Skip to main content

Advertisement

SpringerLink
Log in

Pediatric soft tissue sarcoma of the limbs: clinical outcome of 97 patients

  • Original Article • TUMORS - PAEDIATRICS
  • Published:
European Journal of Orthopaedic Surgery & Traumatology Aims and scope Submit manuscript

Abstract

Purpose

Soft tissue sarcomas (STS) of the extremities in children are a heterogeneous group of tumors with a very different prognosis for which optimal treatment remains controversial.

Patients and methods

We retrospectively evaluated 97 patients younger than 15 years old affected by limb soft tissue sarcomas. All cases were histologically revised, and tumor grade was assessed according to the FNLCC system. Thirty-two were rhabdomyosarcoma (RMS) and 65 non-rhabdomyosarcoma (NRMSTS); among these, 40 (61.5%) were grade 3 according to FNLCC classification. Overall survival, local recurrence and distant metastasis were analyzed.

Results

Overall survival was 77.8% at 5 years and 69.7% at 10 years. Among grade 3 tumors, RMS had a worse prognosis over NRSTS. Similarly, tumors larger than 5 cm had a worse prognosis compared to smaller ones. Local recurrence-free survival was 90.7% at 5 years and 87.1% at 10 years with a better local control in grade 3 NRSTS over RMS and in tumors smaller than 2 cm.

Conclusion

Children affected by extremities RMS were confirmed to have the worst prognosis, in particular in case of metastasis at presentation. Differently from adult patients, hand and feet locations are frequent site for STS and 2 cm diameter should be taken as cut off for higher risk of LR. Similarly to adulthood STS, grading correlates with prognosis in NRSTS. The identification of prognostic variables should enable risk-adapted therapies to be planned.

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

Similar content being viewed by others

References

  1. Grovas A, Fremgen A, Rauck A, Ruymann FB, Hutchinson CL, Winchester DP, Menck HR (1997) The National Cancer Data Base report on patterns of childhood cancers in the United States. Cancer 80(12):2321–2332

    Article  CAS  PubMed  Google Scholar 

  2. Siegel DA, King J, Tai E, Buchanan N, Ajani UA, Li J (2014) Cancer incidence rates and trends among children and adolescents in the United States, 2001–2009. Pediatrics 134(4):e945–e955. doi:10.1542/peds.2013-3926

    Article  PubMed  PubMed Central  Google Scholar 

  3. Williams RF, Fernandez-Pineda I, Gosain A (2016) Pediatric sarcomas. Surg Clin North Am 96(5):1107–1125. doi:10.1016/j.suc.2016.05.012

    Article  PubMed  Google Scholar 

  4. Spunt SL, Skapek SX, Coffin CM (2008) Pediatric nonrhabdomyosarcoma soft tissue sarcomas. Oncologist 13(6):668–678. doi:10.1634/theoncologist.2007-0182

    Article  PubMed  Google Scholar 

  5. Kapoor G, Das K (2012) Soft tissue sarcomas in children. Indian J Pediatr 79(7):936–942. doi:10.1007/s12098-011-0560-4

    Article  PubMed  Google Scholar 

  6. Sangkhathat S (2015) Current management of pediatric soft tissue sarcomas. World J Clin Pediatr 4(4):94–105. doi:10.5409/wjcp.v4.i4.94

    Article  PubMed  PubMed Central  Google Scholar 

  7. Dasgupta R, Rodeberg DA (2012) Update on rhabdomyosarcoma. Semin Pediatr Surg 21(1):68–78. doi:10.1053/j.sempedsurg.2011.10.007

    Article  PubMed  Google Scholar 

  8. Loeb DM, Thornton K, Shokek O (2008) Pediatric soft tissue sarcomas. Surg Clin North Am 88(3):615–627, vii. doi:10.1016/j.suc.2008.03.008

    Article  PubMed  PubMed Central  Google Scholar 

  9. Ferrari A, Miceli R, Meazza C, Zaffignani E, Gronchi A, Piva L, Collini P, Podda M, Massimino M, Luksch R, Cefalo G, Terenziani M, Spreafico F, Polastri D, Fossati-Bellani F, Casanova M, Mariani L (2009) Soft tissue sarcomas of childhood and adolescence: the prognostic role of tumor size in relation to patient body size. J Clin Oncol 27(3):371–376. doi:10.1200/JCO.2007.15.4542

    Article  PubMed  Google Scholar 

  10. Pratt CB, Pappo AS, Gieser P, Jenkins JJ, Salzbergdagger A, Neff J, Rao B, Green D, Thomas P, Marcus R, Parham D, Maurer H (1999) Role of adjuvant chemotherapy in the treatment of surgically resected pediatric nonrhabdomyosarcomatous soft tissue sarcomas: a Pediatric Oncology Group Study. J Clin Oncol 17(4):1219. doi:10.1200/JCO.1999.17.4.1219

    Article  CAS  PubMed  Google Scholar 

  11. Bramwell VH (2001) Adjuvant chemotherapy for adult soft tissue sarcoma: is there a standard of care? J Clin Oncol 19(5):1235–1237

