Abstract
Pediatric soft tissue sarcomas (STS) are a heterogeneous group of malignant tumors constituting about 7% of all cancer cases. Rhabdomyosarcomas (RMS) constitute about half of all soft tissue sarcomas in children, the rest being constituted by non- rhabdomyosarcoma soft tissue sarcomas (NRSTS). Most RMS present in young children <6 y of age while the NRSTS occur in adolescents and young adults. The latter constitute a diverse group of tumors and are rare in children. The STS generally present as painless enlarging mass or with symptoms of compression/infiltration of adjacent organs or structures. Staging, risk stratification and multidisciplinary approach are needed for the treatment of STS and outcome depends on stage, site and histological type. Treatment of RMS has evolved systematically through various clinical trials. Chemotherapy remains the backbone of treatment for RMS and local control is achieved either with surgery or radiotherapy or both. Management of NRSTS is still a challenge as it is generally chemotherapy-resistant and surgery remains the mainstay of treatment. Outcome therefore depends on whether wide local excision with negative margins is possible. Local radiotherapy is reserved for recurrent, residual and large high grade NRST. The prognosis of metastatic as well as recurrent STS remains dismal.
Similar content being viewed by others
References
Consolidated Report of Population Based Cancer Registries 2001–2001. National Cancer Registry Programme, Indian Council Medical Research, Bangalore, India, Dec 2006. Available from : http://www.icmr.nic.in/ncrp/report_pop_2001-04/cancer_p_based.htm
First Report of the Population Based Cancer Registries under North Eastern Regional Cancer Registry 2003–2004. National Cancer Registry Programme, Indian Council of Medical Research, Bangalore, India, Sep 2006. Available from: http://www.icmr.nic.in/ncrp/first_report_2003-04/first_report.htm.
Jemal A, Siegel R, Ward E, et al. Cancer Statistics, 2008. CA Cancer J Clin. 2008;58:71–96.
Gurney JG, Young JL Jr, Roffers SD, et al. Soft tissue sarcomas. In: Ries LAG, Smith MA, Gurney JG, et al.eds. Cancer incidence and survival among children and adolescents: United States SEER Program 1975–1995. NIH Pub. No. 99–4649. Bethesda, MD: National Cancer Institute, SEER Program 1999:pp111.
Ries LA, Smith MA, Gurney J, et al. Cancer incidence and survival among children and adolescents: United States SEER Program 1975–1995. Bethesda, MD: National Cancer Institute, 1999. SEER Program Pub No.99-4649.
McClain KL, Leach CT, Jenson HB, et al. Association of Epstein-Barr virus with leiomyosarcomas in children with AIDS. N Engl J Med. 1995;332:12–8.
Steliarova-Foucher E, Stiller C, Lacour B, Kaatsch P. International Classification of Childhood Cancer, third edition. Cancer 2005;103:1457–67.
Mihara S, Matsumoto H, Tokunaga F, Yano H, Ota M, Yamashita S. Botryoid rhabdomyosarcoma of the gallbladder in a child. Cancer. 1982;49:812–8.
Kedar A, Cantrel G, Rosen G. Rhabdomyosarcoma of the trachea. J Laryngol Otol. 1988;102:735–6.
Schmaltz AA, Apitz J. Primary rhabdomyosarcoma of the heart. Pediatr Cardiol. 1982;2:73–5.
Ferrari A, Casanova M, Collini P, et al. Adult-type soft tissue sarcomas in pediatric-age patients: experience at the Istituto Nazionale Tumori in Milan. J Clin Oncol. 2005;23:4021–30.
Fong Y, Coit DG, Woodruff JM, Brennan MF. Lymph node metastasis from soft tissue sarcoma in adult. Analysis of data from a prospective database of 1772 sarcoma patients. Ann Surg. 1993;217:72–7.
Rikhof B, de Jong S, Suurmeijer AJ, Meijer C, van der Graaf WT. The insulin-like growth factor system and sarcomas. J Pathol. 2009;217:469–82.
Hanukoglu A, Chalew SA, Sun CJ, Dorfman GD, Bright RW. Surgically curable hypophosphatemic rickets. Diagnosis and management. Clin Pediatr. 1989;28:321–5.
Parham DM, Webber BL, Jenkins 3rd JJ, Cantor AB, Maurer HM. Nonrhabdomyosarcomatous soft tissue sarcomas of childhood: formulation of a simplified system for grading. Mod Pathol. 1995;8:705–10.
Crist W, Gehan EA, Ragab AH, et al. The third intergroup rhabdomyosarcoma study. J Clin Oncol. 1995;13:610–30.
Crist WM, Garnsey L, Beltangady MS, et al. Prognosis in children with rhabdomyosarcoma: a report of the intergroup rhabdomyosarcoma studies I and II. Intergroup Rhabdomyosarcoma Committee. J Clin Oncol. 1990;8:443–52.
Lawrence W, Gehan E, Hays D, Beltangady M, Maurer H. Prognostic significance of staging factors of the UICC staging system in childhood rhabdomyosarcoma: a report from the Intergroup Rhabdomyosarcoma Study (IRS II). J Clin Oncol. 1987;5:46–54.
Lawrence Jr W, Anderson JR, Gehan EA, Maurer H. Pre-treatment TNM staging of childhood rhabdomyosarcoma. A report of the Intergroup Rhabdomyosarcoma Study Group. Cancer. 1997;80:1165–70.
Gehan EA, Glover FN, Maurer HM, et al. Prognostic factors in children with rhabdomyosarcoma. Natl Cancer Inst Monogr. 1981;56:83–92.
Greene FL, Page DL, Fleming ID, et al. AJCC staging manual. 6th ed. New York: Springer; 2002.
