Lhermitte-Duclos Disease Associated with Cowden Disease
Journal of Korean Neurosurgical Society
;
: 375-378, 2003.
Artículo
en Inglés
| 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.
Texto completo:
Disponible
Índice:
WPRIM (Pacífico Occidental)
Asunto principal:
Papiloma
/
Glándula Tiroides
/
Síndrome de Hamartoma Múltiple
/
Mama
/
Corteza Cerebelosa
/
Incidencia
/
Estudios de Seguimiento
/
Síndromes Neurocutáneos
/
Hamartoma
/
Queratosis
Tipo de estudio:
Estudio de incidencia
/
Estudio observacional
/
Estudio pronóstico
/
Factores de riesgo
/
Estudio de tamizaje
Idioma:
Inglés
Revista:
Journal of Korean Neurosurgical Society
Año:
2003
Tipo del documento:
Artículo
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