Head and Neck Pathology | 2021

Review of Pediatric Head and Neck Neoplasms that Raise the Possibility of a Cancer Predisposition Syndrome



Cancer predisposition syndromes (CPS) are generally heritable conditions that predispose individuals to develop cancer at a higher rate and younger age than their representative general population. They are a significant cause of cancer related morbidity and mortality in the pediatric population. Therefore, recognition of lesions that may be associated with a CPS and alerting the clinicians to its implications is a crucial task for a diagnostic pathologist. In this review we discuss benign pediatric head and neck lesions associated with CPS namely: odontogenic keratocyst, juvenile nasopharyngeal angiofibroma, ossifying fibroma of the jaw, paraganglioma, plexiform neurofibroma, plexiform schwannoma, mucosal neuroma, and nevus sebaceous syndrome; along with malignant tumors such as squamous cell carcinoma. Several head and neck melanocytic, endocrine, and central nervous system tumors can also be associated with CPS; they are beyond the scope of this article. Nasal chondromesenchymal hamartoma is discussed elsewhere in this issue.

Volume 15
Pages 16 - 24
DOI 10.1007/s12105-021-01292-x
Language English
Journal Head and Neck Pathology

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