التفاصيل البيبلوغرافية
| العنوان: |
Patterns of Prior and Subsequent Neoplasms in Children and Adolescents With Soft Tissue Sarcomas |
| المؤلفون: |
Uckunkaya, Oliver, Nöllke, Peter, Hallmen, Erika, Becker, Cornelia, Stegmaier, Sabine, Sparber-Sauer, Monika, Vokuhl, Christian, Koscielniak, Ewa, Hettmer, Simone |
| المصدر: |
Journal of Pediatric Hematology/Oncology ; volume 42, issue 5, page e265-e270 ; ISSN 1077-4114 |
| بيانات النشر: |
Ovid Technologies (Wolters Kluwer Health) |
| سنة النشر: |
2020 |
| الوصف: |
Background: The occurrence of prior, concurrent and subsequent neoplasms (SN) represents a serious problem in children and adolescents with soft tissue sarcomas. Pathogenic germline variants contribute to the diagnosis of multiple neoplasms in sarcoma survivors. Materials and Methods: The records of 748 children and adolescents, diagnosed with soft tissue sarcomas and registered in trials/registries by the cooperative soft tissue sarcoma (Cooperative Weichteilsarkom Studie) group, were reviewed for the occurrence of SNs. Reference histology review was available for all cases; the presence of oncogenic fusions known at the time of diagnosis was confirmed for fusion-positive (F+) entities. Results: Concurrent or subsequent SNs developed in 13 of 473 survivors of fusion-negative (F−) sarcomas, for an 8-year cumulative SN incidence of 5% in survivors of F− sarcomas. In contrast, only 1 of 278 survivors of F+ sarcoma developed an SN. Twenty of 748 patients with soft tissue sarcomas had a history of prior neoplasms. Six of 14 patients who developed SNs after their index sarcomas met Chompret criteria for Li-Fraumeni syndrome. Nine of 20 patients who had tumors before their index sarcoma diagnosis had neurofibromatosis type 1 or neurofibromatosis type 1 spectrum tumors. Conclusion: Sarcoma phenotype/genotype and the sequence and nature of prior and subsequent neoplasms provide a window into underlying germline genetic susceptibilities in children and adolescents with soft tissue sarcomas. |
| نوع الوثيقة: |
article in journal/newspaper |
| اللغة: |
English |
| DOI: |
10.1097/mph.0000000000001837 |
| DOI: |
10.1097/MPH.0000000000001837 |
| الإتاحة: |
https://doi.org/10.1097/mph.0000000000001837Test |
| رقم الانضمام: |
edsbas.9EFD11A0 |
| قاعدة البيانات: |
BASE |
