Head and neck pleomorphic myxoid liposarcoma in a child with Li-Fraumeni syndrome.
Int J Pediatr Otorhinolaryngol
; 123: 191-194, 2019 Aug.
Article
em En
| MEDLINE
| ID: mdl-31129458
INTRODUCTION: Pleomorphic myxoid liposarcoma is a rare and aggressive cancer seen in the pediatric population that has been previously associated with hereditable cancer disorders like Li Fraumeni syndrome. We present a case report and review of the relevant literature. CASE PRESENTATION: Pleomorphic myxoid liposarcoma presenting as a second primary tumor in a child with a strong family history for cancer led to diagnosis of Li-Fraumeni syndrome, which is associated with TP53 tumor suppressor gene inactivation. MANAGEMENT AND OUTCOME: The tumor was fully excised, but postoperative radiation was deferred to limit future radiation-induced tumorgenesis. DISCUSSION: Pleomorphic myxoid liposarcoma is rare but aggressive, and should prompt caregivers to test for potential hereditable cancer disorders. Li-Fraumeni syndrome is associated with early onset neoplasia and development of recurrent primary tumors. Its presence affects treatment decisions and methods of surveillance. Chemoradiation should be used judiciously in this population.
Palavras-chave
Texto completo:
1
Coleções:
01-internacional
Temas:
Geral
/
Tipos_de_cancer
/
Outros_tipos
Base de dados:
MEDLINE
Assunto principal:
Neoplasias Faciais
/
Síndrome de Li-Fraumeni
/
Lipossarcoma Mixoide
Tipo de estudo:
Etiology_studies
/
Prognostic_studies
Limite:
Child
/
Humans
/
Male
Idioma:
En
Revista:
Int J Pediatr Otorhinolaryngol
Ano de publicação:
2019
Tipo de documento:
Article
País de afiliação:
Estados Unidos