VHL-Associated Optic Nerve Hemangioblastoma Treated with Stereotactic Radiosurgery.

Central nervous system hemangioblastomas are generally restricted to the cerebellum, spinal cord, and brainstem. Supratentorial hemangioblastomas are uncommon, and optic nerve hemangioblastomas are extremely rare, with fewer than 25 reports including this case. In this report, we present the case of a 36-year-old woman with von Hippel-Lindau (VHL) disease who presented with progressive diminution of vision in the left eye due to a retrobulbar optic nerve hemangioblastoma. The patient had a history of cerebellar/spinal hemangioblastomas and pancreatic cysts, and her father and brother were patients with VHL disease. Gadolinium-enhanced magnetic resonance imaging showed intraorbital retrobulbar-enhanced mass on the left optic nerve. The optic nerve hemangioblastoma was treated with fractionated stereotactic radiosurgery using Novalis. Eighteen months after the stereotactic radiosurgery, the tumor volume decreased although the patient lost vision. This report presents an extremely rare case of optic nerve hemangioblastoma, which is the first case treated with stereotactic radiosurgery.

Gamma Knife surgery for patients with jugular foramen schwannomas: a multiinstitutional retrospective study in Japan.

OBJECTIVE This study aimed to explore the efficacy and safety of stereotactic radiosurgery in patients with jugular foramen schwannomas (JFSs). METHODS This study was a multiinstitutional retrospective analysis of 117 patients with JFSs who were treated with Gamma Knife surgery (GKS) at 18 medical centers of the Japan Leksell Gamma Knife Society. The median age of the patients was 53 years. Fifty-six patients underwent GKS as their initial treatment, while 61 patients had previously undergone resection. At the time of GKS, 46 patients (39%) had hoarseness, 45 (38%) had hearing disturbances, and 43 (36%) had swallowing disturbances. Eighty-five tumors (73%) were solid, and 32 (27%) had cystic components. The median tumor volume was 4.9 cm3, and the median prescription dose administered to the tumor margin was 12 Gy. Five patients were treated with fractionated GKS and maximum and marginal doses of 42 and 21 Gy, respectively, using a 3-fraction schedule. RESULTS The median follow-up period was 52 months. The last follow-up images showed partial remission in 62 patients (53%), stable tumors in 42 patients (36%), and tumor progression in 13 patients (11%). The actuarial 3- and 5-year progression-free survival (PFS) rates were 91% and 89%, respectively. The multivariate analysis showed that pre-GKS brainstem edema and dumbbell-shaped tumors significantly affected PFS. During the follow-up period, 20 patients (17%) developed some degree of symptomatic deterioration. This condition was transient in 12 (10%) of these patients and persistent in 8 patients (7%). The cause of the persistent deterioration was tumor progression in 4 patients (3%) and adverse radiation effects in 4 patients (3%), including 2 patients with hearing deterioration, 1 patient with swallowing disturbance, and 1 patient with hearing deterioration and hypoglossal nerve palsy. However, the preexisting hoarseness and swallowing disturbances improved in 66% and 63% of the patients, respectively. CONCLUSIONS GKS resulted in good tumor control in patients with either primary or residual JFSs. Although some patients experienced some degree of symptomatic deterioration after treatment, persistent adverse radiation effects were seen in only 3% of the entire series at the last follow-up. Lower cranial nerve deficits were extremely rare adverse radiation effects, and preexisting hoarseness and swallowing disturbances improved in two-thirds of patients. These results indicated that GKS was a safe and reasonable alternative to surgical resection in selected patients with JFSs.

Gamma Knife surgery for patients with facial nerve schwannomas: a multiinstitutional retrospective study in Japan.

OBJECTIVE: The aim of this study was to explore the efficacy and safety of stereotactic radiosurgery for patients with facial nerve schwannomas (FNSs). METHODS: This study was a multiinstitutional retrospective analysis of 42 patients with FNSs treated with Gamma Knife surgery (GKS) at 1 of 10 medical centers of the Japan Leksell Gamma Knife Society (JLGK1301). The median age of the patients was 50 years. Twenty-nine patients underwent GKS as the initial treatment, and 13 patients had previously undergone surgery. At the time of the GKS, 33 (79%) patients had some degree of facial palsy, and 21 (50%) did not retain serviceable hearing. Thirty-five (83%) tumors were solid, and 7 (17%) had cystic components. The median tumor volume was 2.5 cm(3), and the median prescription dose to the tumor margin was 12 Gy. RESULTS: The median follow-up period was 48 months. The last follow-up images showed partial remission in 23 patients and stable tumors in 19 patients. Only 1 patient experienced tumor progression at 60 months, but repeat GKS led to tumor shrinkage. The actuarial 3- and 5-year progression-free survival rates were 100% and 92%, respectively. During the follow-up period, 8 patients presented with newly developed or worsened preexisting facial palsy. The condition was transient in 3 of these patients. At the last clinical follow-up, facial nerve function improved in 8 (19%) patients, remained stable in 29 (69%), and worsened in 5 (12%; House-Brackmann Grade III in 4 patients, Grade IV in 1 patient). With respect to hearing function, 18 (90%) of 20 evaluated patients with a pure tone average of ≤ 50 dB before treatment retained serviceable hearing. CONCLUSIONS: GKS is a safe and effective treatment option for patients with either primary or residual FNSs. All patients, including 1 patient who required repeat GKS, achieved good tumor control at the last follow-up. The incidence of newly developed or worsened preexisting facial palsy was 12% at the last clinical follow-up. In addition, the risk of hearing deterioration as an adverse effect of radiation was low. These results suggest that GKS is a safe alternative to resection.

