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Craniopharyngiomas remain surgically challenging because of the strong adhesion to vital neurovascular structures. We propose a system for the selection of surgical approaches based on the optic recess (OR) displacement pattern to facilitate the surgical planning and obtain the optimum visual and endocrinological outcomes.Craniopharyngiomas were divided into three types based on the OR displacement pattern: superior, anterior, and involvement types. Selected surgical approaches and patient outcome were retrospectively reviewed according to these classifications. Visual and endocrinological outcomes were compared between the groups.This study included 26 patients with primary craniopharyngiomas who underwent surgery at our institution, classified into 11 anterior, 11 superior, and 4 involvement types. Extended endoscopic endonasal approach provided excellent exposure infero-dorsal aspect of the chiasm for manipulation of the dissection plane in the anterior and superior types with midline location. Unilateral subfrontal approach was required for tumor of the superior type with lateral extension. Interhemispheric translamina terminalis approach (TLA) could provide safe dissection under direct vision of strong adhesion at the superior aspect of the chiasm in the involvement type. Visual and endocrinological outcomes were better in the involvement type compared with the superior and anterior types. Visual outcome was significantly correlated with preoperative visual function.Craniopharyngiomas with the involvement type are indicated for TLA to achieve the best visual and endocrinological outcome. Our classification of OR displacement pattern is useful to select the optimal surgical approach for craniopharyngiomas more accurately and concisely, especially in cases with third ventricular extension.

After the new molecular-based classification was reported to be useful for predicting prognosis, the T2-fluid-attenuated inversion recovery (FLAIR) mismatch sign has gained interest as one of the promising methods for detecting lower grade gliomas (LGGs) with isocitrate dehydrogenase (IDH) mutations and chromosome 1p/19q non-codeletion (IDH mut-Noncodel) with high specificity. Although all institutions could use T2-FLAIR mismatch sign without any obstacles, this sign was not completely helpful because of its low sensitivity. In this study, we attempted to uncover the mechanism of T2-FLAIR mismatch sign for clarifying the cause of this sign's low sensitivity. Among 99 patients with LGGs, 22 were T2-FLAIR mismatch sign-positive (22%), and this sign as a marker of IDH mut-Noncodel showed a sensitivity of 55.6% and specificity of 96.8%. Via pathological analyses, we could provide evidence that not only microcystic changes but the enlarged intercellular space was associated with T2-FLAIR mismatch sign (p = 0.017). As per the molecular analyses, overexpression of mTOR-related genes (m-TOR, RICTOR) were detected as the molecular events correlated with T2-FLAIR mismatch sign (p = 0.020, 0.030. respectively). Taken together, we suggested that T2-FLAIR mismatch sign could pick up the IDH mut-Noncodel LGGs with enlarged intercellular space or that with overexpression of mTOR-related genes.

PURPOSE Half of the surgically proven Rathke's cleft cysts (RCCs) can be preoperatively misdiagnosed as cystic pituitary adenoma (CPA). We aimed to evaluate the usefulness of contrast-enhanced (CE) 3D T2 fluid-attenuated inversion-recovery (3D T2-FLAIR) imaging for differentiating between CPA and RCC. METHODS This retrospective study included six patients with RCC (all pathologically confirmed) and six patients with CPA (five pathologically confirmed, one clinically diagnosed). The 12 patients underwent pre- and post-contrast T1-weighted (T1W)- and 3D T2-FLAIR imaging at 3T. Based on the degree of enhancement of the lesion wall, two radiologists independently scored the images using a 3-point grading system. Interobserver agreement was calculated by using the κ coefficient. The statistical significance of grading differences was analyzed with the Mann-Whitney U-test. Another neuroradiologist first interpreted conventional MR images (1st session), and then the reader read images to which the 3D T2-FLAIR images had been added (2nd session). Sensitivity, specificity, and accuracy of the reader's interpretation were calculated. RESULTS Interobserver agreement for post-contrast T1W- and 3D T2-FLAIR images was excellent (κ = 1.000 and 0.885, respectively). Although the mean enhancement grade on post-contrast T1W images of RCCs and CPAs was not significantly different, on post-contrast 3D T2-FLAIR images it was significantly higher for RCCs and CPAs (P < 0.05). Three CPAs (50%) showed remarkable, donut-like enhancement along the inner margin of the cyst on CE-3D T2-FLAIR images; this was not the case on CE-T1W images. The sensitivity, specificity, and accuracy of the 2nd session were 1.00, 0.83, and 0.92, respectively, which were improved compared to the 1st session (1.00, 0.50, and 0.75, respectively). CONCLUSION CE-3D FLAIR imaging is useful for discriminating CPAs and RCCs.

