Importance of whole-body imaging with complete coverage of hands and feet in alveolar rhabdomyosarcoma staging
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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.
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.
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.
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.
KeywordsAdolescent Alveolar rhabdomyosarcoma Child Extremity tumors Metastases Silent lesions Soft-tissue sarcoma Staging examinations Whole-body imaging
We are indebted to the personnel of all participating institutions and thank all patients, parents and guardians for their willingness to participate in the CWS-trials.
The study was supported by the German Childhood Cancer Foundation, Bonn; German Cancer Aid, Bonn; Federal Ministry of Research and Technology, Bonn, and the Förderkreis Krebskranke Kinder e.V., Stuttgart, Germany.
Compliance with Ethical Standards
Conflicts of Interest
- 4.Weigel BJ, Lyden E, Anderson JR et al (2016) Intensive multiagent therapy, including dose-compressed cycles of ifosfamide/etoposide and vincristine/doxorubicin/cyclophosphamide, irinotecan, and radiation, in patients with high-risk rhabdomyosarcoma: a report from the Children's Oncology Group. J Clin Oncol 34:117–122CrossRefPubMedGoogle Scholar
- 6.The ESMO/European Sarcoma Network Working Group (2014) Soft tissue and visceral sarcomas: ESMO Clinical Practice Guidelines for diagnosis, treatment and follow-up. Ann Oncol 25, Issue suppl_3, 1, iii102–iii112Google Scholar
- 14.Mohan AC, Venkatramani R, Okcu MF et al (2017) Local therapy to distant metastatic sites in stage IV rhabdomyosarcoma. Pediatr Blood Cancer. https://doi.org/10.1002/pbc.26859
- 16.Armitage P, Berry G (ed) (1994) Inferences from proportions. In: Statistical methods in medical research, 3rd edn. Blackwell Scientific Publications, Oxford, pp 118–125Google Scholar
- 21.Roberge D, Hickeson M, Charest M et al (2009) Utility of total body FDG PET/CT imaging in the initial staging of soft-tissue sarcoma. J Clin Oncol 27:10531Google Scholar