Further Outpatient Care

Frequent outpatient visits are required to monitor the effects and adverse effects of therapy. CBC counts should be assessed once or twice a week while patients are receiving granulocyte-colony stimulating factor (G-CSF) therapy. Periodically monitoring the toxic effects of chemotherapy on liver and renal function is required with serum chemistry levels. Supportive treatment with blood products, including packed RBCs and platelets, may be necessary if patients are given chemotherapy

Long-term follow-up is required after therapy is completed to monitor the following:

Disease recurrence
Development of a secondary malignancy, particularly if the patient received radiation therapy and/or chemotherapy with etoposide
Growth and development
Cardiac function in children who received anthracycline chemotherapy (Perform annual ECG and echocardiography.)

Further Inpatient Care

Inpatient care of patients with nonrhabdomyosarcoma soft tissue sarcomas (NRSTS) involves admissions for chemotherapy and for supportive care related to therapy.

Patients must be hospitalized to manage episodes of fever and neutropenia and to start broad-spectrum antibiotic coverage while culture results or recovery from illness are awaited.

Complications

The long-term effects of NRSTS treatment in children are many.

Surgical treatments may cause marked permanent functional deficits, depending on the location and extent of surgery.

The use of chemotherapy has been associated with numerous late effects. Doxorubicin can cause life-threatening cardiomyopathy. Ifosfamide can cause permanent renal impairment, hemorrhagic cystitis, infertility, gonadal failure, and an increased risk of secondary malignancies. Radiation therapy can also have lifelong debilitating effects. The effects of radiation depend on the location irradiated and the amount of radiation given to the site. Common effects can include growth plate arrest, pulmonary fibrosis, development of muscle fibrosis and contractures, and development of a secondary cancer in the radiation field.

Prognosis

Prognostic factors for children with NRSTS include the presence of metastatic disease, ability to achieve local control, tumor size and invasiveness, and tumor histologic grade.

According to IRS groups, survival rates are as follows:

Group I - 82%
Group II - 67%
Group III - 12%
Group IV - 5

Tumor, node, and metastases (TNM) stage 4 ( the presence of metastasis) correlates with an extremely poor prognosis of (< 20%).

Many factors contribute to survival, but the degree of resectability at the time of diagnosis is the most important factor identified to date.

