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A Mouse Model of Incompletely Resected Soft Tissue Sarcoma for Testing (Neo)adjuvant Therapies
Published on: July 28, 2020
The role of radiology in paediatric soft tissue sarcomas
1Radiology Department, Great Ormond Street Hospital for Children, London, WC1N 3JH, UK. parkk1@gosh.nhs.uk
Insights
Paediatric soft tissue sarcomas (STS) are childhood cancers originating from mesenchymal tissue. Improved survival rates are noted, with radiology crucial for diagnosis, staging, and management of these diverse tumours.
Area of Science:
- Paediatric Oncology
- Medical Imaging
- Surgical Pathology
Background:
- Paediatric soft tissue sarcomas (STS) represent 7% of childhood malignancies, arising from primitive mesenchymal tissue.
- Rhabdomyosarcomas (RMS) and non-rhabdomyomatous soft tissue sarcomas (NRSTS) constitute the main subtypes.
- Prognosis is influenced by patient age, primary site, tumour characteristics, and disease extent.
Purpose of the Study:
- To review the epidemiology, histology, clinical presentation, staging, and prognosis of paediatric STS.
- To elucidate the critical role of radiology in the management of paediatric STS.
Main Methods:
- Comprehensive literature review of paediatric soft tissue sarcomas.
- Analysis of epidemiological data, histological classifications, and clinical presentation.
- Evaluation of the diagnostic and follow-up utility of radiological modalities.
Main Results:
- Survival rates for paediatric STS have significantly improved.
- Radiology is indispensable for initial diagnosis, staging, and monitoring treatment complications.
- Tumour biology and patient factors significantly impact outcomes.
Conclusions:
- Paediatric STS management requires a multidisciplinary approach, integrating clinical, pathological, and radiological expertise.
- Continued international research aims to further enhance survival and minimize treatment-related morbidity.
- Radiological assessment is fundamental throughout the patient journey for soft tissue sarcomas.
Abstract:
Paediatric soft tissue sarcomas (STS) are a group of malignant tumours that originate from primitive mesenchymal tissue and account for 7% of all childhood tumours. Rhabdomyosarcomas (RMS) and undifferentiated sarcomas account for approximately 50% of soft tissue sarcomas in children and non-rhabdomyomatous soft tissue sarcomas (NRSTS) the remainder. The prognosis and biology of STS tumours vary greatly depending on the age of the patient, the primary site, tumour size, tumour invasiveness, histologic grade, depth of invasion, and extent of disease at diagnosis. Over recent years, there has been a marked improvement in survival rates in children and adolescents with soft tissue sarcoma and ongoing international studies continue to aim to improve these survival rates whilst attempting to reduce the morbidity associated with treatment. Radiology plays a crucial role in the initial diagnosis and staging of STS, in the long term follow-up and in the assessment of many treatment related complications. We review the epidemiology, histology, clinical presentation, staging and prognosis of soft tissue sarcomas and discuss the role of radiology in their management.
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