Evolving treatment strategies for parameningeal rhabdomyosarcoma: the experience of the Istituto Nazionale Tumori of Milan.

BACKGROUND: Ninety pediatric patients with parameningeal rhabdomyosarcoma (PM-RMS) were treated between 1970 and 2002 at the Istituto Nazionale Tumori, Milan. METHODS: Combined treatment was implemented in high-risk patients with whole-brain irradiation and intrathecal chemotherapy between 1978 and 1981 and intrathecal methotrexate alone between 1982 and 1987. Radiotherapy was given with conventional fractionation until 1988 and hyperfractionated accelerated radiotherapy (HART) thereafter. RESULTS: The 5-year disease-free survival was 41.3%, increasing from 15.4% (1970-1977) to 55.9% (1989-2002) for localized cases. Local failure rates were 41% and 24% with conventional fractionation and HART, respectively. In patients treated with HART, acute toxicities were more pronounced, but the treatment compliance was better. Severe radiation-related late sequelae were observed. CONCLUSIONS: The outcome for patients with PM-RMS has improved over the years but is still not as satisfactory as that for patients with RMS of other sites. Thanks to improvements in radiotherapy techniques and chemotherapy efficacy, meningeal prophylaxis is no longer necessary. Although more evidence is needed to confirm our data, the results obtained with twice-daily HART seem promising.