appendiceal from cecal malignant tumors may also yield misclassification across these tumor types. We outlined that clinical histories, including appendix cancer diagnosis, were ascertained from clinician-completed test requisition forms (alongside clinical documentation when provided) so as not to imply that there was universal pathologic annotation of cases. 1 However, we believe that a potential conflation of appendiceal and cecal malignant tumors lends additional support to genetic testing in patients with appendix cancer-particularly those younger than 50 years at diagnosis (early-onset). Genetic testing results could lead to a prompt pathology re-review, which could in turn either prevent misdiagnoses or, if pathology is confirmed, provide a more definitive characterization into the role of germline predisposition in appendix cancer. This highlights the importance of driving efforts forward to improve gene discovery specific to appendix cancer predisposition and to validate our findings, which we mentioned in the article.In light of the potential pathological discordance that complicates a clinical diagnosis of appendiceal cancer, it is also likely that we currently have an incomplete understanding of the true/generalizable population of patients with appendiceal cancer. For now, we highlighted that patients with appendix cancer with a family history of cancer (and potentially a hereditary cancer syndrome) may have been overrepresented in this cohort, who underwent clinical multigene panel testing given the current absence of genetic testing guidelines specific to this population. Moving forward, these topics remain integral to consider in elucidating a potential link to Lynch syndrome and tailoring recommendations for germline testing in this patient population using prospective cohorts of patients with appendix cancer (NCT05734430) that curate clinical, pathological, genetic, biological, and individuallevel information.