eMedicine Specialties > Pediatrics: General Medicine > Oncology

Nonrhabdomyosarcoma Soft Tissue Sarcomas: Follow-up

Author: 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
Coauthor(s): Kathleen M Sakamoto, MD, PhD, Professor and Chief, Division of Hematology-Oncology, Vice-Chair of Research, Mattel Children's Hospital at UCLA; Department of Pathology and Laboratory Medicine, Jonsson Comprehensive Cancer Center, David Geffen School of Medicine at UCLA and California Nanosystems Institute and Molecular Biology, UCLA; Gary D Crouch, MD, Program Director of Pediatric Hematology-Oncology Fellowship, Department of Pediatrics, Associate Professor, Uniformed Services University of the Health Sciences
Contributor Information and Disclosures

Updated: Dec 3, 2008

Follow-up

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.

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.)

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.
 


More on Nonrhabdomyosarcoma Soft Tissue Sarcomas

Overview: Nonrhabdomyosarcoma Soft Tissue Sarcomas
Differential Diagnoses & Workup: Nonrhabdomyosarcoma Soft Tissue Sarcomas
Treatment & Medication: Nonrhabdomyosarcoma Soft Tissue Sarcomas
Follow-up: Nonrhabdomyosarcoma Soft Tissue Sarcomas
References
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References

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Further Reading

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Keywords

nonrhabdomyosarcoma soft tissue sarcoma, NRSTS, tumor, fibrosarcoma, malignant peripheral nerve sheath tumor, malignant fibrous histiocytoma, synovial sarcoma, alveolar soft part sarcoma, leiomyosarcoma, liposarcoma, dermatofibrosarcoma protuberans, epithelioid sarcoma, desmoplastic small round cell tumor, infantile fibrosarcoma, IFS, nodular fasciitis, myositis ossificans, neurofibromatosis type I, NF1, human immunodeficiency virus, Epstein-Barr virus, EBV, hemangiopericytomas, hypoglycemia, hypophosphatemic rickets, hyperglycemia, Li-Fraumeni syndrome, Gorlin syndrome

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Contributor Information and Disclosures

Author

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.

Coauthor(s)

Kathleen M Sakamoto, MD, PhD, Professor and Chief, Division of Hematology-Oncology, Vice-Chair of Research, Mattel Children's Hospital at UCLA; Department of Pathology and Laboratory Medicine, Jonsson Comprehensive Cancer Center, David Geffen School of Medicine at UCLA and California Nanosystems Institute and Molecular Biology, UCLA
Kathleen M Sakamoto, MD, PhD is a member of the following medical societies: American Society of Hematology, American Society of Pediatric Hematology/Oncology, New York Academy of Sciences, Society for Pediatric Research, and Western Society for Pediatric Research
Disclosure: Nothing to disclose.

Gary D Crouch, MD, Program Director of Pediatric Hematology-Oncology Fellowship, Department of Pediatrics, Associate Professor, 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.

Medical Editor

Samuel Gross, MD, Professor Emeritus, Department of Pediatrics, University of Florida, Clinical Professor, Department of Pediatrics, UNC, 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.

Pharmacy Editor

Mary L Windle, PharmD, Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy, Pharmacy Editor, eMedicine
Disclosure: Pfizer Inc Stock Investment from broker recommendation; Avanir Pharma Stock Investment from broker recommendation

Managing Editor

Steven K Bergstrom, MD, Assistant to the Chairman, 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.

CME Editor

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, Department of Oncology, Division of Pediatric Oncology, 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.

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