eMedicine Specialties > Pediatrics: Surgery > General Surgery

Rhabdomyosarcoma, Surgical Treatment: Workup

Author: Holly L Neville, MD, Assistant Professor of Clinical Surgery, Division of Pediatric Surgery, University of Miami Miller School of Medicine
Coauthor(s): Richard Andrassy, MD, Denton A Cooley, MD Chair and Professor, Department of Surgery, University of Texas at Houston Health Science Center; Chief, Section of Pediatric Surgery, MD Anderson Cancer Center; Chief Surgeon, Department of Surgery, Memorial-Hermann Hospital
Contributor Information and Disclosures

Updated: Jun 4, 2009

Workup

Laboratory Studies

  • Perform routine chemistries and CBC count with differential and platelets to obtain basic baseline information prior to administration of general anesthesia or chemotherapy.

Imaging Studies

  • The goal of the evaluation in a patient with a suspected rhabdomyosarcoma should be to define the local extent of the tumor (ie, resectability), lymph node involvement, and distant metastasis.
  • CT scanning or MRI of the primary tumor is necessary to assess the size of the tumor and its extension into adjacent structures. These studies also assist in narrowing the differential diagnoses. Positron emission tomography (PET)/CT scanning may prove to be valuable in staging and preoperative evaluation of pediatric rhabdomyosarcoma.6
  • Perform a bone scan to rule out metastatic disease to the skeletal structures.
  • A CT scan of the chest is also recommended to rule out metastasis to the pulmonary parenchyma.

Diagnostic Procedures

  • Evaluate the bone marrow using bone marrow aspiration to rule out metastatic spread to the bone marrow.

Histologic Findings

The diagnosis of rhabdomyosarcoma should be confirmed through a tissue specimen, which may be attained via fine needle, core, incisional, or excisional biopsy. The characteristic histologic finding of rhabdomyosarcoma is that of a small, blue, round cell. Cross striations may be seen with light microscopy. The histologic subtypes are discussed in Pathophysiology. For additional information, see the article Rhabdomyosarcoma in eMedicine’s Pathology volume.

The histologic findings in rhabdomyosarcoma.

The histologic findings in rhabdomyosarcoma.

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The histologic findings in rhabdomyosarcoma.

The histologic findings in rhabdomyosarcoma.

Staging

Once the diagnosis of rhabdomyosarcoma has been made, the tumor should be staged. The staging system currently used for rhabdomyosarcomas is the Lawrence/Gehan Staging System, which is initiated preoperatively and then completed after resection.

  • Stage and primary disease sites
    • Stage 1 - Orbit/eyelid, head and neck (excluding parameningeal [PM]), genitourinary (excluding bladder/prostate)
    • Stage 2 - Bladder/prostate, extremity, PM, other (eg, trunk, retroperitoneum), smaller than 5 cm
    • Stage 3 - Bladder/prostate, extremity, PM, other (eg, trunk, retroperitoneum), larger than 5 cm
    • Stage 4 - All others
  • IRS Group postsurgical pathologic grouping
    • Group I - Localized disease, completely resected (clear margins, negative regional nodes)
    • Group II - Microscopic disease remaining (at margins or in regional nodes)
    • Group III - Incomplete resection or biopsy findings indicating gross residual disease (locally or in regional nodes)
    • Group IV - Distant metastases present at onset
Recent studies suggest that a cutoff tumor size of 5 cm may not be the best tool for staging pediatric rhabdomyosarcoma. Owing to variations in the body surface area of children, the tumor size in relation to the patient’s body surface area may be more useful in staging.7

More on Rhabdomyosarcoma, Surgical Treatment

Overview: Rhabdomyosarcoma, Surgical Treatment
Workup: Rhabdomyosarcoma, Surgical Treatment
Treatment: Rhabdomyosarcoma, Surgical Treatment
Follow-up: Rhabdomyosarcoma, Surgical Treatment
Multimedia: Rhabdomyosarcoma, Surgical Treatment
References
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References

  1. Qualman S, Lynch J, Bridge J, Parham D, Teot L, Meyer W, et al. Prevalence and clinical impact of anaplasia in childhood rhabdomyosarcoma : a report from the Soft Tissue Sarcoma Committee of the Children's Oncology Group. Cancer. Dec 1 2008;113(11):3242-7. [Medline].

