Improved survival for children with parameningeal rhabdomyosarcoma: results from the AIEOP soft tissue sarcoma committee.
BACKGROUND
Parameningeal rhabdomyosarcoma (PM-RMS) is a rare, highly malignant pediatric tumor arising from locations adjacent to the meninges, from where it can spread intracranially.


METHODS
We reviewed 109 children with non-metastatic PM-RMS enrolled in the Italian RMS79, RMS88 and RMS96 protocols over a 24-year period. All patients received intensive chemotherapy and standard or hyperfractionated and accelerated radiotherapy. Some had delayed surgery.


RESULTS
Five-year overall survival rose from 40% in the RMS79 to 72% in the RMS88 and RMS96 protocols (P = 0.01), where more intensive chemotherapy and hyperfractionated accelerated radiotherapy (HART) was used. Delayed surgery after initial treatment was statistically associated with a better prognosis. Unfavorable tumor characteristics for RMS arising in other sites, for example, histology, invasiveness or node involvement, did not predict outcome for PM-RMS.


CONCLUSIONS
Outcome in PM-RMS patients enrolled in three consecutive Italian protocols has progressively improved, as a result of intensive chemotherapy, delayed surgery and, possibly, HART, though improved imaging and radiotherapeutic tools may have had a role as well.
Bisogno
Gianni
G
Division of Hematology/Oncology, Department of Pediatrics, University Hospital of Padova, Padova, Italy. gianni.bisogno@unipd.it
De Rossi
Costanza
C
Gamboa
Yéssika
Y
Sotti
Guido
G
Ferrari
Andrea
A
Dallorso
Sandro
S
Donfrancesco
Alberto
A
Cecchetto
Giovanni
G
Calderone
Milena
M
Gandola
Lorenza
L
Rosolen
Angelo
A
Carli
Modesto
M
D000293:Adolescent; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D002648:Child; D002675:Child, Preschool; D003131:Combined Modality Therapy; D005260:Female; D006258:Head and Neck Neoplasms / Q000401:mortality / Q000628:therapy; D006801:Humans; D007223:Infant; D008297:Male; D008577:Meningeal Neoplasms / Q000401:mortality* / Q000628:therapy; D011379:Prognosis; D012208:Rhabdomyosarcoma / Q000401:mortality* / Q000628:therapy; D015996:Survival Rate