Intraoperative radiotherapy to treat newly diagnosed solitary brain metastasis: initial experience and long-term outcomes.

OBJECT: The authors assessed the feasibility of intraoperative radiotherapy (IORT) using a portable radiation source to treat newly diagnosed, surgically resected, solitary brain metastasis (BrM). METHODS: In a nonrandomized prospective study, 23 patients with histologically confirmed BrM were treated with an Intrabeam device that delivered 14 Gy to a 2-mm depth to the resection cavity during surgery. RESULTS: In a 5-year minimum follow-up period, progression-free survival from the time of surgery with simultaneous IORT averaged (± SD) 22 ± 33 months (range 1-96 months), with survival from the time of BrM treatment with surgery+IORT of 30 ± 32 months (range 1-96 months) and overall survival from the time of first cancer diagnosis of 71 ± 64 months (range 4-197 months). For the Graded Prognostic Assessment (GPA), patients with a score of 1.5-2.0 (n = 12) had an average posttreatment survival of 21 ± 26 months (range 1-96 months), those with a score of 2.5-3.0 (n = 7) had an average posttreatment survival of 52 ± 40 months (range 5-94 months), and those with a score of 3.5-4.0 (n = 4) had an average posttreatment survival of 17 ± 12 months (range 4-28 months). A BrM at the treatment site recurred in 7 patients 9 ± 6 months posttreatment, and 5 patients had new but distant BrM 17 ± 3 months after surgery+IORT. Six patients later received whole-brain radiation therapy, 7 patients received radiosurgery, and 2 patients received both treatments. The median Karnofsky Performance Scale scores before and 1 and 3 months after surgery were 80, 90, and 90, respectively; at the time of this writing, 3 patients remain alive with a CNS progression-free survival of > 90 months without additional BrM treatment. CONCLUSIONS: The results of this study demonstrate the feasibility of resection combined with IORT at a dose of 14 Gy to a 2-mm peripheral margin to treat a solitary BrM. Local control, distant control, and long-term survival were comparable to those of other commonly used modalities. Surgery combined with IORT seems to be a potential adjunct to patient treatment for CNS involvement by systemic cancer.

Preoperative imaging of cervical spine hemangioblastomas using three-dimensional fusion digital subtraction angiography. Report of two cases.

Angiography is often performed to identify the vascular supply of hemangioblastomas prior to resection. Conventional two-dimensional (2D) digital subtraction (DS) angiography and three-dimensional (3D) DS angiography provides high-resolution images of the vascular structures associated with these lesions. However, such 3D DS angiography often does not provide reliable anatomical information about nearby osseous structures, or when it does, resolution of vascular anatomy in the immediate vicinity of bone is sacrificed. A novel angiographic reconstruction algorithm was recently developed at The Johns Hopkins University to overcome these inadequacies. By combining two separate sequences of images of bone and blood vessels in a single 3D representation, 3D fusion DS (FDS) angiography provides precise topographic information about vascular lesions in relation to the osseous environment, without a loss of resolution. In this paper, the authors present the cases of two patients with cervical spine hemangioblastomas who underwent preoperative evaluation with FDS angiography and then successful gross-total resection of their tumors. In both cases, FDS angiography provided high-resolution 3D images of the hemangioblastoma anatomy, including each tumor's topographic relationship with adjacent osseous structures and the location and size of feeding arteries and draining veins. These cases provide evidence that FDS angiography represents a useful adjunct to magnetic resonance imaging and 2D DS angiography in the preoperative evaluation and surgical planning of patients with vascular lesions in an osseous environment, such as hemangioblastomas in the spinal cord.

Familial Chiari type I malformation with syringomyelia in two siblings: case report and review of the literature.

OBJECTIVE: Familial cases of Chiari malformation with syringomyelia are rare. The majority of the reported series and case reports detail sporadic cases. The authors report two siblings who presented with Chiari type I malformation and syringomyelia (CMI+S). CLINICAL PRESENTATION: We report two sisters who each presented with scoliosis on routine school physicals. Their clinical examination was unremarkable; however, imaging studies demonstrated a Chiari malformation with syringomyelia. Both underwent cervicomedullary decompression, and follow-up imaging studies revealed resolution of the syringomyelia. CONCLUSION: A review of the literature reveals fewer than ten previous reports of familial CMI+S in the past 30 years. Although rare, the existence of familial cases of CMI+S suggests a genetic component to the pathogenesis of this condition in at least a proportion of patients. Neurosurgeons should be aware of the familial aggregation of CMI+S.