Potentially malignant oral disorders in patients with fanconi anemia: A cross-sectional study

Patients diagnosed with Fanconi Anemia present multiple congenital anomalies, pancytopenia, and defective DNA repair mechanisms, making them more susceptible to malignant neoplasia development, with notably higher incidence of oral squamous cell carcinoma. Hematopoietic stem cell transplantation is the definitive treatment for the hematological manifestations but it is considered a factor that significantly increases the risk of malignant transformation. Oral potentially malignant disorders frequently precede the development of oral carcinoma. Given the specific characteristics of this population, there is a critical need to strengthen surveillance programs and screening strategies for these lesions. The aim of this study was to evaluate the overall prevalence of different oral potentially malignant disorders in patients with Fanconi Anemia, without distinguishing among specific subtypes.

This was an observational cross-sectional study conducted with individuals who attended the Dental Clinic in the Onco-Hematology department of the Hospital de Clínicas Complex, Federal University of Paraná from March 2022 to January 2024. Demographic and clinical data were collected from patients with no history of oral squamous cell carcinoma.

The study involved 110 patients of both genders (51.91% men and 49.09% women), with a mean age of 19.96 years. Of these, 40.9% presented at least one oral potentially malignant disorder; the presence of these disorders was significantly associated with having undergone hematopoietic stem cell transplantation. Older patients also had a higher risk of developing these lesions.

These disorders should be regarded as distinct entities in this population, with the risk of malignant transformation potentially being higher than in the general population not affected by Fanconi Anemia.