Childhood pineoblastoma: experiences from the prospective multicenter trials HIT-SKK87, HIT-SKK92 and HIT91.
OBJECTIVE
To analyze the outcome of children with pineoblastoma (PB), treated within the prospective multicenter trials HIT-SKK87, HIT-SKK92 and HIT91 of German-speaking countries.


METHODS
We report on 11 children suffering from PB. Five children younger than 3 years of age received chemotherapy after surgery until eligible for radiotherapy (HIT-SKK87 and HIT-SKK92). Five of six children older than 3 years were treated after surgery with immediate chemotherapy and craniospinal irradiation, and one child received maintenance chemotherapy after postoperative radiotherapy (HIT91).


RESULTS
Five of the six older children are still alive in continuous complete remission (CCR) with a median overall survival (OS) and progression free survival (PFS) of 7.9 years. Five of these six HIT91 patients responded to postoperative chemotherapy and radiotherapy. The only patient with tumor progression during initial chemotherapy achieved complete remission with radiotherapy and is alive. In contrast, all five young children died of tumor progression after a median OS of 0.9 years (PFS 0.6 years). They had either metastatic disease (M1) and/or postoperative residual tumor. Response to postoperative chemotherapy was lower than in the older age group, and only one of these children received radiotherapy.


CONCLUSIONS
Combined chemotherapy and radiotherapy were feasible and effective in the older age group, leading to prolonged remissions in five of six children. Tumor biology may be more aggressive in younger children with PB, who presented more frequently with high-risk features at diagnosis and had poorer response rates to neoadjuvant postoperative chemotherapy. More intensified treatment regimens may be needed for young children with PB.
Hinkes
Bernward G
BG
Children's University Hospital, University of Wuerzburg, Josef-Schneider-Str. 2, 97080, Wuerzburg, Germany.
von Hoff
Katja
K
Deinlein
Frank
F
Warmuth-Metz
Monika
M
Soerensen
Niels
N
Timmermann
Beate
B
Mittler
Uwe
U
Urban
Christian
C
Bode
Udo
U
Pietsch
Torsten
T
Schlegel
Paul G
PG
Kortmann
Rolf D
RD
Kuehl
Joachim
J
Rutkowski
Stefan
S
D000293:Adolescent; D000328:Adult; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D001932:Brain Neoplasms / Q000188:drug therapy* / Q000473:pathology / Q000532:radiotherapy*; D002648:Child; D002675:Child, Preschool; D003131:Combined Modality Therapy; D005260:Female; D006801:Humans; D008297:Male; D010870:Pineal Gland / Q000473:pathology*; D010871:Pinealoma / Q000188:drug therapy* / Q000473:pathology / Q000532:radiotherapy*; D011446:Prospective Studies; D016896:Treatment Outcome