Fanconi's anemia as a nature's evolutionary experiment on carcinogenesis

In order to obtain insights into the possible carcinogenetic mechanisms, the cancer incidence of Fanconi's anemia (FA) was surveyed in the literature, and the results were compared with those of other cancer-prone diseases of chromosomal breakage, and primary and secondary immunodeficiency syndromes along with an appropriate control population. With FA, there were 43% of leukemias but no lymphomatous diseases. Epithelial tumors consisting of hepatomas and squamous cell carcinomas were 17 and 38%, respectively. Their age distribution was 14.5 +/- 6.7 years of age for leukemias; 18.5 +/- 9.3 for hepatomas and 23.8 +/- 6.9 for squamous cell carcinomas. Putting aside the hepatomas which may be iatrogenic, the neoplastic trend here again is to show the non-epithelial--epithelial tumor shift (Okuyama and Mishina 1986). Fanconi's anemia can thus be one of the chromosomal breakage syndromes whose leukemogenic and carcinogenetic processes are amplified by an increased DNA damage, NK cell depletion, and IgA deficiency as the result of deficient Cu, Zn-SOD and increased suerpoxide toxicity.