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Childhood Rhabdomyosarcoma Treatment (PDQ®)

Health Professional Version
Last Modified: 05/02/2014

Stage Information

Before a biopsy of a suspected tumor mass is performed, imaging studies of the mass and baseline laboratory studies should be obtained. After the diagnosis of rhabdomyosarcoma has been made, an extensive evaluation to determine the extent of the disease should be performed before instituting therapy. This evaluation should include a chest x-ray, computed tomography (CT) scan of the chest, bilateral bone marrow aspirates and biopsies, bone scan, magnetic resonance imaging (MRI) of the base of the skull and brain (for parameningeal primary tumors only), and CT scan of the abdomen and pelvis (for lower extremity or genitourinary primary tumors).

A CT or MRI scan of regional lymph nodes should be considered. Abnormal-appearing lymph nodes should be biopsied when possible. One study has demonstrated that sentinel lymph node biopsies can be safely performed in children with rhabdomyosarcoma, and tumor-positive biopsies may alter the treatment plan.[1] Positron emission tomography (PET) with fluorine-18-fluorodeoxyglucose (FDG) scans can identify areas of possible metastatic disease not seen by other imaging modalities.[2-4] However, the efficacy of these two procedures for identifying involved lymph nodes or other sites is currently under investigation, and these procedures are not required by current treatment protocols.

Terms used in this summary section are defined below in Table 1.

Table 1. Definition of Terms
Term Definition 
Favorable siteOrbit; nonparameningeal head and neck; genitourinary tract other than kidney, bladder, and prostate; biliary tract.
Unfavorable siteAny site other than favorable.
T1Tumor confined to anatomic site of origin (noninvasive).
T2Tumor extension and/or fixation to surrounding tissue (invasive).
aTumor ≤5 cm in maximum diameter.
bTumor >5 cm in maximum diameter.
N0No clinical regional lymph node involvement.
N1Clinical regional lymph node involvement.
NXRegional lymph nodes not examined; no information.
M0No metastatic disease.
M1Metastatic disease.

Staging of rhabdomyosarcoma is relatively complex. The process includes the following steps:

  1. Assigning a Stage: Determined by primary site, tumor size (widest diameter), and presence or absence of regional lymph node and/or distant metastases.

  2. Assigning a local tumor Group: Determined by status postsurgical resection/biopsy, with pathologic assessment of the tumor margin and of lymph node disease.

  3. Assigning a Risk Group: Determined by Stage, Group, and histology.

As noted previously, prognosis for children with rhabdomyosarcoma depends predominantly on the primary site, tumor size, Group, and histologic subtype. Favorable prognostic groups were identified in previous Intergroup Rhabdomyosarcoma Study Group (IRSG) studies, and treatment plans were designed on the basis of assignment of patients to different treatment groups according to prognosis. Several years ago, the IRSG merged with the National Wilms Tumor Study Group and two large cooperative pediatric cancer treatment groups to form the Children's Oncology Group (COG). New protocols for children with soft tissue sarcoma are developed by the Soft Tissue Sarcoma Committee of the COG (COG-STS).

Current COG-STS protocols for rhabdomyosarcoma use the TNM-based pretreatment staging system that incorporates the primary tumor site, presence or absence of tumor invasion of surrounding tissues, tumor size, regional lymph node status, and the presence or absence of metastases. This staging system is described in Table 2 below.[5,6]

Table 2. Soft Tissue Sarcoma Committee of the Children's Oncology Group: Pretreatment Staging System
Stage  Sites of Primary Tumor T Stage Tumor Size Regional Lymph Nodes Distant Metastasis 
N0 = absence of nodal spread; N1 = presence of regional nodal spread beyond the primary site; X = unknown N status; M0 = absence of metastatic spread; M1 = presence of metastatic spread beyond the primary site and regional lymph nodes; T1 = tumor confined to anatomic site of origin (noninvasive); T2a = tumor extension and/or fixation to surrounding tissue (invasive), tumor ≤5 cm in maximum diameter; T2b = tumor extension and/or fixation to surrounding tissue (invasive), tumor >5 cm in maximum diameter.
1Favorable sitesT1 or T2Any sizeN0 or N1 or NXM0
2Unfavorable sitesT1 or T2a, ≤ 5 cmN0 or NXM0
3Unfavorable sitesT1 or T2a, ≤ 5 cmN1M0
b, > 5 cmN0 or N1 or NX
4Any siteT1 or T2Any sizeN0 or N1 or NXM1

The IRS-I, IRS-II, and IRS-III studies prescribed treatment plans based on the Surgical-pathologic Group system. In this system, Groups are defined by the extent of disease and by the completeness or extent of initial surgical resection after pathologic review of the tumor specimen(s). The definitions for these Groups are shown in Table 3 below.[7,8]