  2. Neville HL, Andrassy RJ, Lobe TE, et al. Preoperative staging, prognostic factors, and outcome for extremity rhabdomyosarcoma: a preliminary report from the Intergroup Rhabdomyosarcoma Study IV (1991-1997). J Pediatr Surg. Feb 2000;35(2):317-21. [Medline].

  3. Raney RB, Stoner JA, Walterhouse DO, et al. Results of treatment of fifty-six patients with localized retroperitoneal and pelvic rhabdomyosarcoma: a report from The Intergroup Rhabdomyosarcoma Study-IV, 1991-1997. Pediatr Blood Cancer. Jun 2004;42(7):618-25. [Medline].

  4. De Corti F, Dall'Igna P, Bisogno G, Casara D, Rossi CR, Foletto M, et al. Sentinel node biopsy in pediatric soft tissue sarcomas of extremities. Pediatr Blood Cancer. Jan 2009;52(1):51-4. [Medline].

  5. Gow KW, Rapkin LB, Olson TA, Durham MM, Wyly B, Shehata BM. Sentinel lymph node biopsy in the pediatric population. J Pediatr Surg. Dec 2008;43(12):2193-8. [Medline].

  6. Murphy JJ, Tawfeeq M, Chang B, Nadel H. Early experience with PET/CT scan in the evaluation of pediatric abdominal neoplasms. J Pediatr Surg. Dec 2008;43(12):2186-92. [Medline].

  7. Ferrari A, Miceli R, Meazza C, Zaffignani E, Gronchi A, Piva L, et al. Soft tissue sarcomas of childhood and adolescence: the prognostic role of tumor size in relation to patient body size. J Clin Oncol. Jan 20 2009;27(3):371-6. [Medline].

  8. Arndt CA, Hawkins DS, Meyer WH, Sencer SF, Neglia JP, Anderson JR. Comparison of results of a pilot study of alternating vincristine/doxorubicin/cyclophosphamide and etoposide/ifosfamide with IRS-IV in intermediate risk rhabdomyosarcoma: a report from the Children's Oncology Group. Pediatr Blood Cancer. Jan 2008;50(1):33-6. [Medline].

  9. Casanova M, Ferrari A, Spreafico F, et al. Vinorelbine in previously treated advanced childhood sarcomas: evidence of activity in rhabdomyosarcoma. Cancer. Jun 15 2002;94(12):3263-8. [Medline].

  10. Cecchetto G, Bisogno G, De Corti F, Dall'Igna P, Inserra A, Ferrari A, et al. Biopsy or debulking surgery as initial surgery for locally advanced rhabdomyosarcomas in children?: the experience of the Italian Cooperative Group studies. Cancer. Dec 1 2007;110(11):2561-7. [Medline].

  11. Andrassy RJ, Corpron CA, Hays D, et al. Extremity sarcomas: an analysis of prognostic factors from the Intergroup Rhabdomyosarcoma Study III. J Pediatr Surg. Jan 1996;31(1):191-6. [Medline].

  12. Andrassy RJ, Hays DM, Raney RB, et al. Conservative surgical management of vaginal and vulvar pediatric rhabdomyosarcoma: a report from the Intergroup Rhabdomyosarcoma Study III. J Pediatr Surg. Jul 1995;30(7):1034-6; discussion 1036-7. [Medline].

  13. Barr FG, Smith LM, Lynch JC, Strzelecki D, Parham DM, Qualman SJ, et al. Examination of gene fusion status in archival samples of alveolar rhabdomyosarcoma entered on the Intergroup Rhabdomyosarcoma Study-III trial: a report from the Children's Oncology Group. J Mol Diagn. May 2006;8(2):202-8. [Medline].

