The Role of Congenital Immunodeficiency Syndromes in Tumor Pathophysiology

Annotation: This article reviews the contribution of congenital immunodeficiency syndromes to tumor development and tumor behavior. Inherited defects in lymphocyte development, DNA repair, antigen presentation, cytokine signaling, and immune regulation weaken immune surveillance and permit accumulation of oncogenic lesions, persistent viral infection, chronic inflammation, and impaired elimination of transformed cells. These mechanisms are particularly relevant for lymphoid neoplasms, virus-associated tumors, and malignancies arising in syndromes such as severe combined immunodeficiency, common variable immunodeficiency, Wiskott-Aldrich syndrome, ataxia-telangiectasia, Nijmegen breakage syndrome, and related inborn errors of immunity. Understanding these links is essential for early recognition, risk stratification, and tailored oncologic management.