A multimodal strategy based on surgery, radiotherapy, ICE regimen and high dose chemotherapy in atypical teratoid/rhabdoid tumours: a single institution experience.
OBJECTIVE
Atypical Teratoid/Rhabdoid Tumour is a rare and aggressive childhood tumour. The outcome of a series treated with the same multimodal strategy was reported.


METHODS
The patients were treated with surgery, 2 courses of ifosfamide/carboplatin/etoposide(ICE), 2 courses of cyclophosphamide/etoposide/carboplatino/thiotepa (CECAT) or 2 other ICE courses, high dose chemotherapy (HDC) and radiotherapy.


RESULTS
Eight patients underwent primary surgery achieving a complete removal in 3. Progressive disease (PD) occurred in 2/8 patients during ICE courses and in 3/4 during CECAT courses. After 4 courses 5 patients presented a PD. HDC was performed in 3 patients followed by local radiotherapy. The Kaplan Meier OS and EFS probability at 5 years are, respectively, 50% (CI 11-80%) and 33% (CI 6-66%).


CONCLUSIONS
A strategy based on surgery, including a second surgical look, and on radiotherapy appears the best option. ICE regimen and HDC correlate with good prognosis in some patients but this approach needs further evaluation.
Fidani
Paola
P
Division of Pediatric Oncology Unit, Ospedale Pediatrico Bambino Gesù, Piazza S.Onofrio 4, Rome, 00100, Italy. fidani@opbg.net
De Ioris
Maria Antonietta
MA
Serra
Annalisa
A
De Sio
Luigi
L
Ilari
Ilaria
I
Cozza
Raffaele
R
Boldrini
Renata
R
Milano
Giuseppe Maria
GM
Garrè
Maria Luisa
ML
Donfrancesco
Alberto
A
D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D001932:Brain Neoplasms / Q000628:therapy*; D016190:Carboplatin / Q000008:administration & dosage; D002648:Child; D002675:Child, Preschool; D003131:Combined Modality Therapy; D003520:Cyclophosphamide / Q000008:administration & dosage; D005047:Etoposide / Q000008:administration & dosage; D005260:Female; D006801:Humans; D007069:Ifosfamide / Q000008:administration & dosage; D007223:Infant; D053208:Kaplan-Meier Estimate; D008279:Magnetic Resonance Imaging; D008297:Male; D019635:Neurosurgical Procedures; D011878:Radiotherapy; D018335:Rhabdoid Tumor / Q000401:mortality / Q000628:therapy*; D013724:Teratoma / Q000401:mortality / Q000628:therapy*; D013852:Thiotepa / Q000008:administration & dosage; D014057:Tomography, X-Ray Computed
D005047:Etoposide; D003520:Cyclophosphamide; D013852:Thiotepa; D016190:Carboplatin; D007069:Ifosfamide