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

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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.

No MeSH data available.


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).
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Fig1: 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).

Mentions: There is a limited number of reports describing the epidemiology and natural history of FA-related SCC [2]. One of the most recent and largest collections of young adult and adult FA patients affected by such tumors was published by Grit Hohnbaum in 2008 [http://d-nb.info/997016965]. As summarized in Figure 1, Hohnbaum compiled data of 129 FA patients 20 years and older (comprising 42 case reports from the literature (1964 to 2008), 36 case histories from newsletters of the German and US FA family support groups (www.fanconi.de and www.fanconi.org), and 51 FA patients with cell cycle studies performed at the Institute of Human Genetics of the University of Wurzburg, Germany). Overall, 60 of these 129 patients (48 no HSCT, 12 after HSCT) developed SCC during their lifetime. The average age at SCC diagnosis for transplanted patients was 25.8 years (range 16 to 44 years) and for non-transplanted patients 30.0 years (range 20 to 52 years).Figure 1


Fanconi anemia: young patients at high risk for squamous cell carcinoma
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).
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC4530570&req=5

Fig1: 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).
Mentions: There is a limited number of reports describing the epidemiology and natural history of FA-related SCC [2]. One of the most recent and largest collections of young adult and adult FA patients affected by such tumors was published by Grit Hohnbaum in 2008 [http://d-nb.info/997016965]. As summarized in Figure 1, Hohnbaum compiled data of 129 FA patients 20 years and older (comprising 42 case reports from the literature (1964 to 2008), 36 case histories from newsletters of the German and US FA family support groups (www.fanconi.de and www.fanconi.org), and 51 FA patients with cell cycle studies performed at the Institute of Human Genetics of the University of Wurzburg, Germany). Overall, 60 of these 129 patients (48 no HSCT, 12 after HSCT) developed SCC during their lifetime. The average age at SCC diagnosis for transplanted patients was 25.8 years (range 16 to 44 years) and for non-transplanted patients 30.0 years (range 20 to 52 years).Figure 1

View Article: PubMed Central

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.

No MeSH data available.