Head and neck cancer associated with Bloom's syndrome.
Bloom's syndrome (BS) is an autosomal recessive growth disorder associated with chromosomal breaks and rearrangements. Affected individuals exhibit a significantly elevated rate of cancer at an early age. Twenty-seven percent of patients with BS have malignant neoplasms at a mean age of 20.7 years. Since the first description of BS in 1954, five cases of primary head and neck cancer have been identified in the first 103 patients, including two tongue carcinomas and three laryngeal carcinomas. The patients ranged in age from 26 to 34 and included smokers and nonsmokers. The presenting symptoms and histology were identical with those of non-Bloom's syndrome patients. Head and neck cancer represents approximately 6% of all human tumors. This is in contrast with an 18% incidence rate of head and neck cancer among all cancers observed in BS patients. The head and neck surgeon should consider BS in the differential diagnosis of young cancer patients.
Berkower
A S
AS
Department of Otolaryngology, Mount Sinai Medical Center, New York, NY 10029-6574.
Biller
H F
HF
D000328:Adult; D001816:Bloom Syndrome / Q000150:complications* / Q000235:genetics; D002294:Carcinoma, Squamous Cell / Q000209:etiology*; D006258:Head and Neck Neoplasms / Q000209:etiology; D006801:Humans; D007822:Laryngeal Neoplasms / Q000209:etiology*; D008297:Male; D010375:Pedigree