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.
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References
Klingebiel T, Boos J, Beske F et al (2008) Treatment of children with metastatic soft tissue sarcoma with oral maintenance compared to high dose chemotherapy: report of the HD CWS-96 trial. Pediatr Blood Cancer 50:739–745
Scheer M, Dantonello T, Hallmen E et al (2016) Primary metastatic synovial sarcoma: experience of the CWS study group. Pediatr Blood Cancer 63:1198–1206
Oberlin O, Rey A, Lyden E et al (2008) Prognostic factors in metastatic rhabdomyosarcomas: results of a pooled analysis from United States and European cooperative groups. J Clin Oncol 26:2384–2389
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 34:117–122
Scheer M, Dantonello T, Hallmen E et al (2016) Synovial sarcoma recurrence in children and young adults. Ann Surg Oncol 23:618–626
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–iii112
Hays DM, Newton W Jr, Soule EH et al (1983) Mortality among children with rhabdomyosarcomas of the alveolar histologic subtype. J Pediatr Surg 18:412–417
Koscielniak E, Rodary C, Flamant F et al (1992) Metastatic rhabdomyosarcoma and histologically similar tumors in childhood: a retrospective European multi-center analysis. Med Pediatr Oncol 20:209–214
Meyer WH, Spunt SL (2004) Soft tissue sarcomas of childhood. Cancer Treat Rev 30:269–280
Casanova M, Meazza C, Favini F et al (2009) Rhabdomyosarcoma of the extremities: a focus on tumors arising in the hand and foot. Pediatr Hematol Oncol 26:321–331
Miller DV, Coffin CM, Zhou H (2004) Rhabdomyosarcoma arising in the hand or foot: a clinicopathologic analysis. Pediatr Dev Pathol 7:361–369
Harms D (1995) Alveolar rhabdomyosarcoma: a prognostically unfavorable rhabdomyosarcoma type and its necessary distinction from embryonal rhabdomyosarcoma. Curr Top Pathol 89:273–296
Ben Arush M, Minard-Colin V, Mosseri V et al (2015) Does aggressive local treatment have an impact on survival in children with metastatic rhabdomyosarcoma? Eur J Cancer 51:193–201
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
Sorensen PH, Lynch JC, Qualman SJ et al (2002) PAX3-FKHR and PAX7-FKHR gene fusions are prognostic indicators in alveolar rhabdomyosarcoma: a report from the Children's Oncology Group. J Clin Oncol 20:2672–2679
Armitage P, Berry G (ed) (1994) Inferences from proportions. In: Statistical methods in medical research, 3rd edn. Blackwell Scientific Publications, Oxford, pp 118–125
Boellaard R, Delgado-Bolton R, Oyen WJ et al (2015) FDG PET/CT: EANM procedure guidelines for tumour imaging: version 2.0. Eur J Nucl Med Mol Imaging 42:328–354
Franzius C, Stauss J, Pfluger T et al (2010) Procedure guidelines for whole-body 18F-FDG PET and PET/CT in children with malignant diseases. Nuklearmedizin 49:225–233
Stauss J, Franzius C, Pfluger T et al (2008) Guidelines for 18F-FDG PET and PET-CT imaging in paediatric oncology. Eur J Nucl Med Mol Imaging 35:1581–1588
Olsen OE (2013) Advances in pediatric oncology MRI. Acta Radiol 54:1030–1036
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 27:10531
Eutsler EP, Khanna G (2016) Whole-body magnetic resonance imaging in children: technique and clinical applications. Pediatr Radiol 46:858–872
Aghighi M, Pisani LJ, Sun Z et al (2016) Speeding up PET/MR for cancer staging of children and young adults. Eur Radiol 26:4239–4248
Davis JT, Kwatra N, Schooler GR (2016) Pediatric whole-body MRI: a review of current imaging techniques and clinical applications. J Magn Reson Imaging 44:783–793
Ponisio MR, McConathy J, Laforest R et al (2016) Evaluation of diagnostic performance of whole-body simultaneous PET/MRI in pediatric lymphoma. Pediatr Radiol 46:1258–1268
Benusiglio PR, Brugieres L, Caron O (2016) Whole-Body MRI screening in children with Li-Fraumeni and other cancer predisposition syndromes. AJR Am J Roentgenol 206:W52
Nievelstein RA, Littooij AS (2016) Whole-body MRI in paediatric oncology. Radiol Med 121:442–453
Schafer JF, Gatidis S, Schmidt H et al (2014) Simultaneous whole-body PET/MR imaging in comparison to PET/CT in pediatric oncology: initial results. Radiology 273:220–231
Sher AC, Seghers V, Paldino MJ et al (2016) Assessment of sequential PET/MRI in comparison with PET/CT of pediatric lymphoma: a prospective study. AJR Am J Roentgenol 206:623–631
Volker T, Denecke T, Steffen I et al (2007) Positron emission tomography for staging of pediatric sarcoma patients: results of a prospective multicenter trial. J Clin Oncol 25:5435–5441
Tateishi U, Hosono A, Makimoto A et al (2009) Comparative study of FDG PET/CT and conventional imaging in the staging of rhabdomyosarcoma. Ann Nucl Med 23:155–161
Weihkopf T, Blettner M, Dantonello T et al (2008) Incidence and time trends of soft tissue sarcomas in German children 1985-2004 — a report from the population-based German Childhood Cancer Registry. Eur J Cancer 44:432–440
Kuttesch JF Jr, Parham DM, Kaste SC et al (1995) Embryonal malignancies of unknown primary origin in children. Cancer 75:115–121
Pappo AS, Shapiro DN, Crist WM et al (1995) Biology and therapy of pediatric rhabdomyosarcoma. J Clin Oncol 13:2123–2139
Dantonello TM, Int-Veen C, Harms D et al (2009) Cooperative trial CWS-91 for localized soft tissue sarcoma in children, adolescents, and young adults. J Clin Oncol 27:1446–1455
Asmar L, Gehan EA, Newton WA et al (1994) Agreement among and within groups of pathologists in the classification of rhabdomyosarcoma and related childhood sarcomas. Report of an international study of four pathology classifications Cancer 74:2579–2588
Acknowledgements
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.
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Scheer, M., Dantonello, T., Brossart, P. et al. Importance of whole-body imaging with complete coverage of hands and feet in alveolar rhabdomyosarcoma staging. Pediatr Radiol 48, 648–657 (2018). https://doi.org/10.1007/s00247-017-4066-8
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DOI: https://doi.org/10.1007/s00247-017-4066-8