Nonrhabdomyosarcoma Soft Tissue Sarcomas Follow-up

  • Author: Justine K Walker, MD; Chief Editor: Robert J Arceci, MD, PhD   more...
 
Updated: Aug 6, 2010
 

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.

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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.)
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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.

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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.

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Contributor Information and Disclosures
Author

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 Association of Pediatrics and American Society of Hematology

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 and American Society of Hematology

Disclosure: Nothing to disclose.

Noah C Federman, MD  Assistant Professor of Pediatrics, Division of Pediatric Hematology/Oncology, Mattel Children's Hospital, David Geffen School of Medicine; Director, Pediatric Bone and Soft Tissue Sarcoma Program, University of California at Los Angeles

Noah C Federman, MD is a member of the following medical societies: American Academy of Pediatrics, American Society of Hematology, and Connective Tissue Oncology Society

Disclosure: Nothing to disclose.

Specialty Editor Board

Samuel Gross, MD  Professor Emeritus, Department of Pediatrics, University of Florida; Clinical Professor, Department of Pediatrics, University of North Carolina; Adjunct Professor, Department of Pediatrics, Duke University

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.

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.

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, American Society of Clinical Oncology, American Society of Hematology, American Society of Pediatric Hematology/Oncology, Children's Oncology Group, and International Society for Experimental Hematology

Disclosure: Nothing to disclose.

Paul D Petry, DO, FACOP, FAAP  Consulting Staff, Freeman Pediatric Care, Freeman Health System

Paul D Petry, DO, FACOP, FAAP is a member of the following medical societies: American Academy of Osteopathy, American Academy of Pediatrics, American College of Osteopathic Pediatricians, and American Osteopathic Association

Disclosure: Nothing to disclose.

Chief Editor

Robert J Arceci, MD, PhD  King Fahd Professor of Pediatric Oncology, Professor of Pediatrics, Oncology and the Cellular and Molecular Medicine Graduate Program, Kimmel Comprehensive Cancer Center at Johns Hopkins University School of Medicine

Robert J Arceci, MD, PhD is a member of the following medical societies: American Association for Cancer Research, American Association for the Advancement of Science, American Pediatric Society, American Society of Hematology, and American Society of Pediatric Hematology/Oncology

Disclosure: Nothing to disclose.

Additional Contributors

The authors and editors of eMedicine gratefully acknowledge the contributions of previous author Kathleen M Sakamoto, MD, PhD, to the original writing and development of this article.

References

  1. 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. [Medline].

  2. 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. Feb 1 2005;23(4):866-73. [Medline].

  3. 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. [Medline]. [Full Text].

  4. 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. [Medline].

  5. Faulkner LB, Hajdu SI, Kher U, et al. Pediatric desmoid tumor: retrospective analysis of 63 cases. J Clin Oncol. 1995;13:2813-8. [Medline].

  6. 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. [Medline].

  7. Daller JA, Bueno J, Gutierrez J, et al. Hepatic hemangioendothelioma: clinical experience and management strategy. J Pediatr Surg. 1999;34:98-105. [Medline].

  8. Randall RL, Albritton KH, Ferney BJ, Layfield L. Malignant fibrous histiocytoma of soft tissue: an abandoned diagnosis. Am J Orthop (Belle Mead NJ). Dec 2004;33(12):602-8. [Medline].

  9. Erlandson RA, Antonescu CR. The rise and fall of malignant fibrous histiocytoma. Ultrastruct Pathol. Sep-Dec 2004;28(5-6):283-9. [Medline].

  10. Federman N, Feig SA. PET/CT in evaluating pediatric malignancies: a clinician's perspective. J Nucl Med. Dec 2007;48(12):1920-2. [Medline].

  11. McCarville MB, Christie R, Daw NC, et al. PET/CT in the evaluation of childhood sarcomas. AJR Am J Roentgenol. Apr 2005;184(4):1293-304. [Medline].

  12. American Joint Committee on Cancer. AJCC Cancer Staging Manual. 6th. New York, NY: Springer; 2002.

  13. 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. [Medline].

  14. Phase III Study of Risk-Based Therapy Comprising Observation Only, Adjuvant Radiotherapy, or Adjuvant Chemoradiotherapy or Neoadjuvant Chemoradiotherapy, Surgery, and Adjuvant Chemotherapy With or Without Radiotherapy in Young Patients With Non-Rhabdomyosarcoma Soft Tissue Sarcoma. Available at http://www.hemonc.theclinics.com/article/S0889-8588(05)00034-1/abstract.

