Optimal timing in the treatment of intracranial hemangioblastoma (HB) remains controversial, particularly for patients of von Hippel–Lindau disease (VHL) with multiple small lesions. We evaluated efficacy of stereotactic radiosurgery (SRS) for intracranial HB based on the longer follow-up data with a larger number of patients and lesions. Twenty-one patients (11 men, 10 women) initially underwent SRS for 57 intracranial HBs. Seven patients had sporadic lesions and 14 had VHL-related lesions. During the follow-up, 40 lesions were additionally treated in VHL patients in 10 SRS treatments. Thus, a total of 97 lesions were included in this study. Median tumor volume was 0.13 cm3 (range, 0.004–9.5 cm3), and median margin dose was 18 Gy (range, 14–20 Gy). Median duration of follow-up was 96 months (range, 3–235 mo) after initial SRS treatment. Ten tumors in 7 patients showed progression after SRS. Actuarial tumor control rates after SRS at 5 and 10 years were 94% and 80%, respectively. Factors associated with longer control were solid lesion (P = .03), smaller volume (P = .01), and lesions associated with VHL (P = .0005) in univariate analysis. Five- and 10-year tumor control rates were 67% and 44% for sporadic patients and 97% and 83% for VHL patients. SRS could be offered as an effective treatment for small, solid, and VHL-associated HBs. If the tumors show apparent enlargement in size or can possibly become symptomatic along with a slight increase in size, SRS should be recommended before they present with the clinical symptoms.
Object: The goal of this study was to assess the efficacy of Gamma Knife surgery (GKS) in the management of dural arteriovenous fistulas (dAVFs). Methods: The authors performed a retrospective analysis of a group of 22 patients who underwent GKS for dAVFs at the University of Tokyo Hospital between 1991 and 2009. The patients underwent CT or MR imaging with contrast enhancement every 6 months after GKS; when obliteration of a dAVF was indicated by these images, patients also underwent angiography. Follow-up in these patients ranged from 12 months to 100 months (median 33 months) after GKS. Results: Obliteration of the dAVF was confirmed by neuroimaging in 12 patients (55%). According to a Kaplan-Meier analysis, obliteration rates for the dAVFs were 51% at 3 years and 80% at 5 years. The obliteration rate for lesions without cortical venous drainage (CVD) was 86%, which was significantly higher than the rate for dAVFs with CVD (47%) (p = 0.007). Hemorrhage at presentation (p = 0.03), a target volume less than 1.5 cm3 (p = 0.009), and Cognard Type III or IV dAVF (p = 0.005) were factors associated with a higher obliteration rate. Among 10 patients whose dAVFs were not obliterated by the initial GKS, 5 patients underwent additional treatment and complete obliteration was achieved in all. Relief of tinnitus was obtained in 5 (83%) of 6 patients with transverse-sigmoid sinus dAVFs, and ophthalmic symptoms improved in 2 (67%) of 3 patients with cavernous sinus dAVFs. No patient experienced interval hemorrhage or radiation-induced complications after treatment. Conclusions: Gamma Knife surgery is a safe and effective treatment for dAVF. It can be a first line of therapy in the multidisciplinary treatment strategy for dAVFs, especially when significant morbidity is anticipated with other therapeutic options. One should be very careful about recommending GKS for patients harboring dAVFs with CVD because of the expected natural history of such a lesion and the possibility of other therapeutic options.
The authors present a case of germinoma that was initially found in the pineal region and recurred 15 years later in the intramedullary cervical spinal cord after intensive chemo- and radiotherapy and diagnosis of complete remission. This 28-year-old man initially presented with seizures. Hydrocephalus and a pineal tumor were found on radiological examination, and partial resection of the tumor was performed. Histological diagnosis showed a pure germinoma. Following surgery, the patient received a combination of chemo- and radiotherapy, and a complete remission was shown. However, after 15 years of follow-up, he presented with gait disturbances. Spinal MRI showed an intramedullary mass lesion in the cervical spinal cord. The cervical lesion was biopsied, and histological examination again revealed a pure germinoma. With germinomas, the possibility of a drop metastasis from an intracranial lesion to the spinal cord must be considered during follow-up. However, in the present case, analysis of a CSF sample showed no abnormalities as in previously published cases. In recent years, multidisciplinary treatments have demonstrated good event-free survival rates in cases of pure germinomas, but long-term outcomes over the decades are not fully known. Continual follow-up of such cases is recommended even after complete remission has been achieved.