Oral squamous cell carcinoma in Fanconi anemia: report of four cases

Summary: Four FA patients who are seen regularly at the University of Minnesota Medical Center and subsequently developed OSCC were selected. Clinical and histological features of oral lesions were systematically documented over time. Results: Two males and 2 females with FA successfully underwent a HCT for severe aplastic anemia (n=3) and advanced myelodysplastic syndrome (n=1). OSCC diagnosis was made 1, 4, 13 and 14 years after transplant. In 3 of 4 cases, abnormal lesions were monitored for a prolonged period of time before SCC was considered. SCCs were found in the sublingual space (n=1), lateral tongue (n=2), and gingiva (n=1). Two had metastatic disease at diagnosis and died rapidly. No patient had evidence of HPV and none had typical OSCC risk factors. Conclusions: FA patients develop oral cancer at an extremely high rate and at a much younger age than non FA patients. Lower threshold for biopsy and histological evaluation should be considered. Chemoprevention strategies could be considered well before the development of dysplasia.