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Prof. Dr. Karen E. Pollok

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Guest Editor

1. Department of Pediatrics, Hematology/Oncology, Indiana University School of Medicine, Indianapolis, IN 46202, USA
2. Department of Pediatrics, Herman B Wells Center for Pediatric Research, Indiana University School of Medicine, Indianapolis, IN 46202, USA
3. In Vivo Therapeutics Core, Indiana University Melvin and Bren Simon Comprehensive Cancer Center, Indiana University School of Medicine, Indianapolis, IN 46202, USA
4. Department of Medical and Molecular Genetics, Indiana University School of Medicine, Indianapolis, IN 46202, USA
5. Department of Pharmacology and Toxicology, Indiana University School of Medicine, Indianapolis, IN 46202, USA
Interests: pediatric sarcoma; glioblastoma; precision genomics; biomarkers; combination therapy; DNA damage response/repair; replication stress; CHK1; BETs; CDK4/CDK6

Prof. Dr. Pankita H. Pandya

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Guest Editor

Division of Pediatric Hematology and Oncology, Indiana University Simon Comprehensive Cancer Center, Indiana University School of Medicine , Indianapolis, IN 46202, USA
Interests: Pediatric sarcoma; Precision genomics; Epigenetics; EZH2; Replication Stress; Systems biology; Biomarkers

Special Issue Information

Dear Colleagues,

Osteosarcoma (OS), Rhabdomyosarcoma (RMS) and the Ewing sarcoma family of tumors (ESFTs) are extremely rare sarcomas that globally affect children as well as adolescents and young adults (AYA). Lack of significant therapeutic advancements in aggressive sarcomas heightens the urgency to understand and target factors involved in the molecular pathogenesis of these diseases. Treatments deemed suitable for adult sarcoma patients may not always be appropriate for pediatric and AYA patients due to differences in tumor biology, genetic alterations, and drug pharmacokinetics. Moreover, with the low number of pediatric patients available to enroll in clinical trials, a targeted personalized approach offers new options that can potentially improve overall survival and quality of life. In the era of precision genomics, the ability to integrate patient history and outcome data linked to pre-clinical investigations provides opportunities to discover and validate novel therapeutic approaches for pediatric sarcomas that are efficacious and safe.

In this Special Issue, we focus on the use of precision genomics to interrogate biomarkers that are diagnostic, prognostic and/or predictors of therapeutic response in pediatric sarcoma. Functional validation of -OMICS data in relevant in-vitro and in-vivo models will contribute to our understanding of the molecular mechanisms involved in the pathogenesis of these sarcomas and help guide decisions on appropriate therapeutic interventions. Research articles and reviews focused on key pathways of dysregulation as well as preclinical modeling of pediatric sarcomas that evaluate aspects of tumor pathogenesis are encouraged. This includes studies focused on inter- and intra-genetic tumor heterogeneity, microenvironmental cues, metastatic potential, and therapeutic resistance mechanisms. Advancing our knowledge in these areas will be essential in our quest to discover improved treatment strategies and ultimately, a cure for aggressive sarcomas in children and AYA.

Prof. Dr. Karen E. Pollok
Prof. Dr. Pankita H. Pandya
Guest Editors

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Keywords

sarcomas
pediatric sarcoma
precision genomics
biomarkers
diagnostic and prognostic

Published Papers (1 paper)

Research

12 pages, 608 KiB

Open AccessArticle

What Do We Know about Survival in Skeletally Premature Children Aged 0 to 10 Years with Ewing Sarcoma? A Multicenter 10-Year Follow-Up Study in 60 Patients

by Sarah E. Bosma, Lizz van der Heijden, Luis Sierrasesúmaga, Hans J. H. M. Merks, Lianne M. Haveman, Michiel A. J. van de Sande and Mikel San-Julián

Cancers 2022, 14(6), 1456; https://doi.org/10.3390/cancers14061456 - 12 Mar 2022

Cited by 6 | Viewed by 1820

Abstract

(1) Background: Younger age has been associated with better overall survival (OS) in Ewing sarcoma (ES), especially under the age of 10. The favorable survival in younger patients underlines the need for minimizing treatment burden and late sequelae. Our study aimed at describing clinical characteristics, treatment and outcome of a cohort of ES patients aged 0–10. (2) Methods: In this retrospective multicenter study, all consecutive ES patients aged 0–10, treated in four sarcoma centers in the Netherlands (n = 33) and one in Spain (n = 27) between 1982 and 2008, with a minimum follow-up of 10 years, were included. OS, local recurrence-free survival (LRFS) and distant metastasis-free survival (DMFS) were calculated. Potential factors of influence on OS (risk and protective factors) were analyzed. (3) Results: 60 patients with median follow-up 13.03 years were included. All patients were treated with chemotherapy in combination with local treatment, being surgery alone in 30 (50%) patients, radiotherapy (RT) alone in 12 (20%) patients or surgery plus RT in 18 (30%) patients (12 pre- and 6 postoperative). Limb salvage was achieved in 93% of patients. The 10-OS, -LRFS and -DMFS are 81% (95% CI: 71–91%), 89% (95% CI: 85–93%) and 81% (95% CI: 71–91%), respectively. Six patients developed LR, of which two developed subsequent DM; all had axial ES (pelvis, spine or chest wall), and these patients all died. Ten patients developed DM; eight died due to progressive disease, and two are currently in remission, both with pulmonary metastasis only. Negative or wide resection margin was significantly associated with better OS. Age < 6 years, tumor volume < 200 mL, absence of metastatic disease and treatment after 2000 showed trends towards better OS. Two patients developed secondary malignancy; both had chemotherapy combined with definitive RT for local treatment. (4) Conclusions: Overall survival of these youngest patients with ES was very good. Limb salvage surgery was achieved in >90% of patients. Wide resection margin was the only factor significantly associated with better survival. Full article

(This article belongs to the Special Issue Pediatric Sarcoma in the Era of Precision Genomics)

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