Solitary intracranial Rosai-Dorfman disease: case report and literature review.

Rosai-Dorfman disease is a well-established autoimmune histioproliferative disorder, and solitary central nervous system involvement is rare. A 35-year-old man presented with headache and transient blurred vision of 4 months' duration and weakness of the left extremities for 1 month. He had left temporal hemianopsia, a right nasal visual field defect of the upper medial quadrant and decreased muscle strength of the left extremities. Magnetic resonance imaging (MRI) showed a contrastenhancing tentorium-based lesion in the right trigone, intruding into the right lateral ventricle. The lesion was totally resected. Rosai-Dorfman disease was confirmed pathologically by an inflammatory infiltrate in the absence of an infectious agent, emperipolesis and positive S100 staining. From a review of the literature on Rosai-Dorfman disease in the central nervous system it is concluded that follow-up MRI should be performed in order to detect possible recurrence and that this rare entity should be considered in the differential diagnosis of intracranial lesions.

Surgical treatment of solid brain stem tumors in adults: a report of 22 cases.

BACKGROUND: Brain stem tumors in adults are infrequent. Most reports of surgical treatment for these tumors involve partial tumor removal in highly selected patients. A more aggressive approach for removing tumors, especially solid and intrinsic ones, has been controversial. METHODS: Twenty-two adult patients with brain stem tumors were surgically treated. Surgical techniques, potential risks, and selection of appropriate treatment were evaluated. RESULTS: Tumors were totally or subtotally removed in 20 patients and only partially removed in two patients. Serious complications such as respiratory disturbances and circulatory dysfunction occurred in 10 patients. Eight patients with these complications recovered after appropriate treatments. Upon discharge, most signs and symptoms improved in 17 patients. CONCLUSION: Most brain stem tumors, except for malignant gliomas and small ventral tumors, are amenable to an aggressive surgical approach. Exophytic medullary tumors that present dorsally comprise the most benign subgroup of brain stem tumors. Total removal can enhance survival, improve the patient's quality of life, and offer a favorable long-term prognosis. Appropriate management of postoperative complications is essential for good results.

Aggressive surgery for intramedullary tumor of cervical spinal cord.

BACKGROUND: Intramedullary tumors of the cervical spinal cord are common and many believe they are amenable to an aggressive approach. However, surgical removal of intramedullary tumors of the cervical spinal cord is still controversial because of the great risk of respiratory dysfunction or quadriplegia or both upon resection of the tumor. METHODS: We present a consecutive series of 58 patients who underwent surgical treatment for intramedullary tumors of the cervical spinal cord. Surgical results are analyzed to refine our indications for surgery and its timing. The roles of preoperative radiotherapy and posttherapeutic cord appearance and function are discussed. RESULTS: Tumors were totally resected in 50 (86.2%), subtotally in seven and partially in one patient. Forty-five (77.6%) patients had improved neurologic status postoperatively. Intramedullary tumors in the cervical area have better results from surgery than intramedullary tumors in other levels of the spine. Patients with moderate neurologic deficits can recover remarkably well after total tumor removal. Laser surgery is especially helpful for lipoma. Preoperative radiotherapy should be avoided because it is associated with difficult surgery and poor clinical outcome. The thin spinal cord can function surprisingly well. CONCLUSIONS: We conclude that intramedullary tumors of the cervical spinal cord are amenable to total surgical removal. Surgery is suitable when a patient presents with a moderate neurologic deficit. Proficient surgical technique for total tumor resection is necessary for good results. Preoperative radiotherapy contributes to difficult surgery and poor prognosis, and is not recommended.

Magnetic resonance imaging and microsurgical treatment of intramedullary hemangioblastoma of the spinal cord.

Thirteen cases of intramedullary hemangioblastoma of the spinal cord are reported. The tumors were diagnosed with radiological studies, especially magnetic resonance imaging. Microsurgery was used to achieve gross total removal of the tumors in all cases. Signs and symptoms improved in 84.6% of the patients after surgery. The authors describe the diagnosis and microsurgical techniques for the excision of intramedullary hemangioblastoma of the spinal cord. This disease should be differentiated from hydromyelia, intramedullary ependymoma, and vascular malformation of the spinal cord. Total removal of the tumor is recommended.