    Article  CAS  PubMed  Google Scholar 

  12. Yang JC, Chang AE, Baker AR, Sindelar WF, Danforth DN, Topalian SL, DeLaney T, Glatstein E, Steinberg SM, Merino MJ, Rosenberg SA (1998) Randomized prospective study of the benefit of adjuvant radiation therapy in the treatment of soft tissue sarcomas of the extremity. J Clin Oncol 16(1):197–203

    Article  CAS  PubMed  Google Scholar 

  13. Reed DR, Hayashi M, Wagner L, Binitie O, Steppan DA, Brohl AS, Shinohara ET, Bridge JA, Loeb DM, Borinstein SC, Isakoff MS (2017) Treatment pathway of bone sarcoma in children, adolescents, and young adults. Cancer. doi:10.1002/cncr.30589

    Google Scholar 

  14. Blakely ML, Spurbeck WW, Pappo AS, Pratt CB, Rodriguez-Galindo C, Santana VM, Merchant TE, Prichard M, Rao BN (1999) The impact of margin of resection on outcome in pediatric nonrhabdomyosarcoma soft tissue sarcoma. J Pediatr Surg 34(5):672–675

    Article  CAS  PubMed  Google Scholar 

  15. Panda SP, Chinnaswamy G, Vora T, Prasad M, Bansal D, Kapoor G, Radhakrishnan V, Agarwala S, Laskar S, Arora B, Kaur T, Rath GK, Bakhshi S (2017) Diagnosis and management of rhabdomyosarcoma in children and adolescents: ICMR consensus document. Indian J Pediatr 84(5):393–402. doi:10.1007/s12098-017-2315-3

    Article  PubMed  Google Scholar 

  16. Terezakis SA, Wharam MD (2013) Radiotherapy for rhabdomyosarcoma: indications and outcome. Clin Oncol 25(1):27–35. doi:10.1016/j.clon.2012.07.009

    Article  CAS  Google Scholar 

  17. Frustaci S, Gherlinzoni F, De Paoli A, Bonetti M, Azzarelli A, Comandone A, Olmi P, Buonadonna A, Pignatti G, Barbieri E, Apice G, Zmerly H, Serraino D, Picci P (2001) Adjuvant chemotherapy for adult soft tissue sarcomas of the extremities and girdles: results of the Italian randomized cooperative trial. J Clin Oncol 19(5):1238–1247. doi:10.1200/JCO.2001.19.5.1238

    Article  CAS  PubMed  Google Scholar 

  18. Walter AW, Shearer PD, Pappo AS, Greenwald CA, Rao BN, Bowman LC, Furman WL, Gajjar A, Jenkins JJ, Pratt CB (1998) A pilot study of vincristine, ifosfamide, and doxorubicin in the treatment of pediatric non-rhabdomyosarcoma soft tissue sarcomas. Med Pediatr Oncol 30(4):210–216

    Article  CAS  PubMed  Google Scholar 

  19. Million L, Donaldson SS (2012) Resectable pediatric nonrhabdomyosarcoma soft tissue sarcoma: which patients benefit from adjuvant radiation therapy and how much? ISRN Oncol 2012:341408. doi:10.5402/2012/341408

    PubMed  PubMed Central  Google Scholar 

  20. Enneking WF, Spanier SS, Goodman MA (1980) A system for the surgical staging of musculoskeletal sarcoma. Clin Orthop Relat Res 153:106–120

    Google Scholar 

  21. Jo VY, Fletcher CD (2014) WHO classification of soft tissue tumours: an update based on the 2013 (4th) edition. Pathology 46(2):95–104. doi:10.1097/PAT.0000000000000050

    Article  CAS  PubMed  Google Scholar 

  22. Trojani M, Contesso G, Coindre JM, Rouesse J, Bui NB, de Mascarel A, Goussot JF, David M, Bonichon F, Lagarde C (1984) Soft-tissue sarcomas of adults; study of pathological prognostic variables and definition of a histopathological grading system. Int J Cancer 33(1):37–42

    Article  CAS  PubMed  Google Scholar 

  23. Rodeberg DA, Stoner JA, Garcia-Henriquez N, Randall RL, Spunt SL, Arndt CA, Kao S, Paidas CN, Million L, Hawkins DS (2011) Tumor volume and patient weight as predictors of outcome in children with intermediate risk rhabdomyosarcoma: a report from the Children’s Oncology Group. Cancer 117(11):2541–2550. doi:10.1002/cncr.25719

    Article  PubMed  Google Scholar 

  24. Malempati S, Rodeberg DA, Donaldson SS, Lyden ER, Anderson JR, Hawkins DS, Arndt CA (2011) Rhabdomyosarcoma in infants younger than 1 year: a report from the Children’s Oncology Group. Cancer 117(15):3493–3501. doi:10.1002/cncr.25887

    Article  PubMed  PubMed Central  Google Scholar 

  25. Giuliano AE, Eilber FR (1985) The rationale for planned reoperation after unplanned total excision of soft-tissue sarcomas. J Clin Oncol 3(10):1344–1348. doi:10.1200/JCO.1985.3.10.1344

