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The role of radiology in paediatric soft tissue sarcomas.

Park K, van Rijn R, McHugh K - Cancer Imaging (2008)

Bottom Line: 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.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.

View Article: PubMed Central - PubMed

Affiliation: Radiology Department, Great Ormond Street Hospital for Children, London, WC1N 3JH, UK. parkk1@gosh.nhs.uk

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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(a) Chest X-ray demonstrating a rhabdoid tumour involving the soft tissues of the left side of the neck and chest wall. (b) Coronal T2 weighted MRI of the same patient.
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Figure 2: (a) Chest X-ray demonstrating a rhabdoid tumour involving the soft tissues of the left side of the neck and chest wall. (b) Coronal T2 weighted MRI of the same patient.

Mentions: NRSTS can arise anywhere in the body but are most common in the extremities and trunk[25]. Presentation is usually with a painless mass but symptoms may occur secondary to local invasion or mass effect (Fig. 2). Systemic symptoms such as fever, night sweats or weight loss are rare but have been observed with widespread metastatic disease. MPNST may present with motor and sensory involvement. Rarely patients may present with metabolic disturbances[31,32]. Dillon et al.[25] looked at the anatomical location of a cohort of 75 cases of paediatric NRSTS and found 65% in the extremities, 28% in the trunk and 7% in the head and neck. Metastases at time of presentation were more common in the truncal tumours than those in the extremities. All upper limb tumours were localised at the time of presentation, whereas 78% of abdominal tumours had metastatic disease at the time of presentation.Figure 2


The role of radiology in paediatric soft tissue sarcomas.

Park K, van Rijn R, McHugh K - Cancer Imaging (2008)

(a) Chest X-ray demonstrating a rhabdoid tumour involving the soft tissues of the left side of the neck and chest wall. (b) Coronal T2 weighted MRI of the same patient.
© Copyright Policy
Related In: Results  -  Collection

Show All Figures
getmorefigures.php?uid=PMC2365455&req=5

Figure 2: (a) Chest X-ray demonstrating a rhabdoid tumour involving the soft tissues of the left side of the neck and chest wall. (b) Coronal T2 weighted MRI of the same patient.
Mentions: NRSTS can arise anywhere in the body but are most common in the extremities and trunk[25]. Presentation is usually with a painless mass but symptoms may occur secondary to local invasion or mass effect (Fig. 2). Systemic symptoms such as fever, night sweats or weight loss are rare but have been observed with widespread metastatic disease. MPNST may present with motor and sensory involvement. Rarely patients may present with metabolic disturbances[31,32]. Dillon et al.[25] looked at the anatomical location of a cohort of 75 cases of paediatric NRSTS and found 65% in the extremities, 28% in the trunk and 7% in the head and neck. Metastases at time of presentation were more common in the truncal tumours than those in the extremities. All upper limb tumours were localised at the time of presentation, whereas 78% of abdominal tumours had metastatic disease at the time of presentation.Figure 2

Bottom Line: 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.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.

View Article: PubMed Central - PubMed

Affiliation: Radiology Department, Great Ormond Street Hospital for Children, London, WC1N 3JH, UK. parkk1@gosh.nhs.uk

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.

Show MeSH
Related in: MedlinePlus