The incidence of associated abnormalities in patients with sacrococcygeal teratoma
Background: Gross genetic causes for SCT are unknown; however, it might be associated with other abnormalities. We assessed the incidence of associated abnormalities in a large national cohort of neonates with SCT and aimed to identify predictive risk factors.
Procedure: The medical records were reviewed of 235 consecutive neonates with SCT treated at the six pediatric surgical centers in the Netherlands from 1970 to 2010. Potential risk factors for associated abnormalities analyzed included sex, gestational age, tumor-volume/histology and Altman-classification.
Results: In 76 patients (32.3%) at least one associated abnormality was diagnosed, with hydronephrosis as the most common (16.2%) and hip dysplasia in 4.3%. Multiple abnormalities were documented for 21 (9.0%). Prematurity and Altman type IV SCT were associated with an increased risk of any associated abnormality. No association between increased tumor-volume and hydronephrosis or hip dysplasia was found. Patients with type IV Altman SCT had a fourfold risk of suffering from hydronephrosis compared to Altman type I SCT.
Conclusions: SCT was associated with other abnormalities in one-third of children. Some were tumor-related while others were related to prematurity or occurred sporadically. In contrast to clinically obvious anomalies, hip dysplasia or hydronephrosis might be latently present with more subtle clinical presentation. We therefore suggest renal- and hip-ultrasound in all patients, certainly those with Altman type IV SCT.
Level of evidence rating: Level II (retrospective study)
|Keywords||Germ cell tumor, Sacrococcygeal teratoma, Congenital anomalies|
|Persistent URL||dx.doi.org/10.1016/j.jpedsurg.2018.01.013, hdl.handle.net/1765/113901|
|Journal||Journal of Pediatric Surgery|
Kremer, M.E.B, Althof, J.F, Derikx, J.P.M, van Baren, R, Heij, H.A, Wijnen, M.H.W.A, … van Heurn, L.W.E. (2018). The incidence of associated abnormalities in patients with sacrococcygeal teratoma. Journal of Pediatric Surgery, 53(10), 1918–1922. doi:10.1016/j.jpedsurg.2018.01.013