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In the general population, these sites (particularly oropharyngeal SCC) may be associated with infection with human papillomavirus (HPV). In FA and DC, however, the majority of HNSCC occur in the oral cavity. We investigated the HPV status of HNSCC and vulvar SCC from nine patients with FA and four with DC using a very sensitive PCR assay, and found HPV16 DNA in only a single vulvar tumor from one patient with FA, and in none of the HNSCC. These results suggest that HPV may not be the cause of SCC in patients with FA or DC, and that vaccination may not reduce the incidence of HNSCC in these patients. Fanconi anemia (FA) is primarily an autosomal recessive DNA repair disorder characterized in many patients by congenital abnormalities, bone marrow failure, acute myeloid leukemia and solid tumors. The relative http://www.selleck.cn/products/U0126.html risk of tumors is ?50-fold higher than in the general population, ?800-fold for head and neck squamous cell carcinomas (HNSCC) and ?3,000-fold for vulvar cancer. Dyskeratosis congenita (DC) is a clinically similar inherited bone marrow failure syndrome, due to abnormalities in telomere biology, with a relative risk of ?8-fold for solid tumors, and ?1,100-fold for HNSCC.[1] In the general population, almost all cervical, and up to half of vulvar, cancers are associated with human papillomavirus (HPV) infection, particularly the high-risk types HPV16 and HPV18.[2] In the USA, HPV16 or 18 is also associated with ?25% of HNSCC, particularly http://www.selleckchem.com/products/INCB18424.html >50% of oropharynx rather than oral cavity ( http://www.selleckchem.com/products/PLX-4032.html with FA, including 6 vulvar and 15 oral cavity.[8] However, positive tumors had as little as one copy of HPV DNA per ten cells; this measure may have been too sensitive, leading to an excess of clinical false positive results. Van Zeeburg et al. found HPV16 in only two of three anogenital SCCs and none of 16 HNSCC or two esophageal SCCs; positives were identified by the broad spectrum HPV GP5+/6+ PCR enzyme immune assay and quantitative PCR.[9] These discrepant studies differ in geography (USA vs. Europe), methods, and assay sensitivities, and did not study additional markers to distinguish transient vs. transforming HPV infections. The association of HPV with cancer in FA is plausible, given the locations of the cancers.