Malignant intracerebral giant nerve sheath tumor in a 14-month-old girl with neurofibromatosis type 1: a case report.
BACKGROUND
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.


METHODS
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.


CONCLUSIONS
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.


CONCLUSIONS
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.
Oztanir
Namik
N
Department of Neurosurgery, Faculty of Medicine, Gazi University, Ankara, Turkey.
Emmez
Hakan
H
Aytar
M Hamit
MH
Dogan
Mehmet
M
Kaymaz
Memduh
M
Baykaner
M Kemali
MK
D001932:Brain Neoplasms / Q000175:diagnosis* / Q000601:surgery; D003937:Diagnosis, Differential; D017809:Fatal Outcome; D005260:Female; D005625:Frontal Lobe / Q000473:pathology / Q000601:surgery; D006801:Humans; D007223:Infant; D008279:Magnetic Resonance Imaging; D018317:Nerve Sheath Neoplasms / Q000175:diagnosis* / Q000601:surgery; D009456:Neurofibromatosis 1 / Q000175:diagnosis*; D010296:Parietal Lobe / Q000473:pathology / Q000601:surgery; D013702:Temporal Lobe / Q000473:pathology / Q000601:surgery