Background/Objectives: The practice of palliative radiation therapy (RT) is based on extrapolation from adult literature. We evaluated patterns of pediatric palliative RT to describe regimens used to identify opportunity for future pediatric-specific clinical trials. Results: Of 3,225 pediatric patients, 365 (11%) were treated with palliative intent to a total of 427 disease sites. Anesthesia was required in 10% of patients. Treatment was delivered to metastatic disease in 54% of patients. Histologies included neuroblastoma (30%), osteosarcoma (18%), leukemia/lymphoma (12%), rhabdomyosarcoma (12%), medulloblastoma/ependymoma (12%), Ewing sarcoma (8%), and other (8%). Indications included pain (43%), intracranial symptoms (23%), respiratory compromise (14%), cord compression (8%), and abdominal distention (6%).Sites included nonspine bone (35%), brain (16% primary tumors, 6% metastases), abdomen/pelvis (15%), spine (12%), head/neck (9%), and lung/mediastinum (5%). Re-irradiation comprised 16% of cases. Techniques employed three-dimensional conformal RT (41%), intensity-modulated RT (23%), conventional RT (26%), stereotactic body RT (6%), protons (1%), electrons (1%), and other (2%). The most common physician-reported barrier to consideration of palliative RT was the concern about treatment toxicity (83%). Conclusion:There is significant diversity of practice in pediatric palliative RT. Combined with ongoing research characterizing treatment response and toxicity, these data will inform the design of forthcoming clinical trials to establish effective regimens and minimize treatment toxicity for this patient population. K E Y W O R D Spalliative radiation therapy, palliative therapy, pediatric radiation therapy Abbreviations: RT, radiation therapy; SBRT, stereotactic body radiation therapy
Use of IGRT in children was prevalent at all consortium institutions. There was treatment site-specific variability in IGRT use and technique across institutions, although practices varied less at proton facilities. Despite use of IGRT, there was no consensus of optimum PTV margin by treatment site. Given the desire to restrict any additional radiation exposure in children to instances where the exposure is associated with measureable benefit, prospective studies are warranted to optimize IGRT protocols by modality and treatment site.
Clear cell sarcoma of the kidney (CCSK) is a rare malignant renal tumor in children, which accounts for 2–5% of pediatric kidney malignancies. The aim of the study was to analyze the results of therapy of patients with CCSK treated in Dmitry Rogachev National Medical Research Center оf Pediatric Hematology, Oncology and Immunology. Retrospective analysis of patients with a histologically confirmed diagnosis of CCSK treated for the period 01.2012–02.2020 (98 months) was done. The study was approved by the Independent Ethics Committee and the Scientific Council of the D. Rogachev NMRCPHOI of the Ministry of Healthcare of the Russian Federation. Demographic characteristics, clinical symptoms, methods of diagnosis, and treatment modalities were analyzed. Patients were treated according to the protocols of the SIOP-RTSG group (SIOP 93-01, SIOP-2001, SIOP-RTSG-2016). The stage was assigned according to the SIOP classification. Overall and event-free survival was assessed by the Kaplan–Mayer method. The analysis of the results was carried out on 01.03.2021. The analysis included 10 patients with CCSK. The median age at the time of diagnosis of CCSK was 30.1 months (range 13.5–70.8 months). All patients were male. The duration from the onset of the first symptoms/detection of the tumor to the diagnosis was 0.8 months (range 0.1–3.2 months). The diagnosis was established on the basis of clinical and radiological data (n = 9) and biopsy (n = 1). Distant metastases at the time of diagnosis were detected in 1 (10%) patient (localization of metastases - lungs). The median tumor volume was 439 cm3 (range 256–996 cm3 ). Preoperative chemotherapy was performed in all patients (AV regimen (actinomycin D, doxorubicin) in 7 (70%) patients). Assessment of response after preoperative chemotherapy showed tumor regression in 3/10 (in 1/7 with AV regimen), tumor progression in 5 and stable disease in 2 patients. Surgical treatment in the extent of nephrectomy was performed in all patients. In 1 (10%) case, intraoperative tumor rupture was documented. Distribution of patients by local stages: I – 4/10 (40%), II – 2/10 (20%), III – 4/10 (40%) (including 1 patient with distant metastases). In 1 patient, a left thoracotomy was performed to exclude lung metastases. Adjuvant chemotherapy was performed in all patients in accordance with the relevant protocols of the high-risk group: 7 – 4–5-drug regimen, 3 – AVD regimen (actinomycin D, vincristine, doxorubicin). Radiation therapy was performed in 6/10 (60%) patients. Outcomes: 9/10 (90%) – alive, 1/10 (10%) patient died (non-tumor-related death). 3-year event-free survival and overall survival were 78.8% (95% confidence interval (CI) 52.5–100) and 90.0% (95% CI 71.4–100) respectively. Intensive program therapy in patients with CCSK allows to achieve satisfactory results of treatment.
Introduction. The development of technologies for molecular genetic typing of tumors of the central nervous system (CNS) has significantly increased the specificity of traditional histological and immunohistochemical research methods. As a result, completely new variants of tumors have appeared, as well as improved results of treatment of tumors with a long-term favorable prognosis. Thanks to new biomarkers, among morphologically similar small-cell embryonic CNS tumors, extremely rare tumors have been identified: ETMR (embryonic tumor with multiple rosettes C19MC), CNS NB-FOXR2 (CNS neuroblastoma (NB) with FOXR2-activation), CNS EFT-CIC (tumor of the CNS sarcoma family of sarcoma alteration), CNS HGNET-MN1 (neuroepithelial tumor of the CNS of high grade with MN1 alteration), CNS HGNET-BCOR (neuroepithelial tumor of the CNS of high grade with BCOR alteration).The aim of the study was to assess the historical prerequisites for the emergence of a new rare nosological form “CNS NB with FOXR2- activation”, as well as presentation of own results of diagnostics and treatment of children with this tumor.Materials and methods. The results of diagnosis and treatment of 7 patients with CNS NB with FOXR2-activation were studied. Tumor identification was carried out using a new DNA methylation technique for CNS tumor profiles using the Heidelberg (Germany) brain tumor classifier (www.molecularneuropathology.org), which has recently become available in Russia.Results and discussion. Based on a review of international experience, the evolution of the appearance in the classification of CNS tumors, as well as the clinical and molecular features of CNS neuroblastoma with FOXR2-activation are presented. The authors present their own results of diagnostics and treatment of 7 patients with a rare tumor CNS neuroblastoma with FOXR2-activation.Conclusion. Biomarkers have been proposed for the differential diagnosis of small-cell PNET-like tumors of the CNS in children. Due to the molecular identification of this tumor in children, it became clear that such patients, provided that the treatment protocol is adequately followed, have quite satisfactory long-term survival results.
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