Colonic mature cystic teratoma

Abstract Aim of the study We would like to report an unusual case of mature cystic teratoma (MCT) of the left colon presenting with intestinal obstruction in a syndromic patient with low-anorectal malformation (ARM). Case report The boy had multiple malformations: low ARM with perineal fistula, situs ambiguous, dextrocardia, double outlet right ventricle with pulmonary stenosis and anterior aorta. At two years he required cardiac surgery but he developed intestinal obstruction. The intestinal condition put us in a diagnostic challenge: we thought about intestinal stenosis with fecal impaction but radiological investigations were inconclusive. During surgery we identified an abdominal mass that was resected together with the adjacent descending colon. Histology showed MCT. Follow-up was uneventful. Conclusions MCT rarely occurs outside the sacrococcygeal–gonadal location. They are frequently associated with ARM in Currarino Syndrome: the mass is located in front of the sacrum and causes anal stenosis. Nevertheless, in the reported patient, MCT did not arise from the spine but from the left colon. Paediatric cases of colonic MCT have never been reported. Affected adults can develop constipation and/or intestinal obstruction. Surgery permits to remove the mass in both symptomatic and asymptomatic patients.