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

S A L Bartels1, P J van Koperen, A F W van der Steeg

  • 1Department of Surgery, Academic Medical Center, Amsterdam, the Netherlands.

Colorectal Disease : the Official Journal of the Association of Coloproctology of Great Britain and Ireland
|May 19, 2010
PubMed

Insights

Congenital presacral tumors in children are often mature teratomas, sometimes associated with Currarino syndrome. Early surgery is crucial for children over one year due to malignancy risk.

Area of Science:

  • Pediatric Surgery
  • Oncology
  • Genetics

Background:

  • Presacral tumors in children are rare congenital anomalies.
  • Malignant transformation risk necessitates understanding tumor types and patient age.

Purpose of the Study:

  • Investigate malignant changes in pediatric presacral tumors.
  • Analyze risk factors including age, presentation, and tumor origin.

Main Methods:

  • Retrospective review of 17 pediatric patients surgically treated for congenital presacral masses.
  • Analysis over a 22-year period.

Main Results:

  • Constipation was the primary symptom in 82% of patients.
  • Mature teratoma (64%) was the most common, with 3 malignancies.
  • Currarino syndrome identified in 71% of patients; HLXB9 mutations found in 29%.

Conclusions:

  • Congenital presacral tumors are predominantly mature teratomas, often linked to Currarino syndrome.
  • Increased malignancy risk in children over one year old mandates prompt surgical intervention.
Abstract

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