Peripheral primitive neuroectodermal tumor of the cavernous sinus: case report.
OBJECTIVE
Ewing sarcoma/peripheral primitive neuroectodermal tumors (pPNET family) are small, round, blue cell tumors that have a decided predilection for young patients and commonly arise in bone and soft tissue. We are reporting a rare case of cavernous sinus pPNET in a 48-year-old woman.


METHODS
A 48-year-old woman presented with headache, ipsilateral maxillary, and ophthalmic and oculomotor nerve palsies. Neuroimaging revealed a cavernous sinus lesion.


METHODS
The patient underwent debulking of the tumor, and the diagnosis of a pPNET was made based on histological, immunohistochemical, and molecular genetics (EWS-FLI1 fusion gene) findings. Bone scans, bone marrow aspiration, and biopsy and chest computed tomographic scans showed no evidence of systemic involvement. The patient had adjuvant treatment with radiotherapy and chemotherapy. After 14 months, the patient had no neurological deficits, and neuroimaging showed stable disease, although some chemotherapy complications occurred.


CONCLUSIONS
This is a case of cavernous sinus pPNET in a 48-year-old woman, in whom the diagnosis is supported by the presence of EWS-FLI1 fusion gene. This seems to be the first reported case of a cavernous sinus pPNET confirmed by molecular genetic analysis.
Attabib
Najmedden A
NA
Department of Surgery, Section of Neurosurgery, University of Manitoba, Winnipeg, Manitoba, Canada.
West
Michael
M
Rhodes
Roy H
RH
D002426:Cavernous Sinus / Q000473:pathology*; D005260:Female; D006801:Humans; D018241:Neuroectodermal Tumors, Primitive, Peripheral / Q000175:diagnosis* / Q000235:genetics* / Q000628:therapy; D015514:Oncogene Proteins, Fusion / Q000235:genetics; D051789:Proto-Oncogene Protein c-fli-1 / Q000235:genetics; D034802:RNA-Binding Protein EWS
C106031:EWS-FLI fusion protein; D015514:Oncogene Proteins, Fusion; D051789:Proto-Oncogene Protein c-fli-1; D034802:RNA-Binding Protein EWS