Central Nervous System Dissemination of Solitary Sporadic Supratentorial Hemangioblastoma: A Case Report and Literature Review.

We report a patient with whole neuroaxis dissemination of a sporadic supratentorial hemangioblastoma (HB) for more than 15 years. A 68-year-old female patient presented with severe radiating pain in the right leg. Gadolinium-enhanced lumbar spine MRI showed an intradural mass (2.5 cm in diameter) at the L4 level. The patient had been severely disabled for 22 years after a previous intraventricular brain tumor resection. At that time, the diagnosis was angioblastic meningioma, which was thought to be incorrect. At 14 years after the brain surgery, gamma knife radiosurgery was performed three times for newly developed or recurred supratentorial and infratentorial tumors in the cerebrospinal fluid pathway. The patient underwent lumbar spinal surgery, and a gross total removal of the mass was performed, which confirmed the histopathological diagnosis of HB. We reexamined the old histopathological specimen of the intraventricular tumor from 20 years ago and changed the diagnosis from angioblastic meningioma to supratentorial HB. Six months after spinal surgery, the patient underwent a second spinal surgery and brain surgery, and the histopathological diagnosis was HB following both surgeries, which was the same following the first spinal surgery. Here, we report a sporadic supratentorial HB patient who showed cranial and spinal disseminations for more than two decades along with a literature review.

Gamma knife radiosurgery as primary management for intracranial meningioma identified as growing on serial imaging.

Gamma knife radiosurgery (GKRS) has emerged as a highly effective therapeutic modality for the management of intracranial meningiomas. However, the role of GKRS in treating growing meningiomas detected during active surveillance remains unclear. This study seeks to investigate the long-term outcomes of GKRS treatment for growing meningiomas. A retrospective analysis was conducted on patients who underwent GKRS as the primary treatment for growing meningiomas between 2004 and 2021. Growing meningiomas were defined as those exhibiting a > 10% increase in tumor volume (TV) compared to the previous imaging. Fifty-nine patients who received GKRS as their initial treatment were included, with a minimum follow-up period of 12 months. Comprehensive clinical, radiological, and procedural data were analyzed. Serial TV assessments were performed for all tumors before and after GKRS. Tumor progression and regression were defined as a > 10% increase or decrease in TV, respectively, compared to the pretreatment image. At a median follow-up of 41 months (range 15-197 months), TV was unchanged in 16 patients (27.1%), decreased in 41 patients (69.5%), and increased in 2 patients (3.4%). Multivariate analysis revealed that both TV (cm3) (hazard ratio [HR], 1.107; 95% confidence interval [CI], 1.002-1.222; P = .045) and volume growing rate (%/yr) (HR, 1.013; 95% CI, 1.000-1.025; P = .04) significantly correlated with tumor progression. Eleven patients (18.6%) experienced new or worsening symptoms. In multivariate analysis, factor predicting new or worsening neurological function was preexisting calcification (HR, 5.297; 95% CI, 1.328-21.124; P = .018). GKRS demonstrates a promising level of tumor control with minimal risk of neurological deterioration when applied to growing meningiomas. These findings provide compelling support for considering GKRS as a valuable therapeutic option following an initial period of active surveillance for these tumors.

Intracranial-intracranial bypass strategies for the treatment of complex intracranial aneurysms: anatomical characteristics and surgical intervention.

OBJECTIVE: The effectiveness of revascularization for complex aneurysms is well-established. This study aimed to describe the technical characteristics and clinical efficacy of intracranial-to-intracranial (IC-IC) bypass for the treatment of complex intracranial aneurysms. METHODS: We retrospectively reviewed all patients with aneurysms who underwent a preplanned combination of surgical or endovascular treatment and IC-IC bypass at our institution between January 2006 and September 2023. IC-IC bypass techniques included four strategies: type A (end-to-end reanastomosis), type B (end-to-side reimplantation), type C (in situ side-to-side anastomosis), and type D (IC-IC bypass with a graft vessel). RESULTS: During the study period, ten patients with aneurysms each underwent IC-IC bypass surgery. Aneurysms were located in the middle cerebral artery (60.0%), anterior temporal artery (10.0%), anterior cerebral artery (20.0%), and vertebral artery (10.0%). There were three saccular aneurysms (30.0%), two fusiform aneurysms (20.0%), one dissecting aneurysm (10.0%), and four pseudoaneurysms (40.0%). We performed the type A strategy on five patients (50.0%), type B on one (10.0%), type C on one (10.0%), and type D on three (30.0%). During a mean period of 68.3 months, good clinical outcomes (modified Rankin Scale score, 0-2) were observed in all patients. Follow-up angiography demonstrated complete aneurysmal obliteration in all patients and good bypass patency in nine of ten patients (90.0%). CONCLUSION: The treatment of complex aneurysms remains a challenge with conventional surgical or endovascular treatments. IC-IC bypass surgery is a useful technique, associated with favorable clinical outcomes, for treating complex aneurysms.

