Presacral masses in children: presentation, aetiology and risk of malignancy.

Abstract

AIM:

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.

METHOD:

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.

CONCLUSION:

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.

• Authors: S A L Bartels, P J van Koperen, A F W van der Steeg, E E Deurloo, W A Bemelman, H A Heij
• Journal: Colorectal disease : the official journal of the Association of Coloproctology of Great Britain and Ireland (Colorectal Dis) Vol. 13 Issue 8 Pg. 930-4 (Aug 2011) ISSN: 1463-1318 [Electronic] England
• PMID: 20478006 (Publication Type: Journal Article)
• Copyright: © 2011 The Authors. Colorectal Disease © 2011 The Association of Coloproctology of Great Britain and Ireland.

Topics

• Adult
• Anal Canal (abnormalities, pathology, surgery)
• Child, Preschool
• Constipation (etiology)
• Defecation
• Digestive System Abnormalities (complications, pathology, surgery)
• Fecal Incontinence (etiology)
• Female
• Humans
• Infant
• Infant, Newborn
• Male
• Rectum (abnormalities, pathology, surgery)
• Retrospective Studies
• Sacrococcygeal Region (pathology)
• Sacrum (abnormalities, pathology, surgery)
• Syringomyelia (complications, congenital, pathology, surgery)
• Teratoma (complications, congenital, pathology, surgery)
• Young Adult