.Результаты. Вследствие анализа полученных данных было установлено, что у большинства детей с ДфПГ имеет место напряженность гуморального звена иммунитета (IgМ, IgA) и фагоцитоза, вторичный клеточный иммунодефицит со снижением общего количества лейкоцитов, лимфоцитов и субпопуляций лимфоцитов (CD3+, СD4+, СD8+). Иммунологические показатели больных после сплено(супра)ренального шунтирования статистически достоверно были более приближенными к возрастным нормам (p&lt;0,05) по сравнению с показателями больных после мезентерикокавального шунтирования. Однако оперативное лечение существенно не улучшает состояния иммунной системы у детей с ДфПГ.Выводы. Иммунокоррегирующая терапия больным после мезентерикокавального и сплено(супра)ренального шунтирования должна быть направлена на уменьшение антигенного воздействия на организм (ферменты, сорбенты и иммуноглобулины), на улучшение киллинга бактерий, дезинтоксикацию (азоксимера бромид) и активацию лейкопоэза (нуклеинат натрия).Мета: вивчити характер і ступінь порушень з боку імунної системи у дітей з допечінковою формою портальної гіпертензії (ДфПГ) після різних типів портокавального шунтування.Матеріали і методи. У дослідження включено 100 дітей, у яких оцінювали клітинну і гуморальну ланку імунної системи, фактори неспецифічного захисту, визначали основні імунопатологічні синдроми.Результати. У більшості дітей з ДфПГ виявлено напруженість гуморальної ланки імунітету (IgМ, IgA) і фагоцитозу, вторинний клітинний імунодефіцит зі зниженням загальної кількості лейкоцитів, лімфоцитів і субпопуляцій лімфоцитів (СD3+, СD4+, СD8+). Імунологічні показники хворих після сплено(супра)ренального шунтування статистично достовірно були більш наближеними до вікових норм (p&lt;0,05) порівняно з показниками хворих після мезентерикокавального шунтування. Однак оперативне лікування істотно не покращує стану імунної системи у дітей з ДфПГ.Висновки. Iмунокорегуюча терапія хворим після мезентерикокавального та сплено(супра)ренального шунтування повинна бути спрямована на зменшення антигенного впливу на організм (ферменти, сорбенти та імуноглобуліни), покращення кілінгу бактерій, дезінтоксикацію (азоксимеру бромід) та активацію лейкопоезу (нуклеїнат натрію)

Pediatric and young adult renal cell carcinoma

Renal cell carcinoma (RCC) is rare in children but is the most common renal tumor in adults. Pediatric RCC has different clinical characteristics, histopathology, and treatment compared with adult disease. Databases were reviewed from inception to February 2020, identifying 32 publications pertaining to 350 patients under 27 years. Surgery is the cornerstone for cure in localized RCC. Lymph node dissection remains controversial. Conventional radiotherapy has no curative role in RCC; similarly, conventional chemotherapy has not proven to be effective in large cohorts. Pediatric metastatic RCC has a poor outlook. There are no published prospective studies demonstrating which adjuvant therapy could improve outcome. Sunitinib, a tyrosine kinase inhibitor, is recommended in this group despite limited evidence. This review provides an overview for pediatric RCC, including the evolving role of precision medicine

Generating a Set of Reference Images for Reliable Condition Monitoring of Critical Infrastructure using Mobile Robots

The aim of this work is to reduce the amount of computational cost when monitoring the state of critical infrastructure objects using flying mobile robots equipped with correlation-extreme navigation system, based on minimizing the number of fragments of reference images. The goal is achieved by establishing a minimum permissible degree of correlation between the individual images, which form a set of reference images. The most essential result is substantiation of the approach to formation of a set of selective images based on scene correlation analysis and sufficiency of conservation of correlation connection of images in limits 0.6 ... 0.7. This reduces the amount of computation and extends the operating time of mobile robots while maintaining accuracy. The significance of the obtained results consists in the possibility of solving a complex task of forming a set of reference images, depending on the information content and stochastic conditions of sighting of critical infrastructure objects. The solution of this task will increase efficiency of critical infrastructure objects state control due to optimization of reference images number used in the monitoring process, increase operability, and provide high control reliability in stochastic sighting conditions. The novelty of the work lies in the fact that the method of process formalized description of forming a reference images set to ensure reliable monitoring of critical infrastructure facilities using flying mobile robots for various sectors of the economy, the practical application of which will ensure reliable control and their condition assessment