Questions

Hayes-Jordan A. Recent advances in non-rhabdomyosarcoma soft-tissue sarcomas. Semin Pediatr Surg. 2012 Feb. 21(1):61-7. [QxMD MEDLINE Link].
Loh ML, Ahn P, Perez-Atayde AR, et al. Treatment of infantile fibrosarcoma with chemotherapy and surgery: results from the Dana-Farber Cancer Institute and Children's Hospital, Boston. J Pediatr Hematol Oncol. 2002. 24 (9):722-6. [QxMD MEDLINE Link].
Drilon A, Laetsch TW, Kummar S, et al. Efficacy of Larotrectinib in TRK Fusion-Positive Cancers in Adults and Children. N Engl J Med. 2018 Feb 22. 378 (8):731-739. [QxMD MEDLINE Link].
Laetsch TW, DuBois SG, Mascarenhas L, Turpin B, Federman N, Albert CM, et al. Larotrectinib for paediatric solid tumours harbouring NTRK gene fusions: phase 1 results from a multicentre, open-label, phase 1/2 study. Lancet Oncol. 2018 May. 19 (5):705-714. [QxMD MEDLINE Link].
McArthur GA, Demetri GD, van Oosterom A, Heinrich MC, Debiec-Rychter M, Corless CL. Molecular and clinical analysis of locally advanced dermatofibrosarcoma protuberans treated with imatinib: Imatinib Target Exploration Consortium Study B2225. J Clin Oncol. 2005 Feb 1. 23(4):866-73. [QxMD MEDLINE Link].
Ferrari A, Chi YY, De Salvo GL, Orbach D, Brennan B, Randall RL, et al. 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. 2017 Jun. 78:1-6. [QxMD MEDLINE Link].
Brecht IB, Ferrari A, Int-Veen C, et al. Grossly-resected synovial sarcoma treated by the German and Italian Pediatric Soft Tissue Sarcoma Cooperative Groups: discussion on the role of adjuvant therapies. Pediatric Blood and Cancer. 2006. 47:11-17. [QxMD MEDLINE Link]. [Full Text].
Okcu MF, Munsell M, Treuner J, et al. Synovial sarcoma of childhood and adolescence: a multicenter, multivariate analysis of outcome. J Clin Oncol. 2003. 21:1602-11. [QxMD MEDLINE Link].
McCarville MB, Muzzafar S, Kao SC, Coffin CM, Parham DM, Anderson JR, et al. Imaging features of alveolar soft-part sarcoma: a report from Children's Oncology Group Study ARST0332. AJR Am J Roentgenol. 2014 Dec. 203(6):1345-52. [QxMD MEDLINE Link].
Faulkner LB, Hajdu SI, Kher U, et al. Pediatric desmoid tumor: retrospective analysis of 63 cases. J Clin Oncol. 1995. 13:2813-8. [QxMD MEDLINE Link].
Brooks MD, Ebbs SR, Colletta AA, Baum M. Desmoid tumours treated with triphenylethylenes. Eur J Cancer. 1992. 28A (6-7):1014-8. [QxMD MEDLINE Link].
Meazza C, Casanova M, Trecate G, Ferrari A. Objective response to hydroxyurea in a patient with heavily pre-treated aggressive fibromatosis. Pediatr Blood Cancer. 2010 Sep. 55 (3):587-8. [QxMD MEDLINE Link].
Heinrich MC, McArthur GA, Demetri GD, Joensuu H, Bono P, Herrmann R, et al. Clinical and molecular studies of the effect of imatinib on advanced aggressive fibromatosis (desmoid tumor). J Clin Oncol. 2006 Mar 1. 24 (7):1195-203. [QxMD MEDLINE Link].
Chugh R, Wathen JK, Patel SR, Maki RG, Meyers PA, Schuetze SM, et al. Efficacy of imatinib in aggressive fibromatosis: Results of a phase II multicenter Sarcoma Alliance for Research through Collaboration (SARC) trial. Clin Cancer Res. 2010 Oct 1. 16 (19):4884-91. [QxMD MEDLINE Link].
Penel N, Le Cesne A, Bui BN, Perol D, Brain EG, Ray-Coquard I, et al. Imatinib for progressive and recurrent aggressive fibromatosis (desmoid tumors): an FNCLCC/French Sarcoma Group phase II trial with a long-term follow-up. Ann Oncol. 2011 Feb. 22 (2):452-7. [QxMD MEDLINE Link].
Kasper B, Gruenwald V, Reichardt P, Bauer S, Rauch G, Limprecht R, et al. Imatinib induces sustained progression arrest in RECIST progressive desmoid tumours: Final results of a phase II study of the German Interdisciplinary Sarcoma Group (GISG). Eur J Cancer. 2017 May. 76:60-67. [QxMD MEDLINE Link].
