Abstract
BACKGROUND/OBJECTIVES: Head and neck cancer is one of the most common malignancies in patients with Fanconi anemia (FA), with a greater than 500-fold incidence of head and neck squamous cell carcinoma. Treatment of HNSCC in these patients is particularly challenging because of poor tolerance to radiation therapy and to some chemotherapeutic agents such as cisplatin. For these reasons, new research is needed to determine the best treatment course for these patients. With this goal in mind, we assessed the characteristics of head and neck squamous cell carcinoma (SCC) in Fanconi anemia (FA). SUBJECTS AND METHODS: Data for 11 patients (mean age 31 years) with head and neck SCC and FA attended to between 2005 and 2021 were analyzed. RESULTS: The primary tumor site was the oral cavity in eight patients, and five had advanced stages. All patients underwent primary tumor resection. Four patients received adjuvant radiotherapy (mean 57.2 Gy), but three developed toxicity. The mean follow-up was 48.4 months. Six patients experienced 19 episodes of primary tumor recurrence and five developed secondary head and neck tumors. The 3-year disease-free survival was 47%, and the 5-year cause-specific survival was 48%. These findings are similar to the data for 72 patients collected from the literature. CONCLUSIONS: The prognosis of head and neck cancer in patients with Fanconi anemia is poor, with an overall survival lower than 50% at 5 years.