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. 2014 Dec;1(1):9.
doi: 10.1186/s40348-014-0009-8. Epub 2014 Nov 1.

Fanconi anemia: young patients at high risk for squamous cell carcinoma

Eunike Velleuer  1 , Ralf Dietrich  2
Affiliations

Fanconi anemia: young patients at high risk for squamous cell carcinoma

Eunike Velleuer et al. Mol Cell Pediatr. 2014 Dec.

Abstract

Background: Fanconi anemia is one of the best studied inherited cancer-prone diseases. Greatly improved protocols for hematopoietic stem cell transplantation increasingly save the lives of these young patients. However, in both transplanted and not transplanted patients, the emergence of aggressive squamous cell carcinoma represents a major medical challenge.

Conclusions: This mini review summarizes current knowledge about the pathogenesis of squamous cell carcinoma (SCC) in the special context of Fanconi anemia.

Keywords: Bone marrow transplant; Fanconi anemia; Squamous cell carcinoma; Young patients.

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Figures

Figure 1
Figure 1
Age of onset of SCC in FA patients with and without HSCT. Forty-three of 83 female FA patients (51.8%) and 17 of 46 male FA patients (37.0%) developed SCC (average age at SCC diagnosis for 48 FA patients without HSCT 30.0 years, for 12 FA patients after HSCT 25.8 years). Data summarized from Hohnbaum (see text).
Figure 2
Figure 2
Simplified model of the Fanconi anemia pathway. Activation of FANCD2 and FANCI by the FA core complex via monoubiquitination (orange circles) regulates downstream genes involved in recombination repair of DNA crosslinks.
See this image and copyright information in PMC

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