Gounder MM, Lefkowitz RA, Keohan ML, D'Adamo DR, Hameed M, Antonescu CR, et al. Activity of Sorafenib against desmoid tumor/deep fibromatosis. Clin Cancer Res. 2011 Jun 15. 17 (12):4082-90. [QxMD MEDLINE Link].
National Cancer Institute (NCI). Sorabenib Tosylate in Treating Patients With Desmoid Tumors or Aggressive Fibromatosis. NLM identifier: NCT02066181. Available at https://clinicaltrials.gov/ct2/show/study/NCT02066181.
Kummar S, O'Sullivan Coyne G, Do KT, Turkbey B, Meltzer PS, Polley E, et al. Clinical Activity of the γ-Secretase Inhibitor PF-03084014 in Adults With Desmoid Tumors (Aggressive Fibromatosis). J Clin Oncol. 2017 May 10. 35 (14):1561-1569. [QxMD MEDLINE Link].
Messersmith WA, Shapiro GI, Cleary JM, Jimeno A, Dasari A, Huang B, et al. A Phase I, dose-finding study in patients with advanced solid malignancies of the oral γ-secretase inhibitor PF-03084014. Clin Cancer Res. 2015 Jan 1. 21 (1):60-7. [QxMD MEDLINE Link].
Skapek SX, Ferguson WS, Granowetter L, et al. Vinblastine and methotrexate for desmoid fibromatosis in children: results of a Pediatric Oncology Group Phase II Trial. J Clin Oncol. 2007. 25 (5):501-6. [QxMD MEDLINE Link].
Patel SR, Evans HL, Benjamin RS. Combination chemotherapy in adult desmoid tumors. Cancer. 1993 Dec 1. 72 (11):3244-7. [QxMD MEDLINE Link].
Yamamoto H, Oshiro R, Nishimura J, Uemura M, Haraguchi N, Hata T, et al. Low-dose dacarbazine-doxorubicin therapy against intra-abdominal desmoid tumors. Oncol Rep. 2013 May. 29 (5):1751-5. [QxMD MEDLINE Link].
Daller JA, Bueno J, Gutierrez J, et al. Hepatic hemangioendothelioma: clinical experience and management strategy. J Pediatr Surg. 1999. 34:98-105. [QxMD MEDLINE Link].
Randall RL, Albritton KH, Ferney BJ, Layfield L. Malignant fibrous histiocytoma of soft tissue: an abandoned diagnosis. Am J Orthop (Belle Mead NJ). 2004 Dec. 33(12):602-8. [QxMD MEDLINE Link].
Erlandson RA, Antonescu CR. The rise and fall of malignant fibrous histiocytoma. Ultrastruct Pathol. 2004 Sep-Dec. 28(5-6):283-9. [QxMD MEDLINE Link].
Guillou L, Wadden C, Coindre JM, Krausz T, Fletcher CD. "Proximal-type" epithelioid sarcoma, a distinctive aggressive neoplasm showing rhabdoid features. Clinicopathologic, immunohistochemical, and ultrastructural study of a series. Am J Surg Pathol. 1997 Feb. 21 (2):130-46. [QxMD MEDLINE Link].
Hasegawa T, Matsuno Y, Shimoda T, Umeda T, Yokoyama R, Hirohashi S. Proximal-type epithelioid sarcoma: a clinicopathologic study of 20 cases. Mod Pathol. 2001 Jul. 14 (7):655-63. [QxMD MEDLINE Link].
Callister MD, Ballo MT, Pisters PW, Patel SR, Feig BW, Pollock RE, et al. Epithelioid sarcoma: results of conservative surgery and radiotherapy. Int J Radiat Oncol Biol Phys. 2001 Oct 1. 51 (2):384-91. [QxMD MEDLINE Link].
Baratti D, Pennacchioli E, Casali PG, Bertulli R, Lozza L, Olmi P, et al. Epithelioid sarcoma: prognostic factors and survival in a series of patients treated at a single institution. Ann Surg Oncol. 2007 Dec. 14 (12):3542-51. [QxMD MEDLINE Link].
Wolf PS, Flum DR, Tanas MR, Rubin BP, Mann GN. Epithelioid sarcoma: the University of Washington experience. Am J Surg. 2008 Sep. 196 (3):407-12. [QxMD MEDLINE Link].
Jawad MU, Extein J, Min ES, Scully SP. Prognostic factors for survival in patients with epithelioid sarcoma: 441 cases from the SEER database. Clin Orthop Relat Res. 2009 Nov. 467 (11):2939-48. [QxMD MEDLINE Link].
