The article presents an analysis of treatment outcomes of pediatric patients with classical Hodgkin's lymphoma (HL) in the Republic of Belarus over more than a 20-year period. Patients aged 0 to 18 years with HL received therapy according to the DALHD-90(m) protocol. Therapy modifications involved the use of systemic chemotherapy according to the OPPA regimen (vincristine, procarbazine, prednisone and doxorubicin) for patients of both sexes and dose-reduced involved-field radiotherapy (20 Gy) for patients with early or intermediate stage HL. The aim of our study was to analyze the survival and clinical characteristics of pediatric patients with HL. The study was approved by the Independent Ethics Committee and the Scientific Council of Belarusian Research Center for Pediatric Oncology, Hematology and Immunology of the Ministry of Health of the Republic of Belarus. The analysis included 606 patients with HL, who had received treatment at the Belarusian Research Center for Pediatric Oncology, Hematology and Immunology from January 1, 1998 until December 31, 2020. The median age was 15.3 years. At 23 years, the event-free survival of the study patients was 85 ± 2%, the overall survival was 93 ± 1%, and the cumulative recurrence rate was 10 ± 1.3%. The long-term treatment results of pediatric patients with HL in the Republic of Belarus indicate that the protocol therapy is highly effective and can achieve outcomes that are equivalent to those in countries with a developed healthcare system. The applied therapeutic strategy demonstrates clinical efficacy in stage I–III HL. About 15% of patients with advanced HL (stage IV) constitute the main target group for which a search for new treatment approaches improving treatment outcomes is required.
For better understanding cancer pathogenesis and searching a potential target for antineoplastic therapy, the authors have studied mRNA expression profile in tissues from 39 children with histological confirmed malignant sarcomas and from 23 patients with bone and soft tissue nonmalignant lesions. mRNA levels of Angiogenesis-related genes VEGFA (including isoforms of 121, 165, 189), VEGFC, VEGFR-1, VEGFR-2, VEGFR-3, HIF-1α, TF, TFPI-1, TFPI-2, uPA, PAI-1 in pediatric specimens were examined using quantitative reverse transcriptase-polymerase chain reaction (qRT-PCR). uPA, HIF-1α, VEGFR-1, VEGFR-2, VEGFR-3, and VEGFC mRNA levels from nonmalignant tissue were significantly higher than those from cancer tissue. On the other hand, isoform VEGFA121 and VEGFA165 and ratio VEGFA165/189 mRNA levels in cancer were higher in comparison with nonmalignant tissue. There was a strong correlation between VEGFA165 and VEGFA189 mRNA expression levels both in cancer tissue and in nonmalignant tissue. In grade 4 tumors in comparison with grade 2 tumors, there was a reduced VEGFA165/189 ratio. Moreover, TFPI-1 and TFPI-2 mRNA levels were significantly lower in sarcomas than in nonmalignant lesions and TFPI-2 was significantly lower in grade 4 tumors than in grade 2. The present data suggested that mRNA overexpression of angiogenesis-related genes is not a prerogative of malignant tissues. The authors supposed that in pediatric bone and soft tissue pathology, high expression of mRNAs of some angiogenesis-related genes may be associated with inflammation and physiological angiogenesis rather than with the development of a malignant tumor. The authors showed the importance of VEGFA121 and/or VEGFA165 and VEGFA165/189 isoform ratio in pediatric sarcomas neoangiogenesis and TFPI-2 for tumors grade 4.
Ряд актуальных публикаций подтверждают важность точной молекулярно-генетической диагностики опухолей мягких тканей. Метод секвенирования следующего поколения может быть эффективным как для поиска мишеней таргетной терапии, прогнозирования агрессивного поведения новообразования, так и для уточнения диагноза саркомы в нестандартных случаях. В настоящем исследовании представлен случай FOXO1-негативной альвеолярной рабдомиосаркомы у 3-летнего ребенка. Для верификации диагноза был применен метод высокопроизводительного секвенирования. Проведена молекулярногенетическая диагностика образца опухолевой ткани с использованием панели генов TruSight Tumor 170 (Illumina, США). В результате исследования обнаружена редкая транслокация NZD1-FGFR4, ранее не описанная в литературе при альвеолярных рабдомиосаркомах. Родители пациентки дали согласие на использование информации, в том числе фотографий ребенка, в научных исследованиях и публикациях.
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