The advent of molecular biology has provided tools to delineate genetic mut
ations that cause disease. Recently, several genetic mutations have been as
sociated with intramedullary spinal cord tumors. Concurrently, advances in
micro-neurosurgical techniques have significantly decreased the morbidity o
f surgical resection. In this review, we describe the current understanding
of genetic mutations in sporadic and familial intramedullary spinal cord t
umors. The future success of innovative gene therapy protocols may depend u
pon establishing a cause and effect relationship between these genetic muta
tions and disease progression. Successful gene therapy will also depend upo
n increasing the efficiency of gene therapy vector delivery.