Evidence of an Inherited Predisposition for Spinal Cord Tumors

STUDY DESIGN: Retrospective study. OBJECTIVES: To determine familial clustering of primary spinal cord tumors using a statewide genealogy database. METHODS: The Utah Population Database (UPDB) was queried using ICD-Oncology (International Classification of Diseases for Oncology) codes for primary spinal cord tumors. The hypothesis of disproportionate familial clustering was tested using the Genealogical Index of Familiality (GIF). The relative risks (RRs) in relatives were calculated using the ratio of observed spinal cord tumors to expected spinal cord tumors in relatives using estimated rates from the UPDB. The related clusters of spinal cord cancer cases with a significant excess number of spinal cord cancer cases descending from a common founder pair were identified using internal UPDB rates. RESULTS: The analysis of the GIF for individual with tumors of the spinal cord showed excess close and distant relatedness (case GIF = 3.82; control mean GIF = 2.68; P = .068). Excess relatedness for spinal cord cancers was observed when only more distant relationships were considered (P = .019). The RRs for spinal cord tumors were elevated in second- and third-degree relatives but this did not reach statistical significance (RR = 2.9, P = .15, and RR = 2.0, P = .14). Multiple extended pedigrees with a significant excess of spinal cord cancer cases among the descendants were identified. CONCLUSIONS: The excess relatedness of tumor cases over controls in distant relationships, the higher RRs to distant relatives, and the discovery of high-risk pedigrees all suggest a familial predisposition to the development of spinal cord tumors.