Τρίτη 6 Μαρτίου 2018

Oral Cancer in Fanconi Anemia: Review of 121 cases

Publication date: Available online 6 March 2018
Source:Critical Reviews in Oncology/Hematology
Author(s): Camila Pinheiro Furquim, Allana Pivovar, José Miguel Céspedes Amenábar, Carmem Bonfim, Cassius Carvalho Torres-Pereira
Fanconi anemia (FA) is a rare autosomal recessive genetic disorder characterized by aplastic anemia, progressive pancytopenia, congenital anomalies, and increased risk of cancer development. After hematopoietic stem cell transplant (HSCT), patients have an estimated 500-fold increase in the risk of developing head and neck cancer compared to a normal population, and the oral cavity is affected in one-third of cases. Thus, this study aimed to better understand the natural history of oral cavity cancer in patients affected by FA. After conducting a keyword search on MEDLINE, we found 121 cases of oral cavity cancer in patients who had been affected by FA. In conclusion, HSCT may increase the risks of oral cancer development, especially after 5 years after the transplant. In the normal population, the tongue is the most affected area. FA patients should be informed of the risks of oral malignant transformation and encouraged to be under ongoing medical surveillance.



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