Brennan B, De Salvo GL, Orbach D, De Paoli A, Kelsey A, Mudry P, et al. Outcome of extracranial malignant rhabdoid tumours in children registered in the European Paediatric Soft Tissue Sarcoma Study Group Non-Rhabdomyosarcoma Soft Tissue Sarcoma 2005 Study-EpSSG NRSTS 2005. Eur J Cancer. 2016 Jun. 60:69-82. [QxMD MEDLINE Link].
Thway K, Jones RL, Noujaim J, Fisher C. Epithelioid Sarcoma: Diagnostic Features and Genetics. Adv Anat Pathol. 2016 Jan. 23 (1):41-9. [QxMD MEDLINE Link].
Chi S, Fouladi M, Shukla N, et al. Phase 1 study of the EZH2 inhibitor, tazemetostat, in children with relapsed or refractory INI1-negative tumors including rhabdoid tumors, epithelioid sarcoma, chordoma; and synovial sarcoma. Proceedings from the 2018 ASPHO Conference; May 2-5, 2018. Abstract 2077.
Epizyme, Inc. A Phase 1 Study of the EZH2 Inhibitor Tazemetostat in Pediatric Subjects With Relapsed or Refractory INI1-Negative Tumors or Synovial Sarcoma. NLM identifier: NCT0260193. Available at https://clinicaltrials.gov/ct2/show/study/NCT02601937.
Gounder MM, Stacchiotti S, Schöffski P, et al. Phase 2 multicenter study of the EZH2 inhibitor tazemetostat in adults with INI1 negative epithelioid sarcoma (NCT02601950). Journal of Clinical Oncology. 2017. 35(15):S:11058.
Epizyme, Inc. A Phase II, Multicenter Study of the EZH2 Inhibitor Tazemetostat in Adult Subjects With INI1-Negative Tumors or Relapsed/Refractory Synovial Sarcoma. NLM identifier: NCT02601950. Available at https://clinicaltrials.gov/ct2/show/NCT02601950.
Italiano A, Soria JC, Toulmonde M, Michot JM, Lucchesi C, Varga A, et al. Tazemetostat, an EZH2 inhibitor, in relapsed or refractory B-cell non-Hodgkin lymphoma and advanced solid tumours: a first-in-human, open-label, phase 1 study. Lancet Oncol. 2018 May. 19 (5):649-659. [QxMD MEDLINE Link].
Casanova M, Ferrari A, Collini P, Bisogno G, Alaggio R, Cecchetto G, et al. Epithelioid sarcoma in children and adolescents: a report from the Italian Soft Tissue Sarcoma Committee. Cancer. 2006 Feb 1. 106 (3):708-17. [QxMD MEDLINE Link].
Jackson EM, Sievert AJ, Gai X, Hakonarson H, Judkins AR, Tooke L, et al. Genomic analysis using high-density single nucleotide polymorphism-based oligonucleotide arrays and multiplex ligation-dependent probe amplification provides a comprehensive analysis of INI1/SMARCB1 in malignant rhabdoid tumors. Clin Cancer Res. 2009 Mar 15. 15 (6):1923-30. [QxMD MEDLINE Link].
Biegel JA, Zhou JY, Rorke LB, Stenstrom C, Wainwright LM, Fogelgren B. Germ-line and acquired mutations of INI1 in atypical teratoid and rhabdoid tumors. Cancer Res. 1999 Jan 1. 59 (1):74-9. [QxMD MEDLINE Link].
Eaton KW, Tooke LS, Wainwright LM, Judkins AR, Biegel JA. Spectrum of SMARCB1/INI1 mutations in familial and sporadic rhabdoid tumors. Pediatr Blood Cancer. 2011 Jan. 56 (1):7-15. [QxMD MEDLINE Link].
Brennan B, Stiller C, Bourdeaut F. Extracranial rhabdoid tumours: what we have learned so far and future directions. Lancet Oncol. 2013 Jul. 14 (8):e329-36. [QxMD MEDLINE Link].
Guzzetta AA, Montgomery EA, Lyu H, Hooker CM, Meyer CF, Loeb DM, et al. Epithelioid sarcoma: one institution's experience with a rare sarcoma. J Surg Res. 2012 Sep. 177 (1):116-22. [QxMD MEDLINE Link].
Federman N, Feig SA. PET/CT in evaluating pediatric malignancies: a clinician's perspective. J Nucl Med. 2007 Dec. 48(12):1920-2. [QxMD MEDLINE Link].
McCarville MB, Christie R, Daw NC, et al. PET/CT in the evaluation of childhood sarcomas. AJR Am J Roentgenol. 2005 Apr. 184(4):1293-304. [QxMD MEDLINE Link].
Mitton B, Seeger LL, Eckardt MA, Motamedi K, Eilber FC, Nelson SD, et al. Image-guided percutaneous core needle biopsy of musculoskeletal tumors in children. J Pediatr Hematol Oncol. 2014 Jul. 36 (5):337-41. [QxMD MEDLINE Link].
