Fact-sheet: Sacrococcygeal teratoma
Updated on 04/23/2019 at 9:57 AM
View all RADEOS cases associated with this fact-sheet
Definition
Most common benign neonatal tumor: 1/30,000
Female predominance
Embryology:
Sacrococcygeal teratoma (SCT) develops from remnants of the caudal cell mass that failed to regress.
The caudal cell mass has multipotent differentiation potential, accounting for the heterogeneity of tissues within the tumor (bone, teeth, hair, nerves...)
Associated malformations:
- 5 to 26% of cases
- mainly anorectal and genital
Clinical features
Altman classification:
Type I: predominantly external development, minimal presacral component
Type II: more significant endopelvic extension
Type III: internal development with intra-abdominal extension
Type IV: purely internal
External component:
- sacrococcygeal mass
- buttock and Perineum deformity
External component: compression of:
- digestive tract: constipation, obstruction
- urinary tract: dysuria, acute urinary retention
- spinal involvement: lower limb paresis
Ultrasound
Better tumor vascular quantification
CT
To be avoided, ionizing
MRI
Better contrast resolution
Large field of view
Useful for assessing the internal component
Management
Prenatally, screening for signs of fetal cardiac decompensation:
- polyhydramnios
- hydrops fetalis
Complete surgical resection before 6 months of age
In utero therapies under evaluation (coagulation of the vascular pedicle(s))