Monika Scheer1, Tobias Dantonello2, Peter Brossart3, Dagmar Dilloo4, Lothar Schweigerer5, Simone Feuchtgruber2, Monika Sparber-Sauer2, Christian Vokuhl6, Stefan S Bielack2,7, Thomas Klingebiel8, Ewa Koscielniak2,9, Thekla von Kalle10,11. 1. Stuttgart Cancer Center, Peadiatric 5 (Oncology, Hematology, Immunology), Klinikum Stuttgart-Olgahospital, Kriegsbergstrasse 62, D-70174, Stuttgart, Germany. monika.scheer@olgahospital-stuttgart.de. 2. Stuttgart Cancer Center, Peadiatric 5 (Oncology, Hematology, Immunology), Klinikum Stuttgart-Olgahospital, Kriegsbergstrasse 62, D-70174, Stuttgart, Germany. 3. Department of Oncology, University Hospital Bonn, Bonn, Germany. 4. Department of Paediatric Oncology, University Hospital Bonn, Bonn, Germany. 5. Department of Paediatric Oncology, Helios Klinikum, Buch, Berlin, Germany. 6. Kiel Peadiatric Tumour Registry, Department of Pediatric Pathology, University Hospital Kiel, Kiel, Germany. 7. Department of Paediatric Hematology and Oncology, University Children's Hospital, Muenster, Germany. 8. Department of Paediatric Oncology, University of Frankfurt (Main), Frankfurt, Germany. 9. Department of Paediatric Oncology, University of Tuebingen, Tuebingen, Germany. 10. Institute of Paediatric Radiology, Klinikum Stuttgart-Olgahospital, Stuttgart, Germany. 11. Department of Radiology, Diagnostic and Interventional Radiology, University of Tuebingen, Tuebingen, Germany.
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.
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.
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