    Article  CAS  PubMed  Google Scholar 

  26. Ferrari A, Chi YY, De Salvo GL, Orbach D, Brennan B, Randall RL, McCarville MB, Black JO, Alaggio R, Hawkins DS, Bisogno G, Spunt SL (2017) Surgery alone is sufficient therapy for children and adolescents with low-risk synovial sarcoma: a joint analysis from the European paediatric soft tissue sarcoma Study Group and the Children’s Oncology Group. Eur J Cancer 78:1–6. doi:10.1016/j.ejca.2017.03.003

    Article  PubMed  Google Scholar 

  27. Bianchi G, Sambri A, Cammelli S, Galuppi A, Cortesi A, Righi A, Caldari E, Ferrari S, Donati D (2017) Impact of residual disease after “unplanned excision” of primary localized adult soft tissue sarcoma of the extremities: evaluation of 452 cases at a single Institution. Musculoskelet Surg. doi:10.1007/s12306-017-0475-y

    Google Scholar 

  28. Fawzy M, Sedky M, ElZomor H, El Sherbiny M, Salama E, Mahdy A (2016) Low-grade nonrhabdomyosarcoma soft tissue sarcoma: what is peculiar for childhood. J Pediatr Hematol Oncol 38(8):615–621. doi:10.1097/MPH.0000000000000657

    Article  CAS  PubMed  Google Scholar 

  29. Khoury JD, Coffin CM, Spunt SL, Anderson JR, Meyer WH, Parham DM (2010) Grading of nonrhabdomyosarcoma soft tissue sarcoma in children and adolescents: a comparison of parameters used for the Federation Nationale des Centers de Lutte Contre le Cancer and Pediatric Oncology Group Systems. Cancer 116(9):2266–2274. doi:10.1002/cncr.24929

    PubMed  PubMed Central  Google Scholar 

  30. Brennan MF, Antonescu CR, Moraco N, Singer S (2014) Lessons learned from the study of 10,000 patients with soft tissue sarcoma. Ann Surg 260(3):416–421. doi:10.1097/SLA.0000000000000869 (discussion 421–412)

    Article  PubMed  PubMed Central  Google Scholar 

  31. Bianchi G, Sambri A, Righi A, Dei Tos AP, Picci P, Donati D (2017) Histology and grading are important prognostic factors in synovial sarcoma. Eur J Surg Oncol. doi:10.1016/j.ejso.2017.05.020

    Google Scholar 

  32. Spunt SL, Poquette CA, Hurt YS, Cain AM, Rao BN, Merchant TE, Jenkins JJ, Santana VM, Pratt CB, Pappo AS (1999) Prognostic factors for children and adolescents with surgically resected nonrhabdomyosarcoma soft tissue sarcoma: an analysis of 121 patients treated at St Jude Children’s Research Hospital. J Clin Oncol 17(12):3697–3705. doi:10.1200/JCO.1999.17.12.3697

    Article  CAS  PubMed  Google Scholar 

  33. Hayes-Jordan AA, Spunt SL, Poquette CA, Cain AM, Rao BN, Pappo AS, Shochat SJ (2000) Nonrhabdomyosarcoma soft tissue sarcomas in children: is age at diagnosis an important variable? J Pediatr Surg 35(6):948–953. doi:10.1053/jpsu.2000.6934 (discussion 953–944)

    Article  CAS  PubMed  Google Scholar 

  34. Smith MA, Altekruse SF, Adamson PC, Reaman GH, Seibel NL (2014) Declining childhood and adolescent cancer mortality. Cancer 120(16):2497–2506. doi:10.1002/cncr.28748

    Article  PubMed  PubMed Central  Google Scholar 

  35. Pisters PW, Leung DH, Woodruff J, Shi W, Brennan MF (1996) Analysis of prognostic factors in 1,041 patients with localized soft tissue sarcomas of the extremities. J Clin Oncol 14(5):1679–1689. doi:10.1200/JCO.1996.14.5.1679

    Article  CAS  PubMed  Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Istituto Ortopedico Rizzoli, Bologna, Italy

    Andrea Sambri, Giuseppe Bianchi, Ilaria Cucurnia, Marco Gambarotti & Davide Maria Donati

Authors
  1. Andrea Sambri
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Giuseppe Bianchi
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Ilaria Cucurnia
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. Marco Gambarotti
    View author publications

    You can also search for this author in PubMed Google Scholar

  5. Davide Maria Donati
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Andrea Sambri.

Ethics declarations

Conflict of interest

All the authors declare that they have no conflict of interest. This study complies with the current laws of the country in which it was conducted.

Ethical standard

The study was approved by the local Ethic committee.

Informed consent

All patients signed an informed consent.

Rights and permissions

Reprints and permissions

About this article

Check for updates. Verify currency and authenticity via CrossMark

Cite this article

Sambri, A., Bianchi, G., Cucurnia, I. et al. Pediatric soft tissue sarcoma of the limbs: clinical outcome of 97 patients. Eur J Orthop Surg Traumatol 28, 1–7 (2018). https://doi.org/10.1007/s00590-017-2019-4

Download citation

  • Received:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s00590-017-2019-4

Keywords

Search

Navigation