American Joint Committee on Cancer. AJCC Cancer Staging Manual. 6th. New York, NY: Springer; 2002.
Spunt S, Million L, Anderson J, et al. Risk-based treatment for nonrhabdomyosarcoma soft tissue sarcomas (NRSTS) in patients under 30 years of age: Children’s Oncology Group study ARST0332. J Clin Oncol. 2014. 32:S15.
Million L, Donaldson SS. Resectable pediatric nonrhabdomyosarcoma soft tissue sarcoma: which patients benefit from adjuvant radiation therapy and how much?. ISRN Oncol. 2012. 2012:341408. [QxMD MEDLINE Link]. [Full Text].
Smith KB, Indelicato DJ, Knapik JA, Lagmay JP, Morris C, Kirwan JM, et al. Adjuvant radiotherapy for pediatric and young adult nonrhabdomyosarcoma soft-tissue sarcoma. Int J Radiat Oncol Biol Phys. 2011 Sep 1. 81(1):150-7. [QxMD MEDLINE Link].
Smith KB, Indelicato DJ, Knapik JA, Morris C, Kirwan J, Zlotecki RA, et al. Definitive radiotherapy for unresectable pediatric and young adult nonrhabdomyosarcoma soft tissue sarcoma. Pediatr Blood Cancer. 2011 Aug. 57(2):247-51. [QxMD MEDLINE Link].
Krasin MJ, Davidoff AM, Spunt SL, et al. Preliminary results from a prospective study using limited margin radiotherapy in pediatric and young adult patients with high-grade nonrhabdomyosarcoma soft-tissue sarcoma. Int J Radiat Oncol Biol Phys. Mar 2010. 76 (3):874-8. [QxMD MEDLINE Link].
Tinkle CL, Fernandez-Pineda I, Sykes A, Lu Z, Hua CH, Neel MD, et al. Nonrhabdomyosarcoma soft tissue sarcoma (NRSTS) in pediatric and young adult patients: Results from a prospective study using limited-margin radiotherapy. Cancer. 2017 Nov 15. 123 (22):4419-4429. [QxMD MEDLINE Link].
Waxweiler TV, Rusthoven CG, Proper MS, Cost CR, Cost NG, Donaldson N, et al. Non-Rhabdomyosarcoma Soft Tissue Sarcomas in Children: A Surveillance, Epidemiology, and End Results Analysis Validating COG Risk Stratifications. Int J Radiat Oncol Biol Phys. 2015 Jun 1. 92 (2):339-48. [QxMD MEDLINE Link].
National Cancer Institute (NCI). Radiation Therapy With or Without Combination Chemotherapy or Pazopanib Hydrochloride Before Surgery in Treating Patients With Newly Diagnosed Non-rhabdomyosarcoma Soft Tissue Sarcomas That Can Be Removed by Surgery. NLM identifier: NCT02180867. Available at https://clinicaltrials.gov/ct2/show/NCT02180867.
Sangkhathat S. Current management of pediatric soft tissue sarcomas. World J Clin Pediatr. 2015 Nov 8. 4 (4):94-105. [QxMD MEDLINE Link].
Tecentriq (atezolizumab) [package insert]. South San Francisco, CA: Genentech, Inc. December 2022. Available at [Full Text].
Sleijfer S, Ray-Coquard I, Papai Z, et al. Pazopanib, a multikinase angiogenesis inhibitor, in patients with relapsed or refractory advanced soft tissue sarcoma: a phase II study from the European organisation for research and treatment of cancer- soft tissue and bone sarcoma group (EORTC study 62043). J Clin Oncol. 2009. 27 (19):3126-32. [QxMD MEDLINE Link].
Tazverik (tazemetostat) [package insert]. Cambridge, MA: Epizyme, Inc. January 2020. Available at [Full Text].
Eli Lilly and Company. Lilly Reports Results of Phase 3 Soft Tissue Sarcoma Study of LARTRUVO. PR Newswire. January 18, 2019. Available at https://www.prnewswire.com/news-releases/lilly-reports-results-of-phase-3-soft-tissue-sarcoma-study-of-lartruvo-300780704.html.
Stacchiotti S, Tamborini E, Marrari A, et al. Response to sunitinib malate in advanced alveolar soft part sarcoma. Clin Cancer Res. 2009. 15 (3):1096-104. [QxMD MEDLINE Link].
Putnam JB Jr, Roth JA. Surgical treatment for pulmonary metastases from sarcoma. Hematol Oncol Clin North Am. 1995. 9 (4):869-87. [QxMD MEDLINE Link].
