Brain metastases as primary manifestation of a melanocytic malignant peripheral nerve sheath tumor in a 60-year-old man

Johannes Tilgner¹ , Klaus Müller² , Nadir Ghanem³ , Johannes Lutterbach⁴ and Jan Vesper¹

¹Dept. of Stereotactic Neurosurgery, Universitätsklinik Freiburg, Germany

²Dept. of Neuropathology, Universitätsklinik Freiburg, Germany

³Dept. of Diagnostic Radiology, Universitätsklinik Freiburg, Germany

⁴Dept. of Radiotherapy, Universitätsklinik Freiburg, Germany

author email corresponding author email

BMC Neurology 2007, 7:2doi:10.1186/1471-2377-7-2


Published:	16 January 2007

Abstract

Background

Malignant peripheral nerve sheath tumors are rare tumor entities that originate from peripheral nerve sheaths and have an unfavorable prognosis. Metastatic spread to the cerebral parenchyma is absolutely rare. This case report describes the clinical course in a 60-year-old man whose tumor came to medical attention because of a seizure.

Case presentation

Magnetic resonance imaging demonstrated two intracerebral lesions. The symptomatic lesion was removed microneurosurgically and histology demonstrated a metastasis from a malignant peripheral nerve sheath tumor. Postoperatively, whole-brain irradiation was performed. The primary tumor was identified in the area of the sciatic nerve on the right. Follow-up 14 months after resection showed that there was no progression of the intracerebral lesions but an increase in size and number of distant metastases.

Conclusion

There are no generally accepted guidelines for the treatment of malignant peripheral nerve sheath tumors with cerebral metastases. This case report presents and discusses one possible therapeutic approach. Due to the poor overall prognosis, the least invasive therapy should be chosen.