guidebook. - Fanconi Anemia Research Fund

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Fanconi Anemia: Guidelines for Diagnosis and Management
surgical treatment of lesions should be considered, and
consultation with a hematologist experienced with FA
should be obtained prior to instituting radiation or chemotherapy.
Human papillomavirus immunization
In two recent studies on a small number of vulvar SCC
tumors in FA patients, the tumors tested positive for
HPV. 16,17 Because of the increased risk of genital tract
neoplasia in women with FA and head and neck cancer
in both men and women with FA, it may be reasonable
to consider HPV vaccination for both male and
female FA patients at age nine. It is unknown whether
FA patients mount the usual immune response to the
vaccine. While the vaccine will not treat existing HPV
disease, it may prevent the acquisition of some other
subtypes. As the HPV vaccine does not prevent all genital
tract cancers and the efficacy of this vaccine in FA
is not known, vaccinated FA patients should continue
regular screening.
Breast cancer surveillance
Breast cancer surveillance should begin by the early 20s
and include annual breast exams. Screening mammograms
should be initiated if a mass is detected or by age
25. The risk of breast cancer in FA does not appear to
be excessive, although a few cases have been reported
to date. 18 A few FA patients have acquired breast cancer
at a median age of 37 years, compared to a median
age of 61 in the non-FA population (see Chapter 2). To
avoid radiation, magnetic resonance imaging may be
considered. However, breast magnetic resonance imaging,
while very sensitive, is non-specific and has a high
false positive rate. Thus, it is usually considered to be
an adjunct to mammography. 19