General Principles

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Successful treatment of rhabdomyosarcoma requires achievement of both local and systemic control of disease. Local control is accomplished using surgery and/or radiotherapy. Systemic control is accomplished with chemotherapy. Patients with localized disease require systemic therapy to eradicate micrometastatic disease typically present at diagnosis.

Surgery

Surgery is most effective if the tumor can be completely excised with an adequate margin of uninvolved tissue. If the tumor cannot be resected because of proximity to blood vessels and nerves or because this would produce major functional or cosmetic sequelae, an incisional or debulking biopsy is performed. This is followed by induction chemotherapy and radiation to cytoreduce the tumor. Second-look surgery should then be performed to resect residual tumor, if possible.

For primary tumors arising in the orbit, head and neck, and certain extremity locations, aggressive surgical debulking, such as enucleation, head and neck dissection, and amputation, are not indicated. Instead, chemotherapy and radiotherapy should be relied on to control the tumor at the primary site. Radical surgical debulking is reserved for cases with residual disease after chemotherapy and radiation therapy.

The role of lymph node biopsy or dissection as part of the primary approach depends on the tumor site. Clinically suspicious nodes should be excised at any site. In addition, routine sampling of regional nodes is appropriate for most sites; search of a sentinel node in extremity lesions is advisable. Sites with a high incidence of regional node involvement include:

Prophylactic regional radical node dissection is not necessary. Radiotherapy

Rhabdomyosarcoma is only moderately radiation sensitive and, for this reason, high doses of radiation are required. The dose of radiation to most sites is not modified for patient age or tumor size, although modifications are recommended for young patients in the following instances:

• Limit of 1440 cGy to both lungs

• Limit of 2400 cGy to whole brain for children <3 years old with parameningeal tumors and positive CSF cytology (older children receive 3060 cGY)

• Lower daily doses (150 cGy) and total doses (1800 cGy) for large fields such as the whole abdomen.

The following are the radiation therapy guidelines (Table 20-7):

• Clinical group I tumors (TNM stages I and II) do not receive radiation therapy.

• Clinical group I (TNM stage III) and clinical group II tumors (all TMN stages) receive conventional external beam radiation at a total dose of 4140 cGy.

• Clinical group III tumors receive conventional radiation of 5040 cGy.

• Clinical group IV tumors receive conventional radiation of the primary tumor according to the extent of resection of the primary site (clinical group as noted

Table 20-7. Guidelines for Radiotherapy for Rhabdomyosarcoma

TNM stage

Clinical group I

Clinical group II (cGy)

Clinical group III (cGy)

Stage I

No RT

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