Presacral masses in children: presentation, aetiology and risk of malignancy.
OBJECTIVE
The risk of malignant changes in presacral tumours in children was investigated in relation to age at diagnosis, type of presentation and origin of the tumour.


METHODS
A retrospective review was carried out in 17 patients surgically treated for congenital presacral masses over a 22-year period.


RESULTS
Constipation was the main symptom in 14 (82%) of 17 patients. The lesions were evident on digital examination in 14 patients. Mature teratoma (n = 9, 64%) was the most common lesion, including three malignancies. Currarino syndrome was diagnosed in 10 (71%) patients. Two unclassified variant HLXB9 gene mutations were found in five (29%) patients who underwent genetic testing.


CONCLUSIONS
Congenital presacral tumours in children were mostly mature teratomas, either as sacrococcygeal teratomas or as part of the Currarino syndrome. The risk of malignancy in patients older than 1 year necessitates early surgical resection.
Bartels
S A L
SA
Department of Surgery, Academic Medical Center, Amsterdam, the Netherlands.
van Koperen
P J
PJ
van der Steeg
A F W
AF
Deurloo
E E
EE
Bemelman
W A
WA
Heij
H A
HA
D000328:Adult; D001003:Anal Canal / Q000002:abnormalities / Q000473:pathology / Q000601:surgery; D002675:Child, Preschool; D003248:Constipation / Q000209:etiology; D003672:Defecation; D004065:Digestive System Abnormalities / Q000150:complications / Q000473:pathology* / Q000601:surgery*; D005242:Fecal Incontinence / Q000209:etiology; D005260:Female; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D012007:Rectum / Q000002:abnormalities / Q000473:pathology / Q000601:surgery; D012189:Retrospective Studies; D012445:Sacrococcygeal Region / Q000473:pathology*; D012447:Sacrum / Q000002:abnormalities / Q000473:pathology / Q000601:surgery; D013595:Syringomyelia / Q000150:complications / Q000151:congenital / Q000473:pathology* / Q000601:surgery*; D013724:Teratoma / Q000150:complications / Q000151:congenital / Q000473:pathology* / Q000601:surgery*; D055815:Young Adult