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.
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References
Beauchesne P, Mosnier JF, Schmitt T et al (2004) Malignant nerve sheath tumor of the right cerebral peduncle. Neurosurgery 54:500–504
Bruner JM, Humphreys JH, Armstrong DL (1984) Immunocytochemistry of recurring intracerebral nerve sheath tumor. J Neuropathol Exp Neurol 43:296
Casadei GP, Komori T, Scheithauer BW et al (1993) Intracranial parenchymal schwannoma. A clinicopathological and neuroimaging study of nine cases. J Neurosurg 79:217–222
Ducatman BS, Scheithauer BW, Piepgras DG (1986) Malignant peripheral nerve sheath tumor. A clinicopathologic study of 120 cases. Cancer 57:2006–2021
Hirose T, Sumitomo M, Kudo E (1989) Malignant peripheral nerve sheath tumour (MPNST) showing perineural cell differentiation. Am J Surg Pathol 13:613–620
Matsubara S, Ebisudani D, Ohshima T et al (1992) A case of metastatic intracerebral malignant schwannoma. No Shinkei Geka 20:187–190
Sharma S, Abbott RI, Zagzag D (1998) Malignant intracerebral nerve sheath tumour: A case report and review of the literature. Cancer 82:545–552
Singh RVP, Suys S, Campbell DA et al (1993) Malignant schwannoma of the cerebellum: case report. Surg Neurol 39:128–132
Stark AM, Mehdorn HM (2006) Multiple intracranial metastases from a malignant peripheral nerve sheath tumor of the extremities. J Neurooncol 78:209–210
Stefanko SZ, Vuzevski VD, Maas AIR (1986) Intracerebral malignant schwannoma. Acta Neuropathol (Berl) 71:321–325
Tanaka M, Shibui S, Nomura K et al (2000) Malignant intracerebral nerve sheath tumor with intratumoral calcification: Case report. J Neurosurg 92:338–341
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Oztanir, N., Emmez, H., Aytar, M.H. et al. Malignant intracerebral giant nerve sheath tumor in a 14-month-old girl with neurofibromatosis type 1: a case report. Childs Nerv Syst 25, 253–256 (2009). https://doi.org/10.1007/s00381-008-0727-0
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DOI: https://doi.org/10.1007/s00381-008-0727-0