Eli Lilly and Company. A Study of Olaratumab Alone and in Combination With Standard Chemotherapies in Children With Cancer. NLM identifier: NCT02677116. Available at https://clinicaltrials.gov/ct2/show/NCT02677116..
Albritton KH. Sarcomas in adolescents and young adults. Hematol Oncol Clin North Am. 2005. 19:527-546. [QxMD MEDLINE Link]. [Full Text].
Antonescu CR. The role of genetic testing in soft tissue sarcoma. Histopathology. 2006 Jan. 48(1):13-21. [QxMD MEDLINE Link].
Blakely ML, Spurbeck WW, Pappo AS, et al. The impact of margin of resection on outcome in pediatric nonrhabdomyosarcoma soft tissue sarcoma. J Pediatr Surg. 1999 May. 34(5):672-5. [QxMD MEDLINE Link].
Dillon PW. Nonrhabdomyosarcoma soft tissue sarcomas in children. Seminars in Pediatric Surgery. 1997. 6(1):24-28. [QxMD MEDLINE Link].
Kayton ML, Meyers P, Wexler LH, et al. Clinical presentation, treatment, and outcome of alveolar soft part sarcoma in children, adolescents, and young adults. J Pediatr Surg. 2006. 41:187-193. [QxMD MEDLINE Link].
Lanzkowsky P. Rhabdomyosarcoma and other soft tissue sarcomas. Manual of Pediatric Hematology and Oncology. 4th ed. San Diego, Calif: Academic; 2005: 561-584.
Loeb DM, Thornton K, Shokek O. Pediatric soft tissue sarcomas. Surg Clin North Am. 2008 Jun. 88(3):615-27, vii. [QxMD MEDLINE Link].
Marcus KC, Grier HE, Shamberger RC, et al. Childhood soft tissue sarcoma: a 20-year experience. J Pediatr. 1997 Oct. 131(4):603-7. [QxMD MEDLINE Link].
Marcus RB Jr. Current controversies in pediatric radiation oncology. Orthop Clin North Am. 1996 Jul. 27(3):551-7. [QxMD MEDLINE Link].
Meyer WH, Spunt SL. Soft tissue sarcomas of childhood. Cancer Treat Rev. 2004. 30:269-80. [QxMD MEDLINE Link]. [Full Text].
Okeu M, Hicks J, Merchant TE, Andrassy RJ, Pappo AS, Horowitz ME. Non-rhabdomyosarcomatous soft tissue sarcomas. Pizzo PA, Poplack DG. Principles and Practice of Pediatric Oncology. 5th. Philadelphia: Lippincott Williams and Wilkins; 2006. 1034-1073.
Pappo AS. Rhabdomyosarcoma and other soft tissue sarcomas in children. Curr Opin Oncol. 1996 Jul. 8(4):311-6. [QxMD MEDLINE Link].
Paulides M, Kremers A, Stohr W, Bielack S, Jurgens H, Treuner J. Prospective longitudinal evaluation of doxorubicin-induced cardiomyopathy in sarcoma patients: a report of the late effects surveillance system (LESS). Pediatr Blood Cancer. 2006 Apr. 46(4):489-95. [QxMD MEDLINE Link].
Paulino AC. Treatment options for children with nonrhabdomyosarcoma soft tissue sarcoma. Expert Rev Anticancer Ther. 2004. 4:247-56. [QxMD MEDLINE Link].
Sirvent N, Maire G, Pedeutour F. Genetics of dermatofibrosarcoma protuberans family of tumors: from ring chromosomes to tyrosine kinase inhibitor treatment. Genes Chromosomes Cancer. 2003 May. 37(1):1-19. [QxMD MEDLINE Link].
Smith KB, Indelicato DJ, Knapik JA, Morris C, Kirwan J, Zlotecki RA, et al. Definitive radiotherapy for unresectable pediatric and young adult nonrhabdomyosarcoma soft tissue sarcoma. Pediatr Blood Cancer. 2011 Aug. 57(2):247-51. [QxMD MEDLINE Link].
Spunt S, Wolden S, Schofield D, Skapek S. Non-Rhabdomyosarcoma soft tissue sarcomas. Pappo, Alberto. Pediatric Bone and Soft Tissue Sarcomas. New York: Springer; 2006. 133-162.
Spunt SL, Poquette CA, Hurt YS, et al. 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. 1999 Dec. 17(12):3697-705. [QxMD MEDLINE Link].
Spunt SL, Skapek SX, Coffin CM. Pediatric nonrhabdomyosarcoma soft tissue sarcomas. Oncologist. 2008 Jun. 13(6):668-78. [QxMD MEDLINE Link].
Womer RB, Sinniah D. Soft tissue sarcomas. Practical Pediatric Oncology. 1992. 318-23.

