Cowden syndrome: mucocutaneous lesions as precursors of internal malignancy

2.50
Hdl Handle:
http://hdl.handle.net/11287/593866
Title:
Cowden syndrome: mucocutaneous lesions as precursors of internal malignancy
Authors:
Stathopoulos, Panagiotis; Raymond, Anna; Esson, Michael
Abstract:
BACKGROUND: Cowden syndrome is an autosomal-dominant hereditary cancer syndrome with high variability and susceptibility. It is characterized by multiple hamartomas and neoplasms of ectodermal, endodermal and mesodermal origin affecting many organs and also by the increased risk of development of internal malignancies. CASE REPORT: A 62-year-old woman was referred to our Maxillofacial Unit with a hamartomatous mass of the left lateral tongue which had slowly grown and was obstructing normal speech and restricting oral intake. The patient had a known history of Cowden syndrome and underwent excision of the lesion under general anaesthetic. DISCUSSION: Orofacial mucocutaneous features are very common in multiple hamartoma and neoplasia syndrome with almost up to 90% of the patients being affected. These cutaneous and mucosal lesions, which are predominantly benign, often manifest prior to the development of the internal malignant tumours associated with the syndrome. CONCLUSIONS: The prompt identification of Cowden syndrome's plethoric signs and symptoms can lead to appropriate surveillance and multidisciplinary management. Oral manifestations are frequent, prominent and usually precede the establishment of malignant tumours of visceral organs; hence, the maxillofacial surgeon or general dentist may have a significant role in the recognition of the disease. Overall prognosis is dependent on prevention or early treatment of internal malignancies; consequently, early diagnosis together with frequent follow-up forms the cornerstone of management.
Citation:
Oral Maxillofac Surg. 2014 Jun;18(2):229-35.
Publisher:
Springer
Journal:
Oral and maxillofacial surgery
Issue Date:
1-Jun-2014
URI:
http://hdl.handle.net/11287/593866
DOI:
10.1007/s10006-014-0445-6
PubMed ID:
24687348
Additional Links:
http://dx.doi.org/10.1007/s10006-014-0445-6
Type:
Case Report
Language:
eng
ISSN:
1865-1569
Appears in Collections:
Oral and Maxillofacial Surgery/Orthodontics; 2014 RD&E publications

Full metadata record

DC FieldValue Language
dc.contributor.authorStathopoulos, Panagiotisen
dc.contributor.authorRaymond, Annaen
dc.contributor.authorEsson, Michaelen
dc.date.accessioned2016-01-19T12:36:01Zen
dc.date.available2016-01-19T12:36:01Zen
dc.date.issued2014-06-01en
dc.identifier.citationOral Maxillofac Surg. 2014 Jun;18(2):229-35.en
dc.identifier.issn1865-1569en
dc.identifier.pmid24687348en
dc.identifier.doi10.1007/s10006-014-0445-6en
dc.identifier.urihttp://hdl.handle.net/11287/593866en
dc.description.abstractBACKGROUND: Cowden syndrome is an autosomal-dominant hereditary cancer syndrome with high variability and susceptibility. It is characterized by multiple hamartomas and neoplasms of ectodermal, endodermal and mesodermal origin affecting many organs and also by the increased risk of development of internal malignancies. CASE REPORT: A 62-year-old woman was referred to our Maxillofacial Unit with a hamartomatous mass of the left lateral tongue which had slowly grown and was obstructing normal speech and restricting oral intake. The patient had a known history of Cowden syndrome and underwent excision of the lesion under general anaesthetic. DISCUSSION: Orofacial mucocutaneous features are very common in multiple hamartoma and neoplasia syndrome with almost up to 90% of the patients being affected. These cutaneous and mucosal lesions, which are predominantly benign, often manifest prior to the development of the internal malignant tumours associated with the syndrome. CONCLUSIONS: The prompt identification of Cowden syndrome's plethoric signs and symptoms can lead to appropriate surveillance and multidisciplinary management. Oral manifestations are frequent, prominent and usually precede the establishment of malignant tumours of visceral organs; hence, the maxillofacial surgeon or general dentist may have a significant role in the recognition of the disease. Overall prognosis is dependent on prevention or early treatment of internal malignancies; consequently, early diagnosis together with frequent follow-up forms the cornerstone of management.en
dc.language.isoengen
dc.publisherSpringeren
dc.relation.urlhttp://dx.doi.org/10.1007/s10006-014-0445-6en
dc.titleCowden syndrome: mucocutaneous lesions as precursors of internal malignancyen
dc.typeCase Reporten
dc.identifier.journalOral and maxillofacial surgeryen

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