guidebook. - Fanconi Anemia Research Fund

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Fanconi Anemia: Guidelines for Diagnosis and Management
sequelae can result from the use of radiation therapy
and/or chemotherapy. Therefore, the use of these
modalities must be individualized and only applied
when absolutely required. Conversely, surgical therapy
of head and neck cancer in patients with FA is reasonably
well-tolerated. There does not seem to be an
increased incidence of complications, including wound
infections or long term sequela associated with surgical
scarring. Accordingly, the consensus opinion is that
surgical therapy needs to be entertained as the primary
curative modality in all FA patients with head and neck
cancer.
Surgery
Surgery in this patient population should follow dicta
established for the general population with head and
neck cancer, with a few modifications. In general, a
wide complete excision of the primary tumor should
be performed with adequate margins. The exact type of
surgical resection required is dictated by the primary
site, size, and the extent of the tumor. In general, oral
cavity and pharyngeal tumors should be excised with at
least one centimeter margins. The margins for laryngeal
tumors need not be as comprehensive, due to the unique
anatomy of the larynx. 13 Reconstruction of the primary
site defect should follow dicta established for reconstruction
in the general population with head and neck
cancer, and should not be limited based on the presence
of FA. Therefore, the standard application of free flaps
for reconstruction should be considered as indicated,
without restriction.
The management of clinically detectable cervical
lymphadenopathy should follow dicta established for
the general population. For lymph nodes greater than
3 cm, multiple lymph nodes on the same side of the
neck or contralateral cervical adenopathy, a modified