Cancer of the Appendix: Very Rare, But Genes May Play Role

"While there is still much to learn from our discovery, we have found the tip of an iceberg -- potentially a really big iceberg," Holowatyj said in a center news release. Historically, experts have not thought this cancer, which affects about one or two people in 1 million annually, was hereditary. The fact that it's rare has made it even harder to understand and study.