Magnetic resonance-guided percutaneous laser disk decompression for lumbar disk herniation--relationship between clinical results and location of needle tip.

BACKGROUND: Magnetic resonance-guided PLDD has been performed for patients with single-level contained focal disk herniation. We investigated the clinical results of our PLDD practice in reference to the site of the needle tip. METHODS: Thirty-two patients (24 male patients and 8 female patients; mean age, 35 years) who presented with low back pain and signs of monoradiculopathy underwent MR-guided PLDD. The final location of the needle tip was plotted on a T(1)-weighted axial plane of the targeted disk. An axial image of the targeted disk was divided into 4 quadrants in a cruciform fashion and into 3 zones in a concentric circle. The clinical results as evaluated by MacNab's criteria 6 months after PLDD were investigated in each subdivided area where the needle tip was located. RESULTS: The overall success rate of our PLDD practice was 68.8%. The success rate in the 27 patients who had the needle tip in the DL quadrant of the targeted disk was 70.4%. Among them, 14 patients who had the needle tip in the middle zone showed a favorable outcome (77.8%). CONCLUSIONS: The final location of the needle tip was demonstrated in all cases. Although our MR imaging study was not sufficient to determine the exact position of the needle tip because of MR image distortion, such error seems to be acceptable in the results of our study obtained from the relative positioning of the needle tip in the disk space. This study suggests that the middle zone in the DL quadrant of the targeted disk space seems to be a favorable target to obtain better clinical outcomes.

Plasma cell myeloma of the skull base: report of two cases.

Plasma cell myeloma (PCM) of the skull base is rarely encountered in neurosurgical practice. PCM has a wide spectrum of pathology, including a quite benign, solitary plasmacytoma (SPC), and an extremely malignant, multiple myeloma (MM) at the two ends of the spectrum. We have described two patients with PCM of the skull base, of which one harbored SPC, and the other progressed to MM. In case 1, a 46-year-old man presented with left multiple cranial nerve impairments and had a large skull base tumor. Subtotal removal was performed. The specimen and general examination, including bone marrow aspiration, revealed SPC. Postoperatively 50Gy of external radiotherapy was administered. The patient has no manifestation of MM 24 months after the initial presentation. In case 2, a 53-year-old woman presented with left abducens palsy and had a left petroclival osteolytic mass. Gross total resection was performed. The specimen revealed a plasmablastic tumor, i.e., myeloma. General examination established the diagnosis of MM. She was administrated adjuvant chemotherapy and autologous bone marrow transplantation. She is alive without local recurrence 30 months after the initial presentation.

[A case of post-traumatic pseudoaneurysm on the posterio meningeal artery].

A case is reported of post-traumatic delayed appearance of a pseudoaneurysm on the posterior meningeal artery. In the immediate post-traumatic stage, the patient had mild disturbance of consciousness, mild subarachnoid hemorrhage, and mid-line skull fracture of the posterior cranial fossa. At 15 hours after the trauma, the patient suddenly developed deep coma and respiratory arrest. CT scanning showed further spreading of the subarachnoid hemorrhage and newly detected subdural hemorrhage. After the patient regained spontaneous respiration, cerebral angiography was performed 24 hours after admission. This initial angiography showed no aneurysm, but at 17 days after the trauma, follow-up angiography demonstrated the delayed appearance of an aneurysmal dilatation on the posterior meningeal artery. The aneurysm was resected surgically and proved to be a pseudoaneurysm. Having developed normal pressure hydrocephalus afterwards, the patient received a V-P shunt, and was discharged 150 days after the trauma with no major neurological deficit. We discussed the indication for and timing of angiography for detecting pseudoaneurysms. Posterior meningeal artery pseudoaneurysms should be resected surgically, because intravascular surgery through the vertebral artery involves considerable risk of embolic complications.