Familial Currarino syndrome presenting with peripheral primitive neuroectodermal tumour arising with a sacral teratoma.
Pediatr Blood Cancer
; 50(1): 172-5, 2008 Jan.
Article
em En
| MEDLINE
| ID: mdl-16685735
ABSTRACT
This report illustrates a rare genetic disorder, Currarino syndrome, in association with an unusual malignant transformation to a peripheral primitive neuroectodermal tumour within a sacral teratoma. The triad of features consists of a presacral mass, partial sacral agenesis and anorectal anomalies. The most common presentation is constipation. In this case there was a history of constipation, teratomas and spinal abnormalities in many of the family members over three generations. Detailed family history taken at time of initial presentation may have prevented delay in diagnosis and averted the need for intensive treatment, which may well cause late sequelae.
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Coleções:
01-internacional
Base de dados:
MEDLINE
Assunto principal:
Região Sacrococcígea
/
Teratoma
/
Tumores Neuroectodérmicos Primitivos Periféricos
/
Constipação Intestinal
/
Neoplasias Primárias Múltiplas
Limite:
Child, preschool
/
Female
/
Humans
Idioma:
En
Revista:
Pediatr Blood Cancer
Assunto da revista:
HEMATOLOGIA
/
NEOPLASIAS
/
PEDIATRIA
Ano de publicação:
2008
Tipo de documento:
Article
País de afiliação:
Reino Unido