Graduate Program in Dentistry, Departament of Stomatology, Federal University of Paraná School of Dentistry, Curitiba, Paraná, Brazil.
Bone Marrow Transplantation Unit, Hospital de Clínicas, Federal University of Paraná, Curitiba, Paraná, Brazil.
Crit Rev Oncol Hematol. 2018 May;125:35-40. doi: 10.1016/j.critrevonc.2018.02.013. Epub 2018 Mar 6.
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 non-affected, 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 undergo medical surveillance.
范可尼贫血（FA）是一种罕见的常染色体隐性遗传疾病，其特征为再生障碍性贫血、进行性全血细胞减少、先天畸形和癌症发展风险增加。造血干细胞移植（HSCT）后，与非受影响者相比，患者发生头颈部癌症的风险估计增加了 500 倍，三分之一的病例会累及口腔。因此，本研究旨在更好地了解 FA 患者口腔癌的自然史。通过在 MEDLINE 上进行关键词搜索，我们发现了 121 例口腔癌病例发生在 FA 患者中。总之，HSCT 可能会增加口腔癌发展的风险，尤其是在移植后 5 年之后。在普通人群中，最易受影响的部位是舌头。FA 患者应被告知口腔恶性转化的风险，并鼓励他们进行医学监测。