  14. Breneman JC, Lyden E, Pappo AS, et al. Prognostic factors and clinical outcomes in children and adolescents with metastatic rhabdomyosarcoma--a report from the Intergroup Rhabdomyosarcoma Study IV. J Clin Oncol. Jan 1 2003;21(1):78-84. [Medline].

  15. Burke M, Anderson JR, Kao SC, Rodeberg D, Qualman SJ, Wolden SL, et al. Assessment of response to induction therapy and its influence on 5-year failure-free survival in group III rhabdomyosarcoma: the Intergroup Rhabdomyosarcoma Study-IV experience--a report from the Soft Tissue Sarcoma Committee of the Children's Oncology Group. J Clin Oncol. Nov 1 2007;25(31):4909-13. [Medline].

  16. Cofer BR, Wiener ES. Rhabdomyosarcoma. In: Andrassy, ed. Pediatric Surgical Oncology. Philadelphia, Pa: WB Saunders; 1998:221-35.

  17. Crist WM, Anderson JR, Meza JL, et al. Intergroup rhabdomyosarcoma study-IV: results for patients with nonmetastatic disease. J Clin Oncol. Jun 15 2001;19(12):3091-102. [Medline].

  18. Felix CA, Kappel CC, Mitsudomi T, et al. Frequency and diversity of p53 mutations in childhood rhabdomyosarcoma. Cancer Res. Apr 15 1992;52(8):2243-7. [Medline].

  19. Hays DM. Bladder/prostate rhabdomyosarcoma: results of the multi-institutional trials of the Intergroup Rhabdomyosarcoma Study. Semin Surg Oncol. Nov-Dec 1993;9(6):520-3. [Medline].

  20. Hays DM, Lawrence W Jr, Wharam M, Newton W Jr, Ruymann FB, Beltangady M, et al. Primary reexcision for patients with 'microscopic residual' tumor following initial excision of sarcomas of trunk and extremity sites. J Pediatr Surg. Jan 1989;24(1):5-10. [Medline].

  21. Leuschner I, Newton WA, Schmidt D, et al. Spindle cell variants of embryonal rhabdomyosarcoma in the paratesticular region. A report of the Intergroup Rhabdomyosarcoma Study. Am J Surg Pathol. Mar 1993;17(3):221-30. [Medline].

  22. Li FP, Fraumeni JF, Mulvihill JJ, et al. A cancer family syndrome in twenty-four kindreds. Cancer Res. Sep 15 1988;48(18):5358-62. [Medline].

  23. Maurer HM, Beltangady M, Gehan EA, et al. The Intergroup Rhabdomyosarcoma Study-I. A final report. Cancer. Jan 15 1988;61(2):209-20. [Medline].

  24. Meza JL, Anderson J, Pappo AS, Meyer WH. Analysis of prognostic factors in patients with nonmetastatic rhabdomyosarcoma treated on intergroup rhabdomyosarcoma studies III and IV: the Children's Oncology Group. J Clin Oncol. Aug 20 2006;24(24):3844-51. [Medline].

  25. Ogawa O, Eccles MR, Szeto J, McNoe LA, Yun K, Maw MA, et al. Relaxation of insulin-like growth factor II gene imprinting implicated in Wilms' tumour. Nature. Apr 22 1993;362(6422):749-51. [Medline].

  26. Raney RB, Hays DM, Tefft M, et al. Rhabdomyosarcoma and the undifferentiated sarcomas. In: Pizzo PA, Poplack DG, eds. Principles and Practice of Pediatric Oncology. Philadelphia, Pa: Lippincott; 1988:635-53.

  27. Raney RB, Maurer HM, Anderson JR, Andrassy RJ, Donaldson SS, Qualman SJ, et al. The Intergroup Rhabdomyosarcoma Study Group (IRSG): Major Lessons From the IRS-I Through IRS-IV Studies as Background for the Current IRS-V Treatment Protocols. Sarcoma. 2001;5(1):9-15. [Medline].

  28. Scrable HJ, Witte DP, Lampkin BC, Cavenee WK. Chromosomal localization of the human rhabdomyosarcoma locus by mitotic recombination mapping. Nature. Oct 15-21 1987;329(6140):645-7. [Medline].

