Fanconi 빈혈 환자에서 발생한 구강 상피세포암과 이시적으로 발생한 간세포암

초록

Fanconi’s anemia (FA) is a rare familiar form of aplastic anemia　(AA) that is associated with increased chromosomal instability and defective DNA repair, and it characterized by progressive pancytopenia, skeletal abnormalities, hyperpigmentation, and an increased risk of leukemia, hepatocellular carcinoma (HCC) and squamous cell carcinoma (SCC), including head and neck SCC. We reported a 31-year-old man who had AA for 17 years and who developed SCC of oral cavity and then metachronously developed HCC. For treatment of his AA, he was given cyclosporin, steroid and androgen in addition to frequent blood transfusion. At the age of 27, he presented with SCC of the maxilla and the right maxillectomy was performed. Four years later in 2003, hepatic resection of the left medial segment of the liver was performed due to hepatocellular carcinoma. Confirmation of FA was done by cytogenetic analysis. The patient died in 2005 of subdural hemorrhage and pneumonia without involvement of the SCC and HCC. (J Korean Surg Soc 2006;70: 148-152)

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