Multiple neoplasia in a 15-year-old girl with familial adenomatous polyposis.
J Pediatr Hematol Oncol
; 31(7): 530-2, 2009 Jul.
Article
em En
| MEDLINE
| ID: mdl-19564752
ABSTRACT
A 15-year-old girl with adenomatous polyposis coli gene (APC) mutation and brain tumor-polyposis syndrome developed an unusual succession of cervicocephalic tumors (medulloblastoma, meningeal low-grade myxoid tumor, and papillary thyroid carcinoma), at the age of 5, 9, and 15 years, respectively. We discuss the genetic profile of the thyroid tumor in which a large somatic deletion of APC gene was found and the physiopathology of thyroid carcinoma in patients with germline APC mutation. We also point out the uncommon phenotype in this young girl with early multiple neoplasias and the difficulties of management of such familial adenomatous polyposis patients with occurrence of extracolonic cancers that require the use of potential trigger agents as radiotherapy or chemotherapy.
Texto completo:
1
Coleções:
01-internacional
Base de dados:
MEDLINE
Assunto principal:
Genes APC
/
Polipose Adenomatosa do Colo
/
Neoplasias Primárias Múltiplas
Limite:
Adolescent
/
Child
/
Child, preschool
/
Female
/
Humans
Idioma:
En
Revista:
J Pediatr Hematol Oncol
Assunto da revista:
HEMATOLOGIA
/
NEOPLASIAS
/
PEDIATRIA
Ano de publicação:
2009
Tipo de documento:
Article
País de afiliação:
Bélgica