Media Gallery

COG-ARST0332 experimental design schema.

of 1

Table. American Joint Commission for Cancer Staging (Tumor, Node, and Metastases [TNM] System)

	Primary Tumor	Regional Lymph Nodes	Distant Metastasis	Histologic Grade
Stage I	Any tumor size, superficial or deep	N0	M0	G1 or G2
Stage II	T1a (tumor < 5 cm, superficial)	N0	M0	G3
	T1b (tumor < 5 cm, deep)	N0	M0	G3
	T2a (tumor >5 cm, superficial)	N0	M0	G3
Stage III	T2b (tumor >5 cm, deep)	N0	M0	G3
Stage IV	Any tumor size, superficial or deep	N1	M0 or M1	G1, G2, or G3
Stage IV	Any tumor size, superficial or deep	N0 or N1	M1	G1, G2, or G3

Table, Risk Based Treatment Stratification used in COG-ARST0332 study. Adapted from Spunt et al., 2014 and Sangkhathat, 2015 ^{[50, 58]}

Risk group	Tumor characteristics				Treatment
	Tumor Grade	Tumor Size	Tumor Stage	Upfront Surgical Resectability
Low	Low	Any	Localized	Gross resection (with negative or positive microscopic margins)	Surgery + observation
Low	High	< 5 cm	Localized	Gross resection with negative microscopic margins	Surgery + observation
Low	High	< 5 cm	Localized	Gross resection with positive microscopic margins	Surgery + adjuvant radiation therapy
Intermediate	High	>5 cm	Localized	Gross resection	Surgery + adjuvant radiation therapy + adjuvant chemotherapy
Intermediate	High	Any	Localized	Unresectable or Planned delayed surgical resection due to high grade, size >5 cm, and anticipate gross resection only possible with positive microscopic margins	Neoadjuvant chemotherapy + surgery + adjuvant chemotherapy with or without radiation therapy
High	Low	Any	Metastatic	Gross resection	Surgery + observation
High	High	Any	Metastatic	Gross resection	Surgery + adjuvant radiation therapy + chemotherapy
High	High	Any	Metastatic	Unresected	Neoadjuvant chemotherapy + surgery + adjuvant chemotherapy with or without radiation therapy

Back to List

Author

Jacquelyn N Crane, MD Clinical Assistant Professor, Division of Pediatric Hematology-Oncology, Stem Cell Transplantation and Regenerative Medicine, Associate Program Director, Pediatric Hematology-Oncology Fellowship Program, Stanford University School of Medicine

Jacquelyn N Crane, MD is a member of the following medical societies: American Society of Clinical Oncology, American Society of Hematology, American Society of Pediatric Hematology/Oncology, Children's Oncology Group, Connective Tissue Oncology Society

Disclosure: Nothing to disclose.

