Lhermitte-Duclos Disease Associated with Cowden Disease
Journal of Korean Neurosurgical Society
;
: 375-378, 2003.
Article
Dans Anglais
| WPRIM
| ID: wpr-227607
ABSTRACT
Lhermitte-Duclos disease, or dysplastic gangliocytoma of the cerebellum, is a peculiar hamartoma arising from the cerebellar cortex. Cowden disease, which is also termed as a multiple hamartoma-neoplasia syndrome, is an unusual autosomal dominant disorder characterized by mucocutaneous lesions, including facial papules, gingival papillomas, and acral keratoses. Other systemic hamartomas are frequently present, and there are high incidence of breast, thyroid, and genitourinary malignancies. Recently, it is accepted that Lhermitte-Duclos disease is a part of Cowden disease, a new phakomatosis. Recognition of this association has clinical significance, because diligent long-term follow-up monitoring of individuals with Lhermitte-Duclos disease and Cowden disease may lead to the early detection of malignancy.
Texte intégral:
Disponible
Indice:
WPRIM (Pacifique occidental)
Sujet Principal:
Papillome
/
Glande thyroide
/
Syndrome des hamartomes multiples
/
Région mammaire
/
Cortex cérébelleux
/
Incidence
/
Études de suivi
/
Syndromes neurocutanés
/
Hamartomes
/
Kératose
Type d'étude:
Etude d'incidence
/
Étude observationnelle
/
Étude pronostique
/
Facteurs de risque
/
Étude de dépistage
langue:
Anglais
Texte intégral:
Journal of Korean Neurosurgical Society
Année:
2003
Type:
Article
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