Primary Ewing’s sarcoma of the kidney: description of two rare clinical cases and review of world literature

O. M. Romantsova, V. Y. Panarina, M. A. Bolokhonova, V. V. Khairullova, D. Khestanov, M. M. Efimova, T. R. Panferova, O. A. Ignatenko, N. A. Kozlov, A. Stroganova, K. Kirgizov, S. Varfolomeeva
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Abstract

Ewing’s sarcoma (ES) of the kidney is an extremely rare malignant tumor characterized by an aggressive course, and therefore the disease has an unfavorable prognosis. Due to the rare occurrence, standards for the treatment of ES of the kidney have not been developed; an integrated approach is often used in therapy. In this article, we present a description of two clinical cases of kidney damage with ES in patients aged 10 and 16 who were treated at the Research Institute of Pediatric Oncology and Hematology named after Academician of the Russian Academy of Medical Sciences L.A. Durnov at N.N. Blokhin National Medical Research Center of Oncology, Ministry of Health of Russia. In the first case, there was a metastatic lesion of the kidney with the localization of the primary focus in the pelvic bones, in the second, primary ES of the kidney was diagnosed. Both patients had stage IVb at the time of diagnosis (TNM classification American Joint Commission on Cancer). In both patients, after induction chemotherapy, a complete therapeutic response (complete therapeutic pathomorphosis) was obtained. In an integrated approach, in addition to standard chemotherapy, surgery and radiation therapy, in both cases, high-dose chemotherapy with autologous peripheral stem cell transplantation was used, which made it possible to create favorable conditions for long-term remission in one of the patients, even despite the initial prevalence of the process.
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肾脏原发性尤文氏肉瘤:两个罕见临床病例的描述和世界文献综述
肾脏尤文氏肉瘤(ES)是一种极为罕见的恶性肿瘤,病程凶险,预后不良。由于其罕见性,肾脏埃文氏肉瘤的治疗标准尚未制定,通常采用综合方法进行治疗。本文介绍了俄罗斯卫生部 N.N. Blokhin 国家肿瘤医学研究中心以俄罗斯医学科学院院士 L.A. Durnov 命名的儿科肿瘤学和血液学研究所治疗的两例肾损伤合并 ES 的临床病例,患者年龄分别为 10 岁和 16 岁。第一个病例是肾脏转移性病变,原发灶位于盆骨,第二个病例被诊断为肾脏原发性 ES。两名患者确诊时均为 IVb 期(美国癌症联合委员会 TNM 分期)。两名患者在接受诱导化疗后,均获得了完全治疗反应(完全治疗性病理改变)。在综合治疗中,除了标准化疗、手术和放疗外,两名患者都接受了大剂量化疗和自体外周干细胞移植,从而为其中一名患者的长期缓解创造了有利条件,尽管这一过程最初很普遍。
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来源期刊
Russian Journal of Pediatric Hematology and Oncology
Russian Journal of Pediatric Hematology and Oncology Medicine-Pediatrics, Perinatology and Child Health
CiteScore
0.40
自引率
0.00%
发文量
36
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