[Rhabdomyosarcoma of the orbits in childhood].
BACKGROUND
Rhabdomyosarcoma is the most common malignant orbital tumor in children. Treatment modalities (individualized therapy or study protocol) have been changed radically. Surgery was supplemented by radiation therapy and chemotherapy. The objective of our retrospective analysis was to define the prognosis in correlation to changes of treatment in an unselected patient group of a single institution.


METHODS
Between 1954 and 1995, 18 patients (age at presentation 1 month to 17 years, 11 male and 7 females) with orbital rhabdomyosarcoma were diagnosed and treated at our institution.


RESULTS
The 5-year survival rate was 76%. Primary exenteration was replaced by tumor resection (microsurgery), radiation therapy and chemotherapy. Favorable outcome in the precytostatic era (with permanent loss of function) was not significantly improved by polychemotherapy. Microsurgery seems to increase the risk of recurrence.


CONCLUSIONS
Considering the high risk of recurrence, primary exenteration in selected patients with expanded primary tumor is still a therapeutic option.
Humpl
T
T
Kinderklinik, Klinikum der Johannes Gutenberg-Universität Mainz.
Brühl
K
K
Pitz
S
S
de Mooy
P
P
Bartels
U
U
Gutjahr
P
P
D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D003131:Combined Modality Therapy; D005260:Female; D006801:Humans; D007223:Infant; D008297:Male; D009364:Neoplasm Recurrence, Local / Q000175:diagnosis / Q000473:pathology / Q000628:therapy; D009367:Neoplasm Staging; D017233:Orbit Evisceration; D009918:Orbital Neoplasms / Q000175:diagnosis / Q000473:pathology / Q000628:therapy*; D011379:Prognosis; D012189:Retrospective Studies; D012208:Rhabdomyosarcoma / Q000175:diagnosis / Q000473:pathology / Q000628:therapy*
Rhabdomyosarkom der Orbita im Kindesalter.