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PURPOSE Since it is known that midline located glioma with H3K27 mutation in children has a disastrous prognosis, in 2016 WHO classification, these tumors were defined as diffuse midline glioma (DMG) and classified as grade IV. A lot of papers about pediatric DMG have been published, but there are not many papers about adult DMG. In this study, we aimed to identify factors affecting the prognosis of adult midline glioma. This is the first paper to study the prognosis of adult DMG according to histological grade and is the largest study to investigate the survival of adult DMG. METHODS We reviewed the chart of adult patients diagnosed with midline glioma with H3K27M mutation after undergoing resection or biopsy among patients who visited our institution between January 2010 and December 2020. Survival analysis was performed according to tumor location, histological grading, Karnofsky Performance Scale (KPS), and age. RESULTS Among the 125 adult midline gliomas we identified, 45 (36.0%) had the H3K27M mutation. As a result of survival analysis performed on 125 adult midline gliomas, low histological grade, KPS ≥ 80, and age ≤ 60 showed significantly better survival. After adjusting for age, the difference in survival between H3K27M mutation and wildtype group was not significant. As a result of survival analysis performed on 45 DMG, low histological grade, KPS ≥ 80, total resection, and concurrent chemoradiation therapy group showed significantly better survival. CONCLUSION In adult midline glioma, disastrous prognosis due to H3K27M mutation was not observed as in children. The prognosis of adult midline gliomas is determined by histological grade, age, KPS, and extent of tumor resection. Therefore, the current WHO classification, which classifies all H3K27M mutant midline gliomas as grade IV regardless of histological diagnoses, is not suitable for adults.

BACKGROUND Endoscopic transorbital approach (ETOA) has been proposed as a minimally invasive technique for the treatment of skull base lesions located around mesial temporal lobe (MTL), mostly extra-axial pathology. OBJECTIVE To explore the feasibility of ETOA in accessing intraparenchymal MTL with cadaveric specimens and describe our initial clinical experience of ETOA for intra-axial lesions in MTL. METHODS Anatomic dissections were performed in 4 adult cadaveric heads using a 0° endoscope. First, a stepwise anatomical investigation of ETOA to intraparenchymal MTL was explored. Then, ETOA was applied clinically for 7 patients with intra-axial lesions in MTL, predominantly high-grade gliomas (HGGs) and low-grade gliomas (LGGs). RESULTS The extradural stage of ETOA entailed a superior eyelid incision followed by orbital retraction, drilling of orbital roof, greater and lesser wing of sphenoid bone, and cutting of the meningo-orbital band. For the intradural stage, the brain tissue medial to the occipito-temporal gyrus was aspirated until the temporal horn was opened. The structures of MTL could be aspirated selectively in a subpial manner without injury to the neurovascular structures of the ambient and sylvian cisterns, and the lateral neocortex. After cadaveric validation, ETOA was successfully performed for 4 patients with HGGs and 3 patients with LGGs. Gross total resection was achieved in 6 patients (85.7%) without significant surgical morbidities including visual field deficits. CONCLUSION ETOA provides a logical line of access for intra-axial lesions in MTL. The safe and natural surgical trajectory of ETOA can spare brain retraction, neurovascular injury, and disruption of the lateral neocortex.

BACKGROUND Orbital angioleiomyoma is generally considered a rare tumor; approximately 40 cases have been reported. However, after their experience with 6 consecutive cases in their single institution during 3 years, the authors speculate that the incidence of orbital angioleiomyomas is possibly underestimated. OBSERVATIONS A 34-year-old female presented with progressive exophthalmos of 2 years’ duration. Orbital computed tomography and magnetic resonance imaging revealed a well-circumscribed orbital tumor with partial and heterogeneous gadolinium enhancement. Technetium-99m red blood cell single-photon emission computed tomography showed positive perfusion in the late blood-pool phase, which was exactly consistent with the finding of a cavernous hemangioma. Under the impression of a cavernous hemangioma, the authors accessed the mass with an endoscopic endonasal approach and completely removed it without neurological deficit. Pathological examination revealed that the final diagnosis was an angioleiomyoma with positive immunostaining results for smooth muscle actin (SMA). LESSONS The incidence of orbital angioleiomyomas may not be very low, as these lesions have possibly been misdiagnosed as orbital cavernous hemangiomas because of their histological similarity. Preoperative presumption and differentiation from cavernous hemangiomas are very challenging because of the rarity of orbital angioleiomyoma and similar radiological findings. SMA immunostaining may be critical to differentiate orbital angioleiomyomas from cavernous hemangiomas.

