eMedicine Specialties > Pediatrics: General Medicine > Oncology

Nonrhabdomyosarcoma Soft Tissue Sarcomas: Differential Diagnoses & Workup

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

Differential Diagnoses

Aggressive fibromatosis (Desmoid tumor)
Langerhans cell histiocytosis
Cysts
Lipoma
Dermatofibroma
Neuroblastoma
Ewing Sarcoma and Primitive Neuroectodermal Tumors
Neurofibroma

Other Problems to Be Considered

Other malignancies that cause masses in children must be considered during evaluation. Examples include lymphomas, osteosarcoma, Ewing sarcoma, rhabdomyosarcoma, and neuroblastoma. Benign lesions (eg, lipomas, rhabdomyomas) should be considered as well.

Workup

Laboratory Studies

  • In patients with nonrhabdomyosarcoma soft tissue sarcoma (NRSTS), a baseline CBC count with differential provides parameters before therapy and is useful in evaluating for involvement of the bone marrow.
  • Chemical tests to assess renal function and creatinine clearance provide baseline parameters before chemotherapy is given and further testing is performed.
  • Liver function testing provides baseline parameters before chemotherapy and is helpful in evaluating for hepatic involvement.

Imaging Studies

Chest radiography and chest CT are useful for evaluating for lung involvement. Perform these studies before the use of general anesthesia, which can cause pulmonary changes that might make the interpretation of images difficult.

  • CT and MRI are used to determine the size of the mass and the extent of local involvement and impingement on adjacent structures.
    • CT and MRI also help in defining options for surgical resection. Contrast-enhanced studies are most helpful.
    • Abdominal CT scanning is important for assessing abdominal primary lesions and to determine hepatic involvement.
  • Radionucleotide bone scanning is necessary to rule out bony involvement.
  • Plain radiography of the involved areas may be useful in the initial evaluation of a mass, depending on its location and suspected involvement of bony structures.
  • The roles of positive emission tomography (PET)-CT and of18 F-fluorodeoxyglucose (FDG) PET to image NRSTS in children have not been well established. However, PET-CT may prove beneficial in distinguishing normal from pathologic processes, in the initial staging of a sarcoma, in monitoring responses to therapy, and in detecting recurrences.3,4

Other Tests

  • A cardiologist may need to be consulted to perform cardiac ECG and echocardiography in patients who will receive anthracycline-based chemotherapy or radiation therapy to the chest.
  • Testing of renal glomerular filtration or creatinine clearance may be necessary before renal-toxic chemotherapy agents (eg, cisplatin, ifosfamide) are administered.

Procedures

  • Carefully planned and executed biopsy of the mass lesion is required for diagnosis.
    • Whatever technique is used, adequate tissue must be obtained to yield a diagnosis. If possible, surgery should be accomplished in a manner that does not compromise the possibility for later local surgical control of the tumor.
    • A surgeon with expertise in oncologic surgery should perform this procedure. The surgeon's specific discipline depends on the location of the mass.
    • The best approach for small lesions in accessible areas may be excisional biopsy.
    • Large masses involving critical organs or structures may require incisional biopsy for diagnosis.
    • Delay the definitive surgical procedure until after adjuvant chemotherapy, radiation therapy, or both are given to shrink the tumor.
    • Fine-needle aspiration biopsy may be possible in certain cases. However, an open procedure is required if this technique yields nondiagnostic pathologic material.
    • Minimally invasive surgical techniques using fiberoptic surgical procedures may be appropriate in certain biopsy situations.
  • Consider long-term venous access. In most cases, placement of an implanted or externalized central venous catheter is useful for monitoring laboratory results and for delivering chemotherapy and supportive care.
  • Bilateral bone marrow aspirates and biopsy samples should be obtained in most cases to rule out tumoral involvement of the bone marrow. Strongly consider examining the bone marrow in all patients with NRSTS.
    • In children, these procedures are best performed in the posterior iliac crests.
    • These tests should be accomplished in conjunction with another procedure requiring sedation, if possible.
    • If patients have parameningeal tumors or tumors involving the CNS, lumbar puncture may be necessary to rule out contamination of the cerebrospinal fluid (CSF) with tumor cells.

Histologic Findings

  • Diagnosis of an NRSTS can be confirmed only with biopsy of the mass. Diagnosis of a specific tumor depends on the mesenchymal an/or support tissue it most closely represents. Immunostaining, electron microscopy, cytogenetic analysis, and tests for molecular markers of genetic rearrangements may all be used for final diagnosis, depending on the differentiation of the specific tumor. NRSTSs possess a wide range of histologic features.
  • A tumor is classified as low-grade or high-grade on the basis of its potential to metastasize. Low-grade lesions are unlikely to metastasize. Certain types of tumors are arbitrarily considered high grade; examples are synovial cell sarcomas and malignant peripheral nerve sheath tumors. Malignant and metastatic potential are based on the degree of anaplasia and mitotic activity the tumor specimen exhibits.

Staging

There is no validated staging system for NRSTSs in children. The Intergroup Rhabdomyosarcoma Study (IRS) group staging system is most commonly used, as follows:

  • Stages (Tumor, node, and metastases [TNM] system)
    • Involvement of an organ or tissue of origin (T1, N0, M0)
    • Invasion of contiguous organs or tissues or adjacent malignant effusion (T2, N0, M0)
    • Involvement of regional nodes  (T1 or T2, N1, M0)
    • Distant metastases (T1 or T2, N0 or N1, M1)
  • Intergroup Rhabdomyosarcoma Study (IRS) groups
    • Group I - Complete resection
    • Group II - Microscopic residual disease after resection
    • Group III - Gross localized residual disease
    • Group IV - Metastatic disease

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
[ CLOSE WINDOW ]

References

  1. 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].

  2. 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].

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

  4. 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].

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

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

  7. 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].

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

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

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

  11. 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].

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

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

  14. 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.

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

  16. 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].

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

  18. 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].

  19. 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.

  20. 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].

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

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

[ CLOSE WINDOW ]

Further Reading

[ CLOSE WINDOW ]

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

[ CLOSE WINDOW ]

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.

RELATED EMEDICINE ARTICLES
 
 
HONcode

We subscribe to the
HONcode principles of the
Health On the Net Foundation

All material on this website is protected by copyright, Copyright© 1994- by Medscape.
This website also contains material copyrighted by 3rd parties.

DISCLAIMER: The content of this Website is not influenced by sponsors. The site is designed primarily for use by qualified physicians and other medical professionals. The information contained herein should NOT be used as a substitute for the advice of an appropriately qualified and licensed physician or other health care provider. The information provided here is for educational and informational purposes only. In no way should it be considered as offering medical advice. Please check with a physician if you suspect you are ill.