Skip to main content

Advertisement

SpringerLink
Log in

Overview of the clinical and imaging features of the most common non-rhabdomyosarcoma soft-tissue sarcomas

  • Pediatric oncologic imaging
  • Published:
Pediatric Radiology Aims and scope Submit manuscript

Abstract

Non-rhabdomyosarcoma soft-tissue sarcoma (NRSTS) refers to a widely heterogeneous group of extraskeletal mesenchymal neoplasms accounting for approximately 4% of all childhood cancers. This article summarizes the clinical and imaging features of these rare tumors and describes in detail the three most common histological types of NRSTSs encountered in children — synovial sarcoma, malignant peripheral nerve sheath tumor and infantile fibrosarcoma. The author discusses the role of non-cross-sectional and cross-sectional imaging.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Log in via an institution

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Fig. 1
Fig. 2
Fig. 3
Fig. 4
Fig. 5
Fig. 6
Fig. 7
Fig. 8
Fig. 9

Similar content being viewed by others

References

  1. Crane JN (2018) Nonrhabdomyosarcoma soft tissue sarcomas. In: Medscape (https://emedicine.medscape.com/article/991351-overview). Accessed 12 April 2019

  2. Spunt SL, Skapek SX, Coffin CM (2008) Pediatric nonrhabdomyosarcoma soft tissue sarcomas. Oncologist 13:668–678

    Article  Google Scholar 

  3. Dasgupta R, Rodeberg D (2016) Non-rhabdomyosarcoma. Semin Pediatr Surg 25:284–289

    Article  Google Scholar 

  4. Black JO, Coffin CM, Parham DM et al (2016) Opportunities for improvement in pathology reporting of childhood nonrhabdomyosarcoma soft tissue sarcomas. Am J Clin Pathol 146:328–338

    Article  Google Scholar 

  5. Spunt SL, Wolden SL, Schofield DE, Skapek SX (2006) Non-rhabdomyosarcoma soft tissue sarcomas. In: Alberto SP (ed) Pediatric bone and soft tissue sarcomas. Springer, New York, pp 133–162

    Chapter  Google Scholar 

  6. American Joint Committee on Cancer (2002) AJCC cancer staging manual, 6th edn. Springer, New York

    Google Scholar 

  7. Spunt SL, Million L, Anderson J et al (2014) Risk-based treatment for nonrhabdomyosarcoma soft tissue sarcoma (NRSTS) in patients under 30 years of age. Children’s Oncology Group study ARST0332. J Clin Oncol 32:S15

  8. Sangkhathat S (2015) Current management of pediatric soft tissue sarcomas. World J Clin Pediatr 4:94–105

    Article  Google Scholar 

  9. McCarville MB (2015) Imaging techniques used in the diagnosis of pediatric tumors. In: Parham DM, Khoury JD, McCarville MB (eds) Pediatric malignancies: pathology and imaging. Springer, New York, pp 7–18

    Google Scholar 

  10. McCarville MB, Muzzafar S, Kao SC et al (2014) Imaging features of alveolar soft part sarcoma: a report from Children’s Oncology Group study ARST0332. AJR Am J Roentgenol 203:1345–1352

  11. Sargar K, Kao SC, Spunt et al (2015) MRI and CT of low-grade fibromyxoid sarcoma in children: a report from Children’s Oncology Group study ARST0332. AJR Am J Roentgenol 205:414–420

  12. Parham DM, Kaste SC, Raju A, McCarville BM (2015) Soft tissue sarcomas. In: Parham DM, Khoury JD, McCarville BM (eds) Pediatric malignancies: pathology and imaging. Springer, New York, pp 19–68

    Google Scholar 

  13. Khoury JD, Coffin CM, Spunt SL et al (2010) Grading of non-rhabdomyosarcoma soft tissue sarcoma in children and adolescents: a comparison of parameters used for the Fédération Nationale des Centers de Lutte Contre le Cancer and Pediatric Oncology Group systems. Cancer 116:2266–2274

  14. McCarville BM, Spunt SL, Skapek SX, Pappo AS (2002) Synovial sarcoma in pediatric patients. AJR Am J Roentgenol 178:797–801

    Article  Google Scholar 

  15. Coffin CM (1997) Synovial-based tumors and synovial sarcoma. In: Coffin CM, O’Shea PA, Dehner LP (eds) Pediatric soft tissue tumors: a clinical, pathological, and therapeutic approach. Williams & Wilkins, Baltimore, pp 255–310

