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Astroblastomas are rare glial tumors. We report a case of 33-year-old woman with high-grade astroblastoma with hypervascularity. She had a one-month history of right visual disturbance and papillar edema. MRI revealed a lobulated mass with cysts and flow voids in the right superficial frontal lobe, a phenomenon described as "bubbly appearance". Right carotid angiography demonstrated marked tumor stain and early venous filling. MR spectroscopy showed an increase in myoinositol and the choline/creatine ratio, and decreased N-acetyl aspartate. The lipid and lactate level was not increased. The well-circumscribed tumor was totally resected. Histological examination showed perivascular pseudorosettes and hyalinization of blood vessels with high cellularity, anaplastic nuclear features, focal necrosis, mitosis, and endothelial proliferation. Immunohistochemically, glial fibrillary acidic protein and S-100 protein were intensely positive and the MIB-1 labeling index was high(20%)in the tumor cells. Based on these findings, a diagnosis of high-grade astroblastoma was made. The patient received postoperative radiotherapy and chemotherapy with temozolomide and suffered no relapse in the course of 3 years after surgery. Characteristically, astroblastomas manifest a "bubbly appearance" and a lobulated mass on MRI scans. As these tumors tend to be hypervascular, angiograms are useful for designing the operative strategy. However, their low-or high grade is difficult to ascertain preoperatively based on MRI-, MRS-, and DSA findings. The standard therapy for high-grade astroblastoma is total resection and postoperative radiation therapy. As the incidence of tumor recurrence is high, we recommend additional chemotherapy with TMZ.

The alveolar soft part sarcoma (ASPS) is a rare soft tissue tumor which usually occurs in the lower extremity of young girls. The incidence of metastasis is said to be highest in the lung (38%), and second highest in the bone and brain (33%). This report describes two cases of metastatic intracerebral alveolar soft part sarcoma, originating in the lower extremity. A female patient noticed a painless swelling in her right leg at the age of 11, and 10 years later she underwent total removal of the tumor. The diagnosis was alveolar soft part sarcoma. At 37 she was admitted to our service with a diagnosis of cerebral metastasis in the left frontal lobe. Since then she has undergone surgical removal 4 times for recurrent cerebral metastasis and twice for lung metastasis. Now she is 55 years old and doing well except for mild left hemiparesis. She survives without cerebral or general metastasis 44 years following the onset of the sarcoma in her right leg and 18 years following the onset of the metastatic brain tumor. A 30-year-old man, who noticed a painless swelling in his left thigh in January 1991, underwent total removal of the tumor and the diagnosis was alveolar soft part sarcoma. He was admitted to our service with no neurological deficits in October 1992, but a CT scan showed a metastatic brain tumor in the left parieto-occipital region. Total removal of this metastatic brain tumor was successfully performed in November 1992. However, he died because of multiple brain and lung metastasis in February, 1994.(ABSTRACT TRUNCATED AT 250 WORDS)

A case of spontaneous middle cerebral artery occlusion with moyamoya-like vessels associated with contralateral middle cerebral artery aneurysm is reported. A 23-year-old male was admitted to our hospital with complaints of severe headache and vomiting. On admission CT scan demonstrated subarachnoid hemorrhage with high density in the left Sylvian fissure and suggested a ruptured left middle cerebral artery aneurysm. Carotid angiograms demonstrated a left middle cerebral artery aneurysm and an occlusion of the right middle cerebral artery at its origin with moyamoya-like vessels. There was no occlusion or stenosis in the bilateral intracranial internal carotid arteries. Furthermore, bilateral vertebral angiograms were also normal. The aneurysm was successfully clipped. The postoperative course was uneventful and the patient was discharged with no neurological deficit. So far as we could ascertain, there have been only 21 cases reported previously of spontaneous middle cerebral artery stenosis or occlusion with moyamoya-like vessels. According to angiographic studies and transcranial Doppler ultrasound findings, we are more inclined to believe that hemodynamic changes secondary to arterial occlusion lead to the formation and growth of aneurysms of the contralateral middle cerebral artery.

A 16-year-old girl had an episode of intermittent involuntary spasm of the right inferior orbicularis oculi muscle at the age of 12. In the following years the right orbicularis oris muscle and platysma were unilaterally and progressively involved in the spasms and with increasing frequency. Medical treatment with minor transquilizer was not effective. She was referred to our department at the age of 16. CT and MRI revealed no abnormality. Angiographical study revealed that the right PICA (posterior inferior cerebellar artery) was tourtously coming off from a relatively high portion of the right vertebral artery. The trunk of the right AICA (anterior inferior cerebellar artery) was not able to be identified. Although the onset of this condition was unusually early, the clinical course and symptoms of hemifacial spasm were so clearly typical that she underwent microvascular decompression surgery on July 26, 1988, at the age of 16. During the operation an upward looping PICA was found crossing and tightly compressing the exit zone of the right facial nerve. The offending artery seemed to be elastic without significant atherosclerotic change. The arterial loop was carefully dissected and replaced with two pieces of sponge prosthesis between the artery and the surface of brain stem. Her hemifacial spasm completely disappeared post-operatively. No recurrence has been noted for over one year in the follow-up observation. Hemifacial spasm is subacutely or chronically a progressive disorder of facial involuntary movement. The arterial compression in the exit zone of the facial nerve is said to be the cause of the disorder and microvascular decompression has been indicated for it since a little before the beginning of this decade.(ABSTRACT TRUNCATED AT 250 WORDS)