Malignant intracerebral giant nerve sheath tumor in a 14-month-old girl with neurofibromatosis type 1: a case report.
Childs Nerv Syst
; 25(2): 253-6, 2009 Feb.
Article
em En
| MEDLINE
| ID: mdl-18972118
ABSTRACT
INTRODUCTION:
Malignant intracerebral nerve sheath tumor (MINST) is extremely rare and the origin is still unclear. The authors present the clinical, radiological, and pathological features of a malignant intracerebral giant nerve sheath tumor. CASE REPORT A giant tumor in the right frontotemporoparietal lobes causing a midline shift was detected in a 14-month-old girl who presented with developmental delay, vomiting, and lethargy. The physical examination was consistent with neurofibromatosis type 1 (NF-1). Subtotal resection was performed and the histopathological examination revealed the diagnosis of MINST.DISCUSSION:
There are only six cases of malignant intracerebral nerve sheath tumor in the literature. The presented case is the youngest and the occurrence of MINST in a 14-month-old girl may support the hypothesis of multipotent mesenchymal stem cell origin; however, the tumors which arise from multipotent mesenchymal stem cells may be seen in later stages of life. Another important feature of the presented case is the occurrence of MINST in NF-1.CONCLUSION:
MINSTs are extremely rare tumors with unknown origin. The location, the degree, and the size of the tumor and the general condition of the patient are prognostic factors in MINSTs, like in other malignant tumors.
Texto completo:
1
Coleções:
01-internacional
Base de dados:
MEDLINE
Assunto principal:
Neoplasias Encefálicas
/
Neurofibromatose 1
/
Neoplasias de Bainha Neural
Tipo de estudo:
Diagnostic_studies
/
Prognostic_studies
Limite:
Female
/
Humans
/
Infant
Idioma:
En
Revista:
Childs Nerv Syst
Assunto da revista:
NEUROLOGIA
/
PEDIATRIA
Ano de publicação:
2009
Tipo de documento:
Article
País de afiliação:
Turquia