Hajdu SI, Shiu MH, Brennan MF. The role of the pathologist in the management of soft tissue sarcomas. World J Surg. 1988;12:326–31.
Enneking WF, Spanier SS, Goodman MA. A system for the surgical staging of musculoskeletal sarcoma. Clin Orthop Relat Res. 1980;153:106–20.
Maurer HM, Beltangady M, Gehan EA, et al. The intergroup rhabdomyosarcoma study-I. A final report Cancer. 1988;61:209–20.
Maurer HM, Gehan EA, Beltangady M, et al. The intergroup rhabdomyosarcoma study-II. Cancer. 1993;71:1904–22.
Arndt C, Tefft M, Gehan E, et al. A feasibility, toxicity, and early response study of etoposide, ifosfamide, and vincristine for the treatment of children withrhabdomyosarcoma: a report from the Intergroup Rhabdomyosarcoma Study (IRS) IV pilot study. J Pediatr Hematol Oncol. 1997;19:124–9.
Ruymann FB, Vietti T, Gehan E, et al. Cyclophosphamide dose escalation in combination with vincristine and actinomycin-D (VAC) in gross residual sarcoma. A pilot study without hematopoietic growth factor support evaluating toxicity and response. J Pediatr Hematol Oncol. 1995;17:331–7.
Cecchetto G, Carli M, Sotti G, et al. Importance of local treatment in pediatric soft tissue sarcomas with microscopic residual after primary surgery: results of the Italian Cooperative Study RMS-88. Med Pediatr Oncol. 2000;34:97–101.
Koscielniak E, Klingebiel TH, Peters C, et al. Do patients with metastatic and recurrent rhabdomyosarcoma benefit from high-dose therapy with hematopoietic rescue? Report of the German/Austrian Pediatric Bone Marrow Transplantation Group. Bone Marrow Transplant. 1997;19:227–31.
Stevens MC, Rey A, Bouvet N, et al. Treatment of nonmetastatic rhabdomyosarcomas in childhood and adolescence: third study of the International Society of Paediatric Oncology-SIOP Malignant Mesenchymal Tumor 89. J Clin Oncol. 2005;23:2618–28.
Koscielniak E, Harms D, Henze G, et al. Results of treatment for soft tissue sarcoma in childhood and adolescence: a final report of the German Cooperative Soft Tissue Sarcoma Study CWS-86. J Clin Oncol. 1999;17:3706–19.
Ferrari A, Casanova M. Current chemotherapeutic strategies for rhabdomyosarcoma. Expert Rev Anticancer Ther. 2005;5:283–94.
Pappo AS, Anderson JR, Crist WM, et al. Survival after relapse in children and adolescents with rhabdomyosarcoma: a report from the Intergroup Rhabdomyosarcoma Study Group. J Clin Oncol. 1999;17:3487–93.
Mazzoleni S, Bisogno G, Garaventa A, et al. Outcomes and prognostic factors after recurrence in children and adolescents with nonmetastatic rhabdomyosarcoma. Cancer. 2005;104:183–90.
Mattke AC, Bailey EJ, Schuck A, et al. Does the time-point of relapse influence outcome in pediatric rhabdomyosarcomas? Pediatr Blood Cancer. 2009;52:772–6.
Van Winkle P, Angiolillo A, Krailo M, et al. Ifosfamide, carboplatin, and etoposide (ICE) reinduction chemotherapy in a large cohort of children and adolescents with recurrent/refractory sarcoma: the Children’s Cancer Group (CCG) experience. Pediatr Blood Cancer. 2005;44:338–47.
Cosetti M, Wexler LH, Calleja E, et al. Irinotecan for pediatric solid tumors: the Memorial Sloan-Kettering experience. J Pediatr Hematol Oncol. 2002;24:101–5.
Pappo AS, Lyden E, Breitfeld P, et al. Two consecutive phase II window trials of irinotecan alone or in combination with vincristine for the treatment of metastatic rhabdomyosarcoma: the Children’s Oncology Group. J Clin Oncol. 2007;25:362–9.
Pappo AS, Devidas M, Jenkins J, et al. Phase II trial of neoadjuvant vincristine, ifosfamide and doxorubicin with granulocyte colony-stimulating factor support in children and adolescents with advanced-stage nonrhabdomyosarcomatous soft tissue sarcomas: a Pediatric Oncology Group Study. J Clin Oncol. 2005;23:4031–8.
Bramwell VH, Mouridsen HT, Santoro A, et al. Cyclophosphamide versus ifosfamide: a randomized phase II trial in adult soft-tissue sarcomas. The European Organization for Research and Treatment of Cancer [EORTC], Soft Tissue and Bone Sarcoma Group. Cancer Chemother Pharmacol. 1993;31(2):S180–4.
Bramwell V, Quirt I, Warr D, et al. Combination chemotherapy with doxorubicin, dacarbazine, and ifosfamide in advanced adult soft tissue sarcoma. Canadian Sarcoma Group-National Cancer Institute of Canada Clinical Trials Group. J Natl Cancer Inst. 1989;81:1496–9.
Edmonson JH, Ryan LM, Blum RH, et al. Randomized comparison of doxorubicin alone versus ifosfamide plus doxorubicin or mitomycin, doxorubicin, and cisplatin against advanced soft tissue sarcomas. J Clin Oncol. 1993;11:1269–75.
Author information
Authors and Affiliations
Corresponding author
Rights and permissions
About this article
Cite this article
Kapoor, G., Das, K. Soft Tissue Sarcomas in Children. Indian J Pediatr 79, 936–942 (2012). https://doi.org/10.1007/s12098-011-0560-4
Received:
Accepted:
Published:
Issue Date:
DOI: https://doi.org/10.1007/s12098-011-0560-4