"Embryonal histology. •"Alveolar histology.

"Embryonal histology. •"Alveolar histology.

above). Patients also receive 5040 cGy to metastatic sites. One exception is that patients with alveolar histology and complete resection of the primary tumor (clinical group I, stages II and II) should receive 3600 cGy to the primary.

• Clinical group IV metastic tumor sites (TNM stage IV, metastases) receive a total dose of 5040 cGy.

The timing of radiation therapy is as follows:

1. Patients receive radiotherapy to the primary tumor and metastatic sites at week 9 (day 62).

2. Patients with parameningeal tumors with evidence of meningeal extension begin radiation to the primary tumor on day 0. Patients with parameningeal tumors without evidence of meningeal extension will receive radiation at week 9.

3. Patients who require emergency radiotherapy (e.g., spinal cord compression) receive radiation on day 0. The entire course of radiation therapy should be administered at this time.


All patients regardless of their initial stage or group receive combination chemotherapy. The effective agents in rhabdomyosarcoma are cyclophosphamide, ifosfamide, melphalan, actinomycin D, Adriamycin, cisplatin, carboplatin, vincristine, etopo-side, topotecan, and irinotecan.

Treatment Regimens

Table 20-8 shows recommendations for therapy of rhabdomyosarcoma and undifferentiated sarcoma of childhood. Figure 20-1 shows the chemotherapy protocol for stage I, clinical group I paratesticular rhabdomyosarcoma and clinical groups I and II orbit and eyelid rhabdomyosarcoma. Figure 20-2 shows the chemotherapy regimens for other stage I, II, III, and IV disease.

Granulocyte colony-stimulating factor (G-CSF) is incorporated into these protocols to shorten the periods of neutropenia, which follow these myelosuppressive combinations.

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