What are the management and outcomes for Fanconi anemia (FA) patients with head and neck squamous cell carcinoma (HNSCC)?
Background: FA is a rare recessive disorder characterized clinically by bone marrow failure that can affect any of the hematopoietic lineages, along with congenital malformations, sensitivity to DNA cross-linking agents, and increased malignancy risk. In particular, HNSCCs are significantly more common in FA patients relative to the general population. Because hematopoietic stem cell transplantation (HSCT), preleukemia detection, leukemia therapies, and other advances have improved FA patient survival, an increasing number of these patients are developing HNSCCs. Management is often difficult because of sensitivity to both radiation therapy and chemotherapy agents.
Explore this issue:April 2016
Study design: Cohort study of 35 FA patients with HNSCC over a 10-year period.
Setting: International Fanconi Anemia Registry (IFAR).