vestimИзвестия Национальной академии наук Беларуси. Серия медицинских наукProceedings of the National Academy of Sciences of Belarus, Medical series1814-60232524-2350The Republican Unitary Enterprise Publishing House "Belaruskaya Navuka"vestim-347Research ArticleКЛИНИЧЕСКАЯ И ЭКСПЕРИМЕНТАЛЬНАЯ МЕДИЦИНАCLINICAL AND EXPERIMENTAL MEDICINEПРИМИТИВНАЯ НЕЙРОЭКТОДЕРМАЛЬНАЯ ОПУХОЛЬ КОСТЕЙ И МЯГКИХ ТКАНЕЙ У ПАЦИЕНТОВ ДЕТСКОГО ВОЗРАСТА РЕСПУБЛИКИ БЕЛАРУСЬ: КЛИНИЧЕСКИЕ ИСХОДЫ И ПОКАЗАТЕЛИ ВЫЖИВАЕМОСТИ ЗА 15-ЛЕТНИЙ ПЕРИОДCLINICAL OUTCOMES AND SURVIVAL RATES OVER THE 15-YEAR PERIOD IN PEDIATRIC PATIENTS WITH PERIPHERAL PRIMITIVE NEUROECTODERMAL TUMOR OF BONE AND SOFT TISSUES IN REPUBLIC OF BELARUSКиселёвЛ. П.KisialeuL.

канд. мед. наук, заведующий отделением.

Ph. D. (Med.), Chief of the Department.

leonslight@mail.ruАлейниковаО. В.AleinikovaO.

чл.-кор., д-р мед. наук, профессор, директор

Corresponding Member, D. Sc. (Med.), Professor, Director

aleinikova2004@mail.ruРеспубликанский научно-практический центр детской онкологии, гематологии и иммунологииRepublican Research Center for Pediatric Oncology, Hematology and Immunology201701032017013845Copyright © Киселёв Л.П., Алейникова О.В., 20172017Киселёв Л.П., Алейникова О.В.Kisialeu L., Aleinikova O.Данная работа распространяется под лицензией Creative Commons Attribution 4.0.This work is licensed under a Creative Commons Attribution 4.0 License.https://vestimed.belnauka.by/jour/article/view/347

Оценены клинико-патологические характеристики и долгосрочные показатели выживаемости пациентов с примитивной периферической нейроэктодермальной опухолью (пПНЭО) костей и мягких тканей. В анализ включены 53 пациента с пПНЭО, получавших лечение с 1999 по 2014 г. Общая выживаемость для всей когорты  пациентов составила 60,2  %, при локализованных и метастатических формах – 70,3 и 20,0  % соответственно. Не выявлено значимых отличий в характеристиках пациентов (пол, возраст, уровень лактатдегидрогеназы, размер и локализация опухоли и др.), позволяющих прогнозировать исход заболевания перед началом терапии.Показатели долгосрочной выживаемости пациентов детского возраста с пПНЭО костей и мягких тканей в Республике Беларусь соответствует стандартам стран с развитой структурой здравоохранения. Молекулярные маркеры, характеризующие биологию новообразования, необходимы для прогнозирования исхода заболевания у пациентов,опухоль которых может быть резистентной к современным схемам лечения.

Peripheral primitive neuroectodermal tumor (pPNET) can occur in any bone or soft tissue and is about a quarter of all pediatric patients with Ewing’s sarcoma family of tumors. The aim of this study was to investigate whether patient characteristics differ between the cases with favorable and unfavorable outcomes. Thus, 53 pediatric patients with pPNET from the Republic of Belarus reported to the sub-cancer registry database from 1999 to 2014 were evaluated. The survival rate was estimated by means of the Kaplan-Meier method and was compared using log-rank tests and Cox proportional hazard models. Overall survival (OS) was superior for localized pPNET compared with metastatic forms (70.3  % vs 20.0  %;P =   0.0016). Fifteen-year OS for the entire cohort of patients was 60.2  %. Known clinical prognostic factors (age, sex, tumors volume, response to treatment and other) did not differ between patients with favorable and unfavorable outcomes.Treatment results for pediatric patients with pPNET from the Republic of Belarus comply with the generally accepted standards. Patients with metastatic status need new treatment strategies. Search for new predictive markers is required for early detection of resistant to conventional treatment patients with localized pPNET.

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The authors declare that there are no conflicts of interest present.