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Adult Head and Neck Cancer and Hematopoietic Mosaicism

Blanche P. Alter, MD, MPH; Hans Joenje, PhD; Anneke B. Oostra; Gerard Pals, PhD

Arch Otolaryngol Head Neck Surg. 2005;131:635-639.

	Since this article does not have an abstract, we have provided the first 150 words of the full text and any section headings.

INTRODUCTION

Fanconi anemia (FA) is an autosomal recessive DNA repair disorder with a very high risk of cancer.^1-2 While most of the homozygotes are identified clinically because of characteristic birth defects and early-onset aplastic anemia,³ a subset of patients, often with milder physical and hematologic phenotypes, remain undiagnosed. They are at very high risk of neoplasms, including acute myeloid leukemias and solid tumors.⁴ All types of solid tumors (combined) develop at a rate that is 48 times greater than that experienced by the general population, and the cancer hazard rate is 2% per year by the age of 24 years, with a cumulative incidence in a competing risk model of 29% by the age of 45 years.⁵

The majority of the solid tumors in patients with FA arise in the aerodigestive or gynecologic areas.^{3, 6} The relative risk . . . [Full Text of this Article]

REPORT OF A CASE

METHODS

RESULTS

COMMENT

AUTHOR INFORMATION

Author Affiliations: Clinical Genetics Branch, Division of Cancer Epidemiology and Genetics, National Cancer Institute, Department of Health and Human Services, Bethesda, Md (Dr Alter); and Department of Clinical Genetics and Human Genetics, VU University Medical Center, Amsterdam, the Netherlands (Drs Joenje and Pals and Ms Oostra).

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