J Korean Surg Soc.  2006 Feb;70(2):148-152.

Squamous Cell Carcinoma of Oral Cavity and Metachronously Developed Hepatocellular Carcinoma Arising from Fanconi's Anemia

• Min JS
• Park JH
• Shin DG
• Kim IS

Abstract

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.

Keyword

Fanconi's anemia; Aplastic anemia; Hepatocellular carcinoma; Squamous cell carcinoma

MeSH Terms

Adult
Anemia, Aplastic
Blood Transfusion
Carcinoma, Hepatocellular*
Carcinoma, Squamous Cell*
Chromosomal Instability
Cyclosporine
Cytogenetic Analysis
DNA Repair
Fanconi Anemia*
Head
Hematoma, Subdural
Humans
Hyperpigmentation
Leukemia
Liver
Maxilla
Mouth*
Neck
Pancytopenia
Pneumonia
Cyclosporine