    Google Scholar 

  16. Skapek SX, Chiu CH (2000) Cytogenetics and the biological basis of sarcoma. Curr Opin Oncol 12:315–322

    Article  CAS  Google Scholar 

  17. Bixby SD, Hettmer S, Taylor GA, Voss SD (2010) Synovial sarcoma in children: imaging features and common benign mimics. AJR Am J Roentgenol 195:1026–1032

    Article  Google Scholar 

  18. Jones BC, Sundaram M, Kransdorf MJ (1993) Synovial sarcoma: MR imaging findings in 34 patients. AJR Am J Roentgenol 161:827–830

    Article  CAS  Google Scholar 

  19. Murphey MD, Gibson MS, Jennings BT et al (2006) From the archives of the AFIP: imaging of synovial sarcoma with radiologic-pathologic correlation. Radiographics 26:1545–1565

    Article  Google Scholar 

  20. Morton MJ, Verquist TH, McLeod RA et al (1991) MR imaging of synovial sarcoma. AJR Am J Roentgenol 156:337–340

    Article  CAS  Google Scholar 

  21. Lisle JW, Eary JF, O’Sullivan J, Conrad EU (2009) Risk assessment based on FDG-PET imaging in patients with synovial sarcoma. Clin Orthop Rela Res 467:1605–1611

    Article  Google Scholar 

  22. Chang KJ, Lim I, Park JY et al (2015) The role of 18F-FDG PET/CT as a prognostic factor in patients with synovial sarcoma. Nucl Med Mol Imaging 49:33–41

    Article  CAS  Google Scholar 

  23. Weissberg Z, Sebro R (2018) Distinguishing synovial sarcoma from benign and malignant mimics: MR imaging indicators. Appl Radiol 47:15–21

    Google Scholar 

  24. Liang C, Mao H, Tan J et al (2015) Synovial sarcoma: magnetic resonance and computed tomography imaging features and differential diagnostic considerations. Oncol Letters 9:661–666

    Article  Google Scholar 

  25. Kao SC-S, Parham DM, Fuller C (2015) Malignant tumors of peripheral nerves. In: Parham DM, Khoury JD, McCarville MB (eds) Pediatric malignancies: pathology and imaging. Springer, New York, pp 399–414

    Google Scholar 

  26. Farid M, Demicco EG, Garcia R et al (2014) Malignant peripheral nerve sheath tumors. Oncologist 19:193–201

    Article  CAS  Google Scholar 

  27. McCarville MB (2016) What MRI can tell us about neurogenic tumors and rhabdomyosarcoma. Pediatr Radiol 46:881–890

    Article  Google Scholar 

  28. Rafailidis V, Kaziani T, Theocharides C (2014) Imaging of the malignant peripheral nerve sheath tumour with emphasis on ultrasonography: correlation with MRI. J Ultrasound 17:219–223

    Article  Google Scholar 

  29. Yu YH, Wu JT, Ye J, Chen MX (2016) Radiological findings of malignant peripheral nerve sheath tumor: reports of six cases and review of literature. World J Surg Oncol 14:142–149

    Article  Google Scholar 

  30. Ainsworth KE, Chavhan GB, Gupta AA et al (2014) Congenital infantile fibrosarcoma: review of imaging features. Pediatr Radiol 44:1124–1129

    Article  Google Scholar 

  31. Canale S, Vanel D, Couanet D (2009) Infantile fibrosarcoma: magnetic resonance imaging findings in six cases. Eur J Radiol 72:30–37

    Article  Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Department of Radiology,, University of Iowa Carver College of Medicine,, 200 Hawkins Drive, 3885JPP, Iowa City, IA, 52242, USA

    Simon C. Kao

Authors
  1. Simon C. Kao
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Simon C. Kao.

Ethics declarations

Conflicts of interest

None

Additional information

Publisher’s note

Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.

Rights and permissions

Reprints and permissions

About this article

Check for updates. Verify currency and authenticity via CrossMark

Cite this article

Kao, S.C. Overview of the clinical and imaging features of the most common non-rhabdomyosarcoma soft-tissue sarcomas. Pediatr Radiol 49, 1524–1533 (2019). https://doi.org/10.1007/s00247-019-04427-z

Download citation

  • Received:

  • Revised:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s00247-019-04427-z

Keywords

Search

Navigation