Multiple endocrine neoplasia syndrome--type 2b. Case report and review.
Int J Oral Maxillofac Surg
; 21(2): 110-4, 1992 Apr.
Article
en En
| MEDLINE
| ID: mdl-1351093
The multiple endocrine neoplasia syndromes are an association of tumours of 2 or more endocrine glands. Multiple endocrine neoplasia type 2b (MEN 2b) patients develop medullary thyroid carcinoma and pheochromocytomas as well as unique physical characteristics. Most commonly, MEN2b is inherited with an autosomal dominant pattern although sporadic cases are not uncommon. If untreated the disease may be lethal. The facial, oral and ocular characteristics are reliable markers of the disease. These patients give a history most commonly of slipped capital femoral epiphysis, hypertension and life-long diarrhoea and/or constipation. MEN2b is most commonly characterised by nodules on the anterior aspect of the tongue, thickened lips with nodules, thickened upper eyelids, broadened nasal bridge, thickened corneal nerves and dilated, symmetrical, pedunculated nodules on the cheek mucosa. The patient described has most of these characteristics. Radiographic features of the jaws which have not been previously described are reported. These include a markedly enlarged and bifurcated inferior alveolar canal and shortened roots of the lower incisor teeth. Due to the lethality of the disease, patients who present with the above physical characteristics must be further investigated to exclude MEN2b.
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Colección:
01-internacional
Base de datos:
MEDLINE
Asunto principal:
Feocromocitoma
/
Neoplasia Endocrina Múltiple
/
Neoplasias de la Tiroides
/
Carcinoma
/
Neoplasias de las Glándulas Suprarrenales
Límite:
Adult
/
Humans
/
Male
Idioma:
En
Revista:
Int J Oral Maxillofac Surg
Asunto de la revista:
ODONTOLOGIA
Año:
1992
Tipo del documento:
Article
País de afiliación:
Australia
Pais de publicación:
Dinamarca