Abstract:
BACKGROUND:Alveolar rhabdomyosarcoma commonly arises in the extremities and is characterized by aggressive biology and high frequency of metastases. Whole-body imaging is increasingly employed in pediatric oncology but not recommended as standard in the staging of soft-tissue sarcomas. OBJECTIVE:After observing patients with a large symptomatic alveolar rhabdomyosarcoma lesion and a smaller silent lesion in the more distal part of an extremity we sought to estimate the frequency of this constellation. MATERIALS AND METHODS:We retrospectively evaluated the data of prospectively registered paediatric patients (age <21 years) with alveolar rhabdomyosarcoma in the SoTiSaR (Soft Tissue Sarcoma Registry) of the Cooperative Weichteilsarkom Studiengruppe (CWS) 09/2011-04/2015 with regard to whole-body imaging. RESULTS:Seventy-five patients were eligible. Images of 57 patients had been submitted for reference consultation, including 80 whole-body examinations in 36 patients. Among them were 5 patients (14%, 95% confidence interval 3-25%) who had been diagnosed because of a symptomatic lesion while an additional silent lesion in the distal part of an extremity had remained unnoticed and had only been detected by later whole-body imaging. It is noteworthy that in 42 (53%) of all 80 whole-body examinations, the hands and feet had been only partially covered or completely excluded. CONCLUSION:In alveolar rhabdomyosarcoma silent lesions can be overlooked when the distal parts of the limbs are not thoroughly examined and not completely covered by imaging. Missing them influences treatment decisions and prognosis. Our results should be considered when evaluating the potential role of whole-body imaging in rhabdomyosarcoma.
journal_name
Pediatr Radioljournal_title
Pediatric radiologyauthors
Scheer M,Dantonello T,Brossart P,Dilloo D,Schweigerer L,Feuchtgruber S,Sparber-Sauer M,Vokuhl C,Bielack SS,Klingebiel T,Koscielniak E,von Kalle T,Cooperative Weichteilsarkom Studiengruppe (CWS).doi
10.1007/s00247-017-4066-8subject
Has Abstractpub_date
2018-05-01 00:00:00pages
648-657issue
5eissn
0301-0449issn
1432-1998pii
10.1007/s00247-017-4066-8journal_volume
48pub_type
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journal_title:Pediatric radiology
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