Intracranial Efficacy of Systemic Therapy in Patients with Asymptomatic Brain Metastases from Lung Cancer.

There has been controversy over whether to radiologically follow up or use local treatment for asymptomatic small-sized brain metastases from primary lung cancer. For brain tumors without local treatment, we evaluated potential factors related to the brain progression and whether systemic therapy controlled the tumor. We analyzed 96 patients with asymptomatic small-sized metastatic brain tumors from lung cancer. These underwent a radiologic follow-up every 2 or 3 months without local treatment of brain metastases. The pathologies of the tumors were adenocarcinoma (n = 74), squamous cell carcinoma (n = 11), and small cell carcinoma (n = 11). The primary lung cancer was treated with cytotoxic chemotherapy (n = 57) and targeted therapy (n = 39). Patients who received targeted therapy were divided into first generation (n = 23) and second or third generation (n = 16). The progression-free survival (PFS) of brain metastases and the overall survival (OS) of patients were analyzed depending on the age, tumor pathology, number, and location of brain metastases, the extent of other organ metastases, and chemotherapy regimens. The median PFS of brain metastases was 7.4 months (range, 1.1-48.3). Targeted therapy showed statistically significant PFS improvement compared to cytotoxic chemotherapy (p = 0.020). Especially, on univariate and multivariate analyses, the PFS in the second or third generation targeted therapy was more significantly improved compared to cytotoxic chemotherapy (hazard ratio 0.229; 95% confidence interval, 0.082-0.640; p = 0.005). The median OS of patients was 13.7 months (range, 2.0-65.0). Univariate and multivariate analyses revealed that the OS of patients was related to other organ metastases except for the brain (p = 0.010 and 0.020, respectively). Three out of 52 patients with brain recurrence showed leptomeningeal dissemination, while the recurrence patterns of brain metastases were mostly local and/or distant metastases (94.2%). Of the 52 patients who relapsed, 25 patients received local brain treatment. There was brain-related mortality in two patients (2.0%). The intracranial anti-tumor effect was superior to cytotoxic chemotherapy in the treatment of asymptomatic small-sized brain metastases with targeted therapy. Consequently, it becomes possible to determine the optimal timing for local brain treatment while conducting radiological follow-up for these tumors, which do not appear to increase brain-related mortality. Furthermore, this approach has the potential to reduce the number of cases requiring brain local treatment.

Neurenteric cyst of the craniovertebral junction treated to reduce recurrence using different strategies: Two case reports and a literature review.

RATIONALE: Neurenteric cysts are rare benign lesions that are usually located in the lower cervical and upper thoracic spine and extremely rare in the craniovertebral junction. It is generally challenging to completely remove the neurenteric cysts of the craniovertebral junction. We report the cases of 2 patients with neurenteric cyst in the ventral craniovertebral junction managed using different treatment strategies. PATIENT CONCERNS: The first patient was a 64-year-old man. He man was admitted with headache, posterior neck pain, and a tingling sensation in both the forearms. The second patient was a 53-year-old woman. She was admitted with tingling sensations and numbness in both the hands and feet. DIAGNOSES: Cervical spine magnetic resonance imaging showed 2 intradural extramedullary cystic lesions in case 1 and a C2 to C3 intradural extramedullary cystic mass in case 2. INTERVENTIONS AND OUTCOMES: The patient of the case 1 underwent a left C1 to C2 hemi-laminectomy and the cysts were completely removed. Eleven years after the surgery, there was no recurrence. In case 2, we performed a left C2 to C3 hemi-laminectomy and removed only a part of the outer membrane to enable sufficient communication with the surrounding normal subarachnoid space. After removing the cyst wall, the patient underwent C1 to 2 trans articular screw fixation to prevent cervical instability. Ten years after surgery, there was no recurrence of the cyst or new lesions. LESSONS: Clinicians should consider neurenteric cyst in the differential diagnosis of arachnoid cyst or epidermoid cyst. If performing a complete surgical removal is difficult, partial surgical removal, using a cysto-subarachnoid shunt and stabilization, such as screw fixation, could be an alternative treatment option to reduce the risk of mortality and morbidity.

Distinct Specialized Center of Excellence, the Story of Hwasun Neurosurgery at Chonnam National University Hwasun Hospital.