OBJECTIVE The endoscopic transorbital approach (ETOA) has been developed, permitting a new surgical corridor. Due to the vertical limitation of the ETOA, some lesions of the anterior cranial fossa are difficult to access. The ETOA with superior-lateral orbital rim (SLOR) osteotomy can achieve surgical freedom of vertical as well as horizontal movement. The purpose of this study was to confirm the feasibility of the ETOA with SLOR osteotomy. METHODS Anatomical dissections were performed in 5 cadaveric heads with a neuroendoscope and neuronavigation system. ETOA with SLOR osteotomy was performed on one side of the head, and ETOA with lateral orbital rim (LOR) osteotomy was performed on the other side. After analysis of the results of the cadaveric study, the ETOA with SLOR osteotomy was applied in 6 clinical cases. RESULTS The horizontal and vertical movement range through ETOA with SLOR osteotomy (43.8° ± 7.49° and 36.1° ± 3.32°, respectively) was improved over ETOA with LOR osteotomy (31.8° ± 5.49° and 23.3° ± 1.34°, respectively) (p < 0.01). Surgical freedom through ETOA with SLOR osteotomy (6025.1 ± 220.1 mm3) was increased relative to ETOA with LOR osteotomy (4191.3 ± 57.2 mm3) (p < 0.01); these values are expressed as the mean ± SD. Access levels of ETOA with SLOR osteotomy were comfortable, including anterior skull base lesion and superior orbital area. The view range of the endoscope for anterior skull base lesions was increased through ETOA with SLOR osteotomy. After SLOR osteotomy, the space for moving surgical instruments and the endoscope was widened. Anterior clinoidectomy could be achieved successfully using ETOA with SLOR osteotomy. The authors performed ETOA with SLOR osteotomy in 6 cases of brain tumor. In all 6 cases, complete removal of the tumor was successfully accomplished. In the 3 cases of anterior clinoidal meningioma, anterior clinoidectomy was performed easily and safely, and manipulation of the extended dural margin and origin dura mater was possible. There was no complication related to this approach. CONCLUSIONS The authors evaluated the clinical feasibility of ETOA with SLOR osteotomy based on a cadaveric study. ETOA with SLOR osteotomy could be applied to more diverse disease groups that do not permit conventional ETOA or to cases in which surgical application is challenging. ETOA with SLOR osteotomy might serve as an opportunity to broaden the indication for the ETOA.

Context In the management of growth hormone (GH)–secreting pituitary adenomas, the oral glucose tolerance test (OGTT) has been the gold standard not only for diagnoses but also for the determination of biochemical remission. Insulin-like growth factor-1 (IGF-1) is an essential biomarker, although it should be adjusted for both age and sex. Objective We evaluated whether IGF-1 levels could serve as a reliable alternative to an OGTT for disease monitoring after the surgical treatment of acromegaly. We retrospectively reviewed the medical records of 320 patients who underwent surgical resection of their GH-secreting pituitary tumors at the Severance hospital. Receiver operator characteristic (ROC) analyses were performed to validate the accuracy of IGF-1 levels for the assessment of remission. In addition, regression analyses were performed to identify factors associated with discrepancy between OGTT and IGF-1 levels. Results Except for 1 week after surgery, ROC analyses showed an area under the curve of greater than 0.8 for IGF-1 at all time points. Of 320 patients, 270 achieved endocrine remission after surgery alone. Among these patients, IGF-1 levels were normalized in 250 patients. The mean duration from surgery to IGF-1 normalization was 4.7 months. Regression analyses demonstrated that risk of failed IGF-1 normalization was increased by 3.1-fold when the tumor invaded the cavernous sinus and increased by 9.0-fold in patients with incomplete tumor removal. Conclusion IGF-1 level is a reliable alternative to OGTT and plays a valuable role in monitoring acromegaly status.

Background Haemodynamic changes after sympathetic stimuli like tracheal intubation are more pronounced in hypertensive patients than in normotensive patients. Heart rate (HR)-corrected QT (QTc) interval changes related to intubation may also be more prominent in hypertensive patients. We hypothesised that there would be a difference in the effect-site concentration (Ce) of remifentanil to attenuate QTc interval prolongation in normotensive and hypertensive patients following intubation. Methods Twenty-two normotensive and 22 hypertensive female patients (≥ 50 year) were enrolled and anaesthesised with remifentanil and propofol using a target-controlled infusion. All hypertensive patients received oral antihypertensive medications for > 6 months and took the medications in the morning of the surgery. The effective concentration of remifentanil Ce in 50% (EC50) and 95% of the population (EC95) required to maintain QTc interval prolongation < 15 ms following intubation was calculated using the isotonic regression and a bootstrapping approach following the Dixon's up-and-down method. Results Median duration of hypertension was 6 years. Isotonic regression revealed that the EC50 (83% confidence interval) of remifentanil Ce for reducing QTc interval prolongation following intubation was 3.8 (3.5-4.1) ng/ml in normotensive and 6.1 (5.8–6.2) ng/ml in hypertensive female patients. The EC95 (95% confidence interval) of remifentanil Ce was 4.4 (4.3–4.5) ng/ml in normotensive and 6.5 (6.4–6.5) ng/ml in hypertensive female patients. Conclusion The Ce of remifentanil required to attenuate QTc interval prolongation following intubation was significantly higher in hypertensive patients than it was in normotensive patients. Thus, more caution should be taken related to QTc interval prolongation when intubating hypertensive patients.