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Chapter 13: Head and Neck SCC in FA patients 255 promote head and neck cancer development. Accordingly, maintenance of proper oral hygiene and routine dental evaluations are recommended. 3. The use of oral appliances: Given the lack of evidence to suggest a causal association with head and neck cancer, the use of oral appliances, including braces, need not be restricted in FA patients. Surveillance for Head and Neck Cancer The high incidence of head and neck cancer in patients with FA, combined with the ineffectiveness of available treatment approaches, underscores the need for aggressive surveillance. Surveillance should begin by the age of 10-12 years (based on literature reports of the earliest age at presentation with head and neck cancer) on a semiannual basis by an experienced professional; i.e., an ear, nose and throat specialist, an oral surgeon or other doctor experienced in head and neck cancer detection and treatment (Figure 1). Since head and neck cancer in patients with FA commonly occurs in the oral cavity, the surveillance approach should focus on this region. However, as part of the routine screening, a flexible fiberoptic examination should be performed which includes evaluation of the nasopharynx, oropharynx, hypopharynx, and larynx. The use of routine esophagoscopy for screening is not mandated, but should be considered in any patient with odynophagia, dysphasia or other localizing symptoms. In these circumstances, evaluation could be performed either with endoscopy or barium swallow, with the specific findings guiding further evaluation and therapy. Focus should be on the cervical esophagus, which represents the region at highest risk for Fanconi-associated squamous cell carcinomas.
256 Fanconi Anemia: Guidelines for Diagnosis and Management Figure 1: This flow chart describes the appropriate evaluation, surveillance, and diagnosis of head and neck lesions identified in the FA patient. The degree of dysplasia within the lesion should not influence decision-making regarding treatment. For example, even mild dysplastic lesions should be excised when feasible. Start evaluation of head and neck at age 10 years. 1. Head and neck exam by ENT with FA experience. 2. Flexible fiberoptic exam Normal exam Lichen planus Leukoplakia Erythroplakia Lesion suspicious for SCC Biopsy of lesion Evaluate patient semi-annually Excisional biopsy Appropriate treatment, if positive for SCC Increase surveillance timing to every 2-3 months Close follow-up every 2-3 months, including yearly chest x-rays
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Chapter 4 Gastrointestinal, Hepatic
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Chapter 7 Endocrine Disorders in Fa
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Chapter 9 Matched Sibling Donor Hem
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