The paper provides a comprehensive overview of the growth and development of Hwasun Neurosurgery at Chonnam National University Hwasun Hospital over the past 18 years. As the first brain tumor center in Korea when it was established in April 2004, Hwasun Neurosurgery has since become one of the leading institutions in brain tumor education and research in the country. Its impressive clinical and basic research capabilities, dedication to professional education, and numerous academic achievements have all contributed to its reputation as a top-tier institution. We hope this will become a useful guide for other brain tumor centers or educational institutions by sharing the story of Hwasun Neurosurgery.

The Tumor Control According to Radiation Dose of Gamma Knife Radiosurgery for Small and Medium-Sized Brain Metastases from Non-Small Cell Lung Cancer.

OBJECTIVE: The effectiveness of gamma knife radiosurgery (GKR) in the treatment of brain metastases is well established. The aim of this study was to evaluate the efficacy and safety of maximizing the radiation dose in GKR and the factors influencing tumor control in cases of small and medium-sized brain metastases from non-small cell lung cancer (NSCLC). METHODS: We analyzed 230 metastatic brain tumors less than 5 mL in volume in 146 patients with NSCLC who underwent GKR. The patients had no previous radiation therapy for brain metastases. The pathologies of the tumors were adenocarcinoma (n=207), squamous cell carcinoma (n=18), and others (n=5). The radiation doses were classified as 18, 20, 22, and 24 Gy, and based on the tumor volume, the tumors were categorized as follows : small-sized (less than 1 mL) and medium-sized (1-3 and 3-5 mL). The progression-free survival (PFS) of the individual 230 tumors and 146 brain metastases was evaluated after GKR depending on the pathology, Eastern Cooperative Oncology Group (ECOG) performance score (PS), tumor volume, radiation dose, and anti-cancer regimens. The radiotoxicity after GKR was also evaluated. RESULTS: After GKR, the restricted mean PFS of individual 230 tumors at 24 months was 15.6 months (14.0-17.1). In small-sized tumors, as the dose of radiation increased, the tumor control rates tended to increase (p=0.072). In medium-sized tumors, there was no statistically difference in PFS with an increase of radiation dose (p=0.783). On univariate analyses, a statistically significant increase in PFS was associated with adenocarcinomas (p=0.001), tumors with ECOG PS 0 (p=0.005), small-sized tumors (p=0.003), radiation dose of 24 Gy (p=0.014), synchronous lesions (p=0.002), and targeted therapy (p=0.004). On multivariate analyses, an improved PFS was seen with targeted therapy (hazard ratio, 0.356; 95% confidence interval, 0.150-0.842; p=0.019). After GKR, the restricted mean PFS of brain at 24 months was 9.8 months (8.5-11.1) in 146 patients, and the pattern of recurrence was mostly distant within the brain (66.4%). The small and medium-sized tumors treated with GKR showed radiotoxicitiy in five out of 230 tumors (2.2%), which were controlled with medical treatment. CONCLUSION: The small-sized tumors were effectively controlled without symptomatic radiation necrosis as the radiation dose was increased up to 24 Gy. The medium-sized tumors showed potential for symptomatic radiation necrosis without signifcant tumor control rate, when greater than 18 Gy. GKR combined targeted therapy improved the tumor control of GKR-treated tumors.

Tumor control of third ventricular central neurocytoma after gamma knife radiosurgery in an elderly patient: A case report and literature review.

RATIONALE: Central neurocytoma is rare benign tumor that occurs in high probability in young adults in the lateral ventricle. Herein, we report an unusual case of an elderly woman who was diagnosed with central neurocytoma isolated to the third ventricle. This deeply located tumor was effectively treated using gamma knife radiosurgery (GKR). PATIENT CONCERNS: A 79-year-old woman was admitted to hospital with gait disturbance and cognitive dysfunction. DIAGNOSIS: Brain magnetic resonance imaging (MRI) revealed a homogenously enhancing multilobulated mass in the posterior third ventricle measuring 1.8 cm in size. The tumor was diagnosed as a central neurocytoma isolated to the third ventricle. INTERVENTIONS: Neuronavigation-guided endoscopic third ventriculostomy and biopsy were performed. One week following surgery, GKR was performed using a prescribed dose of 14 Gy with 50% isodose lines, and a target volume of 1.62 cc. OUTCOMES: Three months after GKR, brain MRI revealed a decrease in the size (to 1.4 cm) of the multilobulated strong enhancing mass in the posterior third ventricle, and the patient's symptom of confusion was improved. LESSONS: Previous studies have reported that tumors in unusual locations, such as those isolated to the third ventricle, are different according to age, either in young children or elderly individuals. Although complete surgical resection is an effective treatment for central neurocytoma, it is often difficult to approach these tumors through surgery. GKR could, therefore, be an alternative primary treatment option for deeply located central neurocytomas in elderly patients.