  29. Sorensen PH, Lynch JC, Qualman SJ, Tirabosco R, Lim JF, Maurer HM, et al. PAX3-FKHR and PAX7-FKHR gene fusions are prognostic indicators in alveolar rhabdomyosarcoma: a report from the children's oncology group. J Clin Oncol. Jun 1 2002;20(11):2672-9. [Medline].

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Further Reading

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Keywords

rhabdomyosarcoma, pediatric rhabdomyosarcoma, rhabdomyosarcoma in children, soft tissue sarcomas, embryonal rhabdomyosarcoma, alveolar rhabdomyosarcoma, botryoid rhabdomyosarcoma, spindle cell rhabdomyosarcoma, undifferentiated rhabdomyosarcoma, p53 mutation, Li-Fraumeni syndrome, p53 gene, bony sarcomas, leukemia, brain neoplasms, adrenal neoplasms, maternal premenopausal breast cancer, neurofibromatosis type I, Beckwith-Wiedemann syndrome

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Contributor Information and Disclosures

Author

Holly L Neville, MD, Assistant Professor of Clinical Surgery, Division of Pediatric Surgery, University of Miami Miller School of Medicine
Holly L Neville, MD is a member of the following medical societies: American Academy of Pediatrics, American College of Surgeons, American Pediatric Surgical Association, and Association of Women Surgeons
Disclosure: Nothing to disclose.

Coauthor(s)

Richard Andrassy, MD, Denton A Cooley, MD Chair and Professor, Department of Surgery, University of Texas at Houston Health Science Center; Chief, Section of Pediatric Surgery, MD Anderson Cancer Center; Chief Surgeon, Department of Surgery, Memorial-Hermann Hospital
Richard Andrassy, MD is a member of the following medical societies: American Academy of Pediatrics, American College of Physician Executives, American College of Surgeons, American Society for Clinical Nutrition, American Society of Pediatric Hematology/Oncology, American Trauma Society, Association for Academic Surgery, International College of Surgeons, Society of Surgical Oncology, and Southwestern Surgical Congress
Disclosure: Nothing to disclose.

Medical Editor

Diana Farmer, MD, Associate Professor, Departments of Clinical Surgery, Pediatrics, Obstetrics, Gynecology and Reproductive Services, Division of Pediatric Surgery and the Fetal Treatment Center, University of California at San Francisco
Diana Farmer, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Pediatrics, American College of Surgeons, and American Pediatric Surgical Association
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 financial planner; Avanir Pharma Stock Investment from financial planner ; WebMD Salary and stock Employment and investment from financial planner

Managing Editor

Nicholas A Shorter, MD, Professor of Clinical Surgery and Clinical Pediatrics, State University of New York-Downstate University; Division Chief, Department of Surgery, Division of Pediatric Surgery, State University of New York-Downstate Medical Center
Disclosure: Nothing to disclose.

CME Editor

H Biemann Othersen Jr, MD, Professor of Surgery and Pediatrics, Emeritus Head, Division of Pediatric Surgery, Medical University of South Carolina
H Biemann Othersen Jr, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Pediatrics, American Association for the Surgery of Trauma, American Burn Association, American Cancer Society, American College of Surgeons, American Medical Association, American Pediatric Surgical Association, American Society for Parenteral and Enteral Nutrition, American Surgical Association, American Thoracic Society, British Association of Paediatric Surgeons, Society for Surgery of the Alimentary Tract, Society of Critical Care Medicine, South Carolina Medical Association, Southeastern Surgical Congress, Southern Medical Association, Southern Society for Pediatric Research, and Southern Thoracic Surgical Association
Disclosure: Nothing to disclose.

Chief Editor

Marleta Reynolds, MD, Professor of Surgery, Feinberg School of Medicine, Northwestern University; Interim Head, Department of Surgery and Surgeon in Chief, Head, Division of Pediatric Surgery, Children's Memorial Hospital of Chicago
Marleta Reynolds, MD is a member of the following medical societies: American Pediatric Surgical Association
Disclosure: Nothing to disclose.

 
 
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