Malignant transformation in sacrococcygeal teratoma and in presacral teratoma associated with Currarino syndrome: a comparative study.
OBJECTIVE
The risk of malignant transformation of sacrococcygeal teratoma (SCT) and of presacral teratoma in Currarino syndrome (CS) may differ despite the similar position and appearance.


METHODS
Malignant transformation and teratoma recurrence were assessed in a national retrospective comparative analysis of 205 SCT and 16 CS patients treated in one of the six pediatric surgical centers in the Netherlands between January 1981 and December 2010.


RESULTS
The malignancy free survival of patients with SCT was lower than for patients with a presacral teratoma associated with CS (80% and 58% after one and two years in SCT versus 100% after two years in CS, p=0.017) CONCLUSIONS: In SCT, malignancy and recurrence risk are high. Therefore, early and complete resection is mandatory. Our data show that the risk of malignant transformation of a presacral teratoma in CS is small.
Department of Pediatric Surgery, Maastricht University Medical Centre, The Netherlands.
Marc
M
Dirix
Department of Pediatric Surgery, Maastricht University Medical Centre, The Netherlands.
Tine
T
van Becelaere
Department of Pediatric Surgery, Maastricht University Medical Centre, The Netherlands.
Lizanne
L
Berkenbosch
Department of Pediatric Surgery, University Medical Centre Groningen, The Netherlands.
Robertine
R
van Baren
Department of Pediatric Surgery, Erasmus Medical Centre-Sophia Children's Hospital, Rotterdam, The Netherlands.
Rene M
RM
Wijnen
Department of Pediatric Surgery, Radboud University Medical Centre, Nijmegen, The Netherlands.
Marc H
MH
Wijnen
Department of Pediatric Surgery, Wilhelmina Children's Hospital, University Medical Centre, Utrecht, The Netherlands.
David C
DC
van der Zee
Pediatric Surgical Centre of Amsterdam (Emma Children's Hospital AMC/VU University Medical Centre), The Netherlands.
Hugo A
HA
Heij
Department of Pediatric Surgery, Maastricht University Medical Centre, The Netherlands.
Joep P M
JP
Derikx
Department of Pediatric Surgery, Maastricht University Medical Centre, The Netherlands. Electronic address: e.van.heurn@mumc.nl.
L W Ernest
LW
van Heurn
D000293:Adolescent; D001003:Anal Canal / Q000002:abnormalities*; D002648:Child; D002675:Child, Preschool; D004065:Digestive System Abnormalities; D005260:Female; D006801:Humans; D007231:Infant, Newborn; D008297:Male; D009364:Neoplasm Recurrence, Local / Q000401:mortality / Q000473:pathology*; D009426:Netherlands; D012007:Rectum / Q000002:abnormalities*; D012189:Retrospective Studies; D012445:Sacrococcygeal Region / Q000473:pathology; D012447:Sacrum / Q000002:abnormalities*; D013125:Spinal Neoplasms / Q000401:mortality / Q000473:pathology* / Q000601:surgery; D013595:Syringomyelia; D013724:Teratoma / Q000401:mortality / Q000473:pathology* / Q000601:surgery
Currarino syndrome; Malignancy; Sacrococcygeal teratoma