Primary craniospinal giant cell tumors are rare (5-15% of all giant cell tumors), locally destructive bone lesions which are generally not associated with Paget's disease. Clinical behavior of the craniospinal giant cell tumor (GCT) is unpredictable but often very aggressive and therefore optimal management of the lesions remains controversial. Wide resection of the involved bone is required, preferably with a wide margin of normal tissue, which may be difficult to achieve in the craniospinal region. The authors analysed a series of 11 patients, which included five cases of cranial and six cases of spinal GCTs. Their ages ranged from 12 to 46 years with a male preponderance. In the five cranial cases, all the tumors involved the skull base, three involved the spheno - ethmoidal region and in two cases the petroclival region was involved. Common presenting symptoms were frontotemporal swelling, epistaxis, local pain and neurological deficits attributable to the neurovascular compression. Proptosis, visual loss, nasal mass and cranial nerve involvement were the common findings. In the six patients with spinal lesions (one cervical, four thoracic and one thoracolumbar), local pain, progressive sensory motor deficits and bladder involvement were common presentations. Neuroimaging studies were performed in various combinations for preoperative evaluation and surgical planning. In cranial cases, multidisciplinary approaches were used to perform a near total or a total excision. In the spinal GCTs, only a partial excision was possible. Postoperative radiotherapy was delivered in ten patients. Histopathological studies were diagnostic. All patients were followed up for periods ranging from one to 10 years, with a mean of 2 years and 3 months. No significant increase in the residual tumor or recurrences, as seen on periodic neuroimaging studies, were noted during their follow-up period.
Copyright 2002 Harcourt Publishers Ltd.