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.
Meazza
Cristina
C
Pediatric Oncology Unit, Istituto Nazionale Tumori, Via G. Venezian, 1-20133 Milano MI, Italy.
Ferrari
Andrea
A
Casanova
Michela
M
Gandola
Lorenza
L
Collini
Paola
P
Massimino
Maura
M
Luksch
Roberto
R
Spreafico
Filippo
F
Cefalo
Graziella
G
Polastri
Daniela
D
Terenziani
Monica
M
Podda
Marta
M
Cantù
Giulio
G
Scaramuzza
Davide
D
Fossati-Bellani
Franca
F
D000293:Adolescent; D000328:Adult; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D001932:Brain Neoplasms / Q000517:prevention & control; D017024:Chemotherapy, Adjuvant; D002648:Child; D002675:Child, Preschool; D016371:Cranial Irradiation; D019583:Dose Fractionation, Radiation; D005260:Female; D006801:Humans; D007223:Infant; D007558:Italy / Q000453:epidemiology; D008297:Male; D008577:Meningeal Neoplasms / Q000401:mortality / Q000556:secondary / Q000628:therapy*; D009364:Neoplasm Recurrence, Local; D009382:Neoplasms, Unknown Primary / Q000401:mortality / Q000628:therapy*; D018714:Radiotherapy, Adjuvant; D012208:Rhabdomyosarcoma / Q000401:mortality / Q000556:secondary / Q000628:therapy*; D016896:Treatment Outcome