Table 3. Soft Tissue Sarcoma Committee of the Children's Oncology Group: Surgical-pathologic Group System
Group Incidence Definition 
IApproximately 13%Localized tumor, completely removed with microscopically clear margins and no regional lymph node involvement. Lymph node biopsy or sampling is encouraged if lymph nodes are clinically or radiographically suspicious.
IIApproximately 20%Localized tumor, completely removed with: (a) microscopic disease at the margin, (b) regional disease with involved, grossly removed regional lymph nodes without microresidual disease, or (c) regional disease with involved nodes, grossly removed but with microscopic residual and/or histologic involvement of the most distal node from the primary tumor.
IIIApproximately 48%Localized tumor, incompletely removed with gross, residual disease after: (a) biopsy only, or (b) gross major resection of the primary tumor (>50%).
IVApproximately 18%Distant metastases are present at diagnosis. This category includes: (a) radiographically identified evidence of tumor spread, and (b) positive tumor cells in cerebral spinal fluid, pleural, or peritoneal fluids, or implants in these regions.

After patients are categorized by Stage and Surgical-pathologic Group, a Risk Group is assigned. This takes into account Stage, Group, and histology. Patients are classified for protocol purposes as having a low risk, intermediate risk, or high risk of disease recurrence.[9,10] Treatment assignment is based on Risk Group, as shown in Table 4. To be designated as alveolar, the tumor must have greater than 50% alveolar elements; if the alveolar component is 50% or less, the tumor is considered embryonal.

Table 4. Soft Tissue Sarcoma Committee of the Children's Oncology Group: Rhabdomyosarcoma Risk Group Classification
Risk Group  Histology Stage Group 
Low riskEmbryonal1I, II, III
Embryonal2, 3I, II
Intermediate riskEmbryonal2, 3III
Alveolar1, 2, 3I, II, III
High riskEmbryonal or Alveolar4IV

 [Note: Since 2006, patients with undifferentiated sarcomas are treated on the COG-STS protocol for nonrhabdomyosarcomatous soft tissue sarcoma. Refer to the PDQ summary on Childhood Soft Tissue Sarcoma for more information.]

References
  1. Kayton ML, Delgado R, Busam K, et al.: Experience with 31 sentinel lymph node biopsies for sarcomas and carcinomas in pediatric patients. Cancer 112 (9): 2052-9, 2008.  [PUBMED Abstract]

  2. Völker T, Denecke T, Steffen I, et al.: Positron emission tomography for staging of pediatric sarcoma patients: results of a prospective multicenter trial. J Clin Oncol 25 (34): 5435-41, 2007.  [PUBMED Abstract]

  3. Tateishi U, Hosono A, Makimoto A, et al.: Comparative study of FDG PET/CT and conventional imaging in the staging of rhabdomyosarcoma. Ann Nucl Med 23 (2): 155-61, 2009.  [PUBMED Abstract]

  4. Federico SM, Spunt SL, Krasin MJ, et al.: Comparison of PET-CT and conventional imaging in staging pediatric rhabdomyosarcoma. Pediatr Blood Cancer 60 (7): 1128-34, 2013.  [PUBMED Abstract]

  5. Lawrence W Jr, Gehan EA, Hays DM, et al.: Prognostic significance of staging factors of the UICC staging system in childhood rhabdomyosarcoma: a report from the Intergroup Rhabdomyosarcoma Study (IRS-II). J Clin Oncol 5 (1): 46-54, 1987.  [PUBMED Abstract]

  6. Lawrence W Jr, Anderson JR, Gehan EA, et al.: Pretreatment TNM staging of childhood rhabdomyosarcoma: a report of the Intergroup Rhabdomyosarcoma Study Group. Children's Cancer Study Group. Pediatric Oncology Group. Cancer 80 (6): 1165-70, 1997.  [PUBMED Abstract]

  7. Crist WM, Garnsey L, Beltangady MS, et al.: Prognosis in children with rhabdomyosarcoma: a report of the intergroup rhabdomyosarcoma studies I and II. Intergroup Rhabdomyosarcoma Committee. J Clin Oncol 8 (3): 443-52, 1990.  [PUBMED Abstract]

  8. Crist W, Gehan EA, Ragab AH, et al.: The Third Intergroup Rhabdomyosarcoma Study. J Clin Oncol 13 (3): 610-30, 1995.  [PUBMED Abstract]

  9. Raney RB, Anderson JR, Barr FG, et al.: Rhabdomyosarcoma and undifferentiated sarcoma in the first two decades of life: a selective review of intergroup rhabdomyosarcoma study group experience and rationale for Intergroup Rhabdomyosarcoma Study V. J Pediatr Hematol Oncol 23 (4): 215-20, 2001.  [PUBMED Abstract]

  10. 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 21 (1): 78-84, 2003.  [PUBMED Abstract]