Why do posterior fossa dermoid tumors happen more frequently in Klippel-Feil syndrome?
Non-cancerous posterior fossa dermoid tumors (PFDT) are frequently seen in people with Klippel-Feil syndrome (KFS). The main theories to explain why KFS and PFDT appear more frequently together relate to the early development of the spine around the 6-7th week of pregnancy. Scientists believe that, through one of several mechanisms, some cells meant to form skin do not separate or move correctly and become trapped near the cells forming the fused vertebrae. These trapped skin cells form the PFDTs.
SOURCE: Emory University - Department of Human Genetics in collaboration with ThinkGenetic • https://www.thinkgenetic.com/diseases/klippel-feil-syndrome-109938/living-with/67683 • DATE UPDATED: 2019-07-26
Adorno, A., Alafaci, C., Sanfilippo, F., Cafarella, D., Scordino, M., Granata, F., Grasso, G., ... Salpietro, F. M. (2015). Malignant teratoma in Klippel-Feil syndrome: a case report and review of the literature. Journal of medical case reports, 9, 229. doi:10.1186/s13256-015-0700-y
Turgut M. Klippel-Feil syndrome in association with posterior fossa dermoid tumour. Acta Neurochir (Wien). 2009 Mar;151(3):269-76. doi: 10.1007/s00701-009-0203-y. Epub 2009 Feb 20. Review. PubMed PMID: 19229468.