Perioperative intensity-modulated brachytherapy for refractory orbital rhabdomyosarcomas in children.
OBJECTIVE
To evaluate the feasibility and toxicity of perioperative intensity-modulated brachytherapy (IMBT) as well as functional outcome in children with therapy-refractory orbital rhabdomyosarcomas (RMS).


METHODS
Since 1993, children with therapy-refractory orbital RMS have been treated by a multidisciplinary approach combining function-preserving, mostly R1 tumor resection and perioperative IMBT at the University Hospital of Schleswig-Holstein, Germany. All children with orbital RMS, who were enrolled in this multidisciplinary treatment protocol between 1993 and 2002, were prospectively assessed with respect to evaluation of side effects and functional outcome.


RESULTS
Ten children (six boys, four girls) were included. Median age was 6.5 years (range, 1-19 years) at the beginning of our treatment and 6.0 years (range 1-17 years) at diagnosis. All children were in Intergroup Rhabdomyosarcoma Study Group (IRSG) group III and had embryonal subtype. Estimated 5-year survival was 62% +/- 18%. There was no radiation-related toxicity grade 3 or 4 observed. The eyes were primarily preserved in all cases. One child underwent secondary orbital exenteration 10 months after completion of IMBT. Visual acuity could be preserved apart from one child developing significant visual deterioration due to radiation cataract grade 2. The cosmetic results were good or very good in eight and moderate in two children. Four children died of their disease.


CONCLUSIONS
This interdisciplinary, individually tailored and function-preserving treatment procedure has proven to be a well-tolerated therapeutic option in cases with refractory orbital RMS. It provides both improvement of local tumor control and quality of life.
Strege
Rainer Joachim
RJ
Department of Neurosurgery, University Clinic Schleswig-Holstein, Campus Kiel, Germany.
Kovács
György
G
Meyer
Jens Eduard
JE
Holland
Detlef
D
Claviez
Alexander
A
Mehdorn
Maximilian H
MH
D000293:Adolescent; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D001918:Brachytherapy / Q000379:methods*; D017024:Chemotherapy, Adjuvant; D002648:Child; D002675:Child, Preschool; D003131:Combined Modality Therapy; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007223:Infant; D008297:Male; D009364:Neoplasm Recurrence, Local / Q000188:drug therapy / Q000473:pathology / Q000532:radiotherapy* / Q000601:surgery; D009915:Orbit / Q000528:radiation effects / Q000601:surgery; D009918:Orbital Neoplasms / Q000188:drug therapy / Q000473:pathology / Q000532:radiotherapy* / Q000601:surgery*; D010348:Patient Care Team; D011446:Prospective Studies; D011832:Radiation Injuries / Q000209:etiology; D011880:Radiotherapy Planning, Computer-Assisted / Q000379:methods; D050397:Radiotherapy, Intensity-Modulated / Q000379:methods*; D018233:Rhabdomyosarcoma, Embryonal / Q000188:drug therapy / Q000473:pathology / Q000532:radiotherapy* / Q000601:surgery*; D055815:Young Adult