• Nir Tsur, Omri Frig, Orna Steinberg-Shemer, Hannah Tamary, Noga Kurman, Aviram Mizrachi, and Aron Popovtzer.
• Departments of Otolaryngology-Head and Neck Surgery.
• Isr Med Assoc J. 2022 Aug 1; 24 (8): 491-496.

BackgroundRecent studies show a high risk of developing malignancy in patients with Fanconi anemia. The most common solid tumor in this condition is head and neck squamous cell carcinoma (HNSCC) and there is often uncertainty and about disease behavior as well as chemotherapy and radiation response.ObjectivesTo describe and characterize HNSCC among Fanconi anemia patients on the Israeli Fanconi Registry.MethodsOur study population included patients in Israel's inherited bone marrow failure registry who were diagnosed with Fanconi anemia between1980 and 2016. Demographic, clinical, and laboratory data were collected from patient charts.ResultsFrom the collected data, HNSCC was confirmed in 6/111 (5.4%) Fanconi anemia patients; 1 (17%) had classic HNSCC risk factors of tobacco abuse and 4 (56%) had undergone primary surgery. The 3 (50%) receiving concurrent chemoradiotherapy had mild side effects, while half developed metachronous primary malignancy, and all developed > 2 primary malignancies. The overall median survival of the patients in our study was 14 (0.5-57) months.ConclusionsFanconi anemia patients have a very high risk of developing HNSCC. Proactive screening for malignancies is needed for the head and neck regions. We also found that chemoradiotherapy can be used safely in high-stage cancers.

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