  15. 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. [Medline].

  16. 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. [Medline].

  17. Putnam JB Jr, Roth JA. Surgical treatment for pulmonary metastases from sarcoma. Hematol Oncol Clin North Am. 1995;9 (4):869-87. [Medline].

  18. Albritton KH. Sarcomas in adolescents and young adults. Hematol Oncol Clin North Am. 2005;19:527-546. [Medline]. [Full Text].

  19. Antonescu CR. The role of genetic testing in soft tissue sarcoma. Histopathology. Jan 2006;48(1):13-21. [Medline].

  20. 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. May 1999;34(5):672-5. [Medline].

  21. Dillon PW. Nonrhabdomyosarcoma soft tissue sarcomas in children. Seminars in Pediatric Surgery. 1997;6(1):24-28. [Medline].

  22. 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. [Medline].

  23. Lanzkowsky P. Rhabdomyosarcoma and other soft tissue sarcomas. In: Manual of Pediatric Hematology and Oncology. 2005. 4th ed. San Diego, Calif: Academic; 561-584.

  24. Loeb DM, Thornton K, Shokek O. Pediatric soft tissue sarcomas. Surg Clin North Am. Jun 2008;88(3):615-27, vii. [Medline].

  25. Marcus KC, Grier HE, Shamberger RC, et al. Childhood soft tissue sarcoma: a 20-year experience. J Pediatr. Oct 1997;131(4):603-7. [Medline].

  26. Marcus RB Jr. Current controversies in pediatric radiation oncology. Orthop Clin North Am. Jul 1996;27(3):551-7. [Medline].

  27. Meyer WH, Spunt SL. Soft tissue sarcomas of childhood. Cancer Treat Rev. 2004;30:269-80. [Medline]. [Full Text].

  28. Okeu M, Hicks J, Merchant TE, Andrassy RJ, Pappo AS, Horowitz ME. Non-rhabdomyosarcomatous soft tissue sarcomas. In: Pizzo PA, Poplack DG. Principles and Practice of Pediatric Oncology. 5th. Philadelphia: Lippincott Williams and Wilkins; 2006:1034-1073.

  29. Pappo AS. Rhabdomyosarcoma and other soft tissue sarcomas in children. Curr Opin Oncol. Jul 1996;8(4):311-6. [Medline].

  30. 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. Apr 2006;46(4):489-95. [Medline].

  31. Paulino AC. Treatment options for children with nonrhabdomyosarcoma soft tissue sarcoma. Expert Rev Anticancer Ther. 2004;4:247-56. [Medline].

  32. Sirvent N, Maire G, Pedeutour F. Genetics of dermatofibrosarcoma protuberans family of tumors: from ring chromosomes to tyrosine kinase inhibitor treatment. Genes Chromosomes Cancer. May 2003;37(1):1-19. [Medline].

  33. Spunt S, Wolden S, Schofield D, Skapek S. Non-Rhabdomyosarcoma soft tissue sarcomas. In: Pappo, Alberto. Pediatric Bone and Soft Tissue Sarcomas. New York: Springer; 2006:133-162.

  34. 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. Dec 1999;17(12):3697-705. [Medline].

  35. Spunt SL, Skapek SX, Coffin CM. Pediatric nonrhabdomyosarcoma soft tissue sarcomas. Oncologist. Jun 2008;13(6):668-78. [Medline].

  36. Womer RB, Sinniah D. Soft tissue sarcomas. In: Practical Pediatric Oncology. 1992:318-23.

 
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Table. American Joint Commission for Cancer Staging (Tumor, Node, and Metastases [TNM] System)
Primary TumorRegional Lymph NodesDistant MetastasisHistologic Grade
Stage IAny tumor size, superficial or deepN0M0G1 or G2
Stage IIT1a (tumor < 5 cm, superficial)N0M0G3
T1b (tumor < 5 cm, deep)N0M0G3
T2a (tumor >5 cm, superficial)N0M0G3
Stage IIIT2b (tumor >5 cm, deep)N0M0G3
Stage IVAny tumor size, superficial or deepN1M0 or M1G1, G2, or G3
Any tumor size, superficial or deepN0 or N1M1G1, G2, or G3
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