Primary intracranial peripheral primitive neuroectodermal tumor/Ewing's sarcoma presenting with acute intracerebral hemorrhage.

OBJECTIVE: To report two cases of intracerebral hemorrhage due to primary intracranial peripheral primitive neuroectodermal tumor (pPNET)/Ewing's sarcoma (ES) and review of related literatures. MATERIAL: Two cases of 17-year-old patients presented with acute increased intracranial pressure one of which also had left hemiparesis. METHOD: On neuroimaging studies, the first patient had an intraparenchymal hematoma with a size of 4 cm at the right fronto-parietal junction adjacent to tumor infiltrating the superior sagittal sinus. The second patient had a large left temporal tumor with intraventricular hemorrhage. Both patients underwent craniotomy with complete removal of tumor and hematoma. RESULTS: Pathological examination in both cases revealed numerous small round tumor cells with stippled chromatin pattern and scanty cytoplasm. Tumor cells strongly expressed CD99. Vimentin immunoreactivity was observed. The final diagnosis of pPNET/ES was rendered. There was no evidence of extracranial disease in both cases. Both patients were doing well without evidence of recurrent disease at 12 and 24-month follow-up respectively. CONCLUSIONS: Peripheral primitive neuroectodermal tumor (pPNET)/Ewing's sarcoma (ES) is a malignant small round cell tumor, commonly arising in soft tissue of the trunk and lower extremity. Those occurring in the intracranium are rare, and most patients present with progressively increased intracranial pressure and/or cranial nerve deficit. The occurrence of intracerebral hemorrhage due to primary intracranial pPNET/ES is exceedingly rare. The role of adjuvant therapy in this condition is yet to be investigated.

Intracranial primitive neuroectodermal tumors: experience of 7 cases with particular reference to survival.

A group of neoplasms consisting of undifferentiated neuroepithelial cells being capable of differentiating into either neuronal or glial line or both directions has been termed "Primitive Neuroectodermal Tumors (PNETs)". They have been shown to possess several similarities in clinical, radiological, and pathological features to the cerebellar medulloblastomas. The latter are well treated by surgical removal followed by radiotherapy. Therefore, we used the same regimen in treating 7 patients with intracranial PNETs and obtained an average survival of 28.7 months which is longer than those of previous reports. The analysis of our patients disclosed an optimistic view that the ideal prognosis of PNETs should be better than this.

Extradural hematoma: analysis of 138 cases.

138 patients with extradural hematoma have been analyzed in order to assess the factors influencing their mortality. Early diagnosis, based on the development of minimal clinical signs, and prompt treatment will lower the mortality rate. The 16% mortality rate in this series might be reduced to 11%, had there been no errors in management.

Management of chronic atlantoaxial dislocation.

Twenty-two patients with chronic atlantoaxial dislocation have been treated. Of 13 patients with a reducible dislocation by skeletal traction, 12 underwent successful posterior infusion of the first, second and third cervical vertebrae. Of nine patients with irreducible dislocation, eight underwent surgical reduction and fusion through the transoral approach. The reduction was successful in all eight, but fusion was accomplished in four.

Primary intracranial rhabdomyosarcoma producing proptosis.

A case is reported of a primary rhabdomyosarcoma occurring in the right subfrontal region of a 16 year old girl. The patient suffered from dull frontal headache and proptosis for three months before hospitalization. The circumscribed and demarcated neoplasm involved the dura mater, and invaded the frontal sinus and roof of the orbit on the same side. The pathogenesis of the tumour is thought to be related to aberrant differentiation of unstable mesenchyme. A suggestion is made that the "medullomyoblastoma" should be classified as a type of neoplasm of mixed mesenchymal and neuroepithelial origin.

Combined neuroepithelial (colloid) cyst and xanthogranuloma (xanthoma) in the third ventricle.

A case is reported of a combined neuroepithelial cyst and xanthogranuloma of the choroid plexus in the third ventricle of a 22-year-old woman. It is suggested that proliferated neuroepithelial cells lining the cyst enter the fibrous wall through the disrupted basal lamina, and then become xanthomatous cells. Disintegration of these foamy epithelial cells releases lipids and other materials into the cyst wall, provoking a response of macrophages and multinucleated giant cells of foreign-body type. A xanthogranuloma is then formed. The origin of "colloid" cysts is from neuroepithelium; these cysts arise from both ependyma and choroid plexus. Those cysts arising in or near the floor of the third ventricle may originate in stomodeal epithelium, but a distinction cannot be made from neuroepithelial cysts by presently available methods.