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The authors report a case of giant cell tumor originating in the sphenoid bone. A 29-year-old woman presented with headache and diplopia. Bilateral ophthalmoplegia developed and MRI showed rapid growth of the tumor in spite of transnasal removal and conventional radiation therapy. The second transnasal surgery was performed 3 weeks after completion of radiotherapy. The symptoms were relieved except for right abducens palsy. Chemotherapy with cisplatin and etoposide was started after the second operation. The patient regained full ocular movement several months after the operation. Clinical remission has continued for four years. We conclude that the combination of repeated operations in the initial phase of rapid tumor growth, irradiation and chemotherapy is important to achieve tumor control.

The prognosis of giant cell tumors arising in the sacrum is difficult to ascertain. Because of the low incidence in Japan there are scanty statistics. We selected 14 cases from the Bone Tumor Registry in Japan. Questionnaires were sent to hospitals which had submitted cases, regarding Enneking's staging, surgical approach, surgical procedure, radiotherapy, chemotherapy, complications and prognosis of this tumor. The results of the questionnaire show that surgery alone gives better results than combined surgery and radiotherapy. Good prognosis called for surgery alone. Curettage resulted in no evidence of disease (NED) in four cases. Bad prognosis called for combined surgery, radiotherapy and chemotherapy. If we surmise from the biological behavior of the giant cell tumor, that the tumor mass extends out extraosseously en-bloc excision with nerve roots is indicated. Extensive curettage will be adequate if the tumor is confined intraosseously. Radiotherapy should not be selected except for inoperative cases.

A 19-year-old man was admitted to the hospital because of blurred vision, visual field defect, diplopia and hypesthesia of the left face. Neurological examination on admission revealed impairments of the II, III, IV, VI cranial nerves bilaterally and the first branch of the V nerve on the left. X-ray films of the skull showed a marked decalcification of the sella and upper portion of the clivus. Cerebral angiography demonstrated a moderately vascularized, large tumor in the sella-clival region. The tumor was supplied mainly by the branches of the right internal carotid artery, which was occluded at the cavernous portion. CT scans showed a large, oval mass located at the mid-portion of the anterior and middle fossa. Hounsfield number of the tumor was approximately 64.0, but several high density spots, probably due to destroyed bone fragments, were seen inside. The tumor was markedly enhanced with contrast medium. Three successive craniotomies were carried out through right fronto-temporal approaches, but total removal of the tumor was not achieved. Histological examination of surgical specimens disclosed that the tumor was consisted of abundant multinucleated giant cells and fewer spindle shaped stromal cells. Postoperative radiotherapy by telecobalt was tried and a total dose of 70Gy was delivered to the residual tumor. Effect of radiotherapy was remarkable and the size of the tumor on CT was markedly reduced to the extent of 10% of the pre-radiation tumor size. The patient was discharged in a good condition and there have been no signs of recurrence for 10 months so far. On the basis of our case and cases reported in the literature so far, the authors discussed clinical and radiological features, difficulty in surgical treatment and radiosensitivity of giant cell tumor of the sphenoid bone.