An Overview of Autosomal Dominant Tumour Syndromes with Prominent Features in the Oral and Maxillofacial Region

An Overview of Autosomal Dominant Tumour Syndromes with Prominent Features in the Oral and... Several autosomal dominant inherited tumour syndromes demonstrate prominent features in the oral and maxillofacial region. Although multiple organ systems are frequently involved, the target organs more frequently affected are the skin (nevoid basal cell carcinoma syndrome, Brooke–Spiegler syndrome, Birt–Hogg–Dube syndrome and Muir–Torre syndrome), gastrointestinal tract (Peutz–Jegher syndrome and Gardner syndrome) or endocrine system (multiple endocrine neoplasia type 2b and hyperparathyroidism-jaw tumour syndrome). In some syndromes, the disease is multisystem with skin index lesions presenting in the head and neck (Cowden syndrome and tuberous sclerosis complex). The pertinent features of these syndromes are reviewed with a systems-based approach, emphasising their clinical impact and diagnosis. http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png Head and Neck Pathology Springer Journals

An Overview of Autosomal Dominant Tumour Syndromes with Prominent Features in the Oral and Maxillofacial Region

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Publisher
Springer US
Copyright
Copyright © 2017 by Springer Science+Business Media New York
Subject
Medicine & Public Health; Pathology; Otorhinolaryngology; Oral and Maxillofacial Surgery; Dentistry
eISSN
1936-0568
D.O.I.
10.1007/s12105-017-0778-1
Publisher site
See Article on Publisher Site

Abstract

Several autosomal dominant inherited tumour syndromes demonstrate prominent features in the oral and maxillofacial region. Although multiple organ systems are frequently involved, the target organs more frequently affected are the skin (nevoid basal cell carcinoma syndrome, Brooke–Spiegler syndrome, Birt–Hogg–Dube syndrome and Muir–Torre syndrome), gastrointestinal tract (Peutz–Jegher syndrome and Gardner syndrome) or endocrine system (multiple endocrine neoplasia type 2b and hyperparathyroidism-jaw tumour syndrome). In some syndromes, the disease is multisystem with skin index lesions presenting in the head and neck (Cowden syndrome and tuberous sclerosis complex). The pertinent features of these syndromes are reviewed with a systems-based approach, emphasising their clinical impact and diagnosis.

Journal

Head and Neck PathologySpringer Journals

Published: Jan 21, 2017

References

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