Coauthor(s)

Gary D Crouch, MD Associate Professor, Program Director of Pediatric Hematology-Oncology Fellowship, Department of Pediatrics, Uniformed Services University of the Health Sciences

Gary D Crouch, MD is a member of the following medical societies: American Academy of Pediatrics, American Society of Hematology

Disclosure: Nothing to disclose.

Noah C Federman, MD Director, Pediatric Bone and Soft Tissue Sarcoma Program, Associate Clinical Professor of Pediatrics, Department of Pediatrics, Division of Pediatric Hematology/Oncology, Adjunct Associate Professor, Department of Orthopedics, Mattel Children’s Hospital, University of California, Los Angeles, David Geffen School of Medicine; Medical Director, Clinical Translational Research Center, Chair, Data Safety Monitoring Board, Vice Chair, Scientific Rationale Committee, Clinical and Translational Science Institute (CTSI) at UCLA

Noah C Federman, MD is a member of the following medical societies: American Society of Clinical Oncology, American Society of Pediatric Hematology/Oncology, Children's Oncology Group, Connective Tissue Oncology Society, International Ewing Sarcoma Research Forum, Sarcoma Alliance for Research through Collaboration, Society for Pediatric Research

Disclosure: Nothing to disclose.

Specialty Editor Board

Mary L Windle, PharmD Adjunct Associate Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Nothing to disclose.

Chief Editor

Vikramjit S Kanwar, MBBS, MBA, MRCP(UK) Professor Emeritus of Pediatrics, Albany Medical College; Chief of Pediatric Oncology, Homi Bhabha Cancer Hospital, Varanasi, India

Vikramjit S Kanwar, MBBS, MBA, MRCP(UK) is a member of the following medical societies: Children's Oncology Group, Indian Academy of Pediatrics, International Society of Pediatric Oncology

Disclosure: Nothing to disclose.

Additional Contributors

Steven K Bergstrom, MD Department of Pediatrics, Division of Hematology-Oncology, Kaiser Permanente Medical Center of Oakland

Steven K Bergstrom, MD is a member of the following medical societies: Alpha Omega Alpha, Children's Oncology Group, American Society of Clinical Oncology, International Society for Experimental Hematology, American Society of Hematology, American Society of Pediatric Hematology/Oncology

Disclosure: Nothing to disclose.

Justine K Walker, MD Fellow, Division of Pediatric Hematology-Oncology, University of California, Los Angeles David Geffen School of Medicine

Justine K Walker, MD is a member of the following medical societies: American Academy of Pediatrics, American Society of Hematology

Disclosure: Nothing to disclose.

Acknowledgements

Samuel Gross, MD Professor Emeritus, Department of Pediatrics, University of Florida College of Medicine; Clinical Professor, Department of Pediatrics, University of North Carolina at Chapel Hill School of Medicine; Adjunct Professor, Department of Pediatrics, Duke University School of Medicine

Samuel Gross, MD is a member of the following medical societies: American Association for Cancer Research, American Society for Blood and Marrow Transplantation, American Society of Clinical Oncology, American Society of Hematology, and Society for Pediatric Research

Disclosure: Nothing to disclose.

Kathleen M Sakamoto, MD, PhD Shelagh Galligan Professor, Division of Hematology/Oncology, Department of Pediatrics, Stanford University School of Medicine

Kathleen M Sakamoto, MD, PhD is a member of the following medical societies: American Society of Hematology, American Society of Pediatric Hematology/Oncology, International Society for Experimental Hematology, and Society for Pediatric Research

Disclosure: Nothing to disclose.