Rhabdomyosarcoma of the head and neck region: experience at the pediatric unit of the Istituto Nazionale Tumori, Milan.
OBJECTIVE
This study aims to describe the clinical features and outcome of pediatric patients with head and neck rhabdomyosarcoma (RMS), which can be divided into parameningeal, orbital, or nonorbital, nonparameningeal.


METHODS
This is a retrospective single-institution analysis.


METHODS
The study was performed at the Istituto Nazionale Tumori, Milan, Italy.


METHODS
The study considered 142 consecutive patients < 21 years treated from 1973 to 2003.


METHODS
Patients were treated using a multimodality approach: complete conservative surgery was performed at diagnosis in only two patients, all patients received chemotherapy, and most (91%) also had radiotherapy. Disease-free survival and overall survival were estimated according to the Kaplan-Meier method.


RESULTS
For the series as a whole, the 5-year disease-free and overall surival rates were 49% and 57%, respectively. The 5-year overall survival rate improved over the years from 38% before 1980 to 78% after 1990; it was 44% for parameningeal, 79% for orbital, and 77% for nonorbital, nonparameningeal RMS.


CONCLUSIONS
The treatment of head and neck RMS is complex and necessarily multidisciplinary. Complete surgery is rarely feasible in the head and neck region. This study confirms that orbital RMS has a favourable outcome, whereas therapeutic results are generally unsatisfactory in parameningeal cases, although recent progress in radiotherapeutic techniques and in the efficacy of chemotherapy would suggest clear improvements in survival.
Meazza
Cristina
C
Pediatric Oncology Unit, Istituto Nazionale Tumori, Milan. cristina.meazza@istitutotumori.mi.it
Ferrari
Andrea
A
Casanova
Michela
M
Massimino
Maura
M
Luksch
Roberto
R
Spreafico
Filippo
F
Cefalo
Graziella
G
Terenziani
Monica
M
Polastri
Daniela
D
Podda
Marta
M
Catania
Serena
S
Cereda
Stefano
S
Gandola
Lorenza
L
Fossati-Bellani
Franca
F
Cantù
Giulio
G
D000293:Adolescent; D002648:Child; D003131:Combined Modality Therapy; D018572:Disease-Free Survival; D005260:Female; D006258:Head and Neck Neoplasms / Q000188:drug therapy / Q000401:mortality* / Q000532:radiotherapy / Q000601:surgery / Q000628:therapy*; D006801:Humans; D008297:Male; D009918:Orbital Neoplasms / Q000401:mortality / Q000628:therapy; D012189:Retrospective Studies; D012208:Rhabdomyosarcoma / Q000188:drug therapy / Q000401:mortality* / Q000532:radiotherapy / Q000601:surgery / Q000628:therapy*; D016019:Survival Analysis