Cerebellar hemangioblastomas remain surgically challenging because of the narrow, deep surgical corridors and tumor hypervascularity. Various surgical approaches are used according to the location, but optimal approaches have not been established. We propose a system of surgical approaches based on the venous drainage systems to facilitate surgical planning and achieve acceptable neurological outcomes. Cerebellar hemangioblastomas were divided into fivefour types based on the main drainage systems: suboccipital hemangioblastomas draining to the transverse sinus (TS) or torcular, tentorial hemangioblastomas draining to the tentorial and straight sinus, petrosal hemangioblastomas draining to the superior petrosal sinus (SPS),, and quadrigeminal hemangioblastomas draining to the galenic system, and tonsillar hemangioblastomas draining to the TS or torcular in conjunction with jugular bulb or SPS. Microsurgical approaches and patient outcome were retrospectively reviewed according to this classification. This study included 17 patients who underwent 21 operations for resection of 19 cerebellar hemangioblastomas, classified into 911 suboccipital, 4 tentorial, 2 petrosal, and 2 quadrigeminal, and 2 tonsillar. Standard suboccipital craniotomies were utilized for suboccipital hemangioblastomas, the occipital transtentorial approach (OTA) and supracerebellar infratentorial approach for tentorial hemangioblastomas, the retrosigmoid approach for petrosal hemangioblastomas, and OTA for quadrigeminal hemangioblastomas, and midline suboccipital approach for tonsillar hemangioblastomashemangioblastomass. Gross total resection was achieved in all patients except one. Two patients with large hemangioblastomas (tonsillarsuboccipital and quadrigeminal) required second-stage operation which finally achieved gross total removal. No single approach had a significantly higher incidence of postoperative neurological deficits. Selection of the optimum surgical approach for cerebellar hemangioblastomas was successful based on the main drainage systems. Understanding of tumor growth and extension with respect to the venous drainage system is critical to select the appropriate surgical approach.

Pituitary adenomas in elderly patients may become more common as the population ages. Surgical benefits, especially for visual outcome, after endoscopic endonasal transsphenoidal surgery for pituitary adenomas remain to be elucidated. This retrospective analysis investigated clinical factors affecting visual outcome.The study included 35 patients with nonfunctional pituitary adenomas who underwent surgery and were subdivided into elderly (≥70 years old, n = 12) and younger (70 years old, n = 23) groups for analysis. Clinical characteristics and preoperative and postoperative visual function evaluated using visual impairment score (VIS) were compared between groups.Mean age at diagnosis was 75.1 ± 1.5 years in the elderly group and 55.5 ± 2.0 years in the younger group. VIS was improved in 91% of elderly patients and 80% of younger patients, but postoperative VIS remained higher in elderly patients. Preoperative VIS and incidence of previous cataract surgery were significantly higher in elderly patients than in younger patients. Preoperative and postoperative VISs were significantly correlated with age. Preoperative VIS was significantly higher in patients with previous cataract surgery and correlated with postoperative VIS.Visual disturbances were improved postoperatively in most patients in both groups at similar rates, but preoperative and postoperative visual disturbances were more severe in elderly patients because the symptoms may have been masked by the presence of lens opacity. Early diagnosis and intervention may be required in elderly patients for better visual outcome.

Background: Since most pituitary adenomas are benign, there is no model of pituitary adenomas that can be continuously cultured without genetic manipulation. In this study, we analyzed the genetic characteristics of the established cell lines by continuous culture of benign pituitary adenomas without genetic manipulation. Method: The pituitary adenoma was continuously cultured by modifying the conditional reprogramming (CR) method, and the morphology was observed and fluorescent immunostaining (IF) was performed. In addition, genetic characteristics were comprehensively analyzed, differences from the surgical samples were analyzed by enrichment analysis using GSEA, and network analysis was performed using cytoscape based on the protein-protein interaction database. (Results) 1) Of the 14 cases of pituitary adenoma that had undergone primary culture (median MIB-1 labeling index 1.5%), 12 cases were capable of continuous culture for more than 2 months. (6 months or more: 5 cases) 2) After 2 weeks of culturing, the cell morphology changed dramatically. These cultured cells were positive for follicular cell marker s100, ectodermal nervous system markers Nestin, Notch1, and mesodermal marker Vimentin. In these cells, synaptophysin, which was strongly positive in the surgical specimen collected at the same time as the culture, disappeared. 3) Enrichment analysis revealed that gene expression such as epithelial mesenchymal transition(EMT), angiogenesis, IL6 signaling via NFkB, extracellular degradation, integrin cell surface interaction increased in cultured cells, and gene expression such as neurotransmitter including synaptophysin decreased significantly.(Conclusion) 1) The modified CR method allowed continuous culture of pituitary adenoma cells. 2) The cultured cells showed characteristic change of EMT, which was seen in invasive pituitary adenoma.

Although carmustine (Gliadel) wafers improve local tumor control and extend the overall survival in patients with malignant glioma, adverse effects have been documented. The authors report the first case of eosinophilic meningitis triggered by the placement of Gliadel wafers. A 61-year-old man with a history of alimentary allergy and glioblastoma in the right frontal lobe underwent resection followed by the implantation of Gliadel wafers. Three weeks later he suffered the sudden onset of headache, vomiting, and progressive consciousness disturbance. Computed tomography revealed enlargement of the ventricular system and subdural space on the side of the tumor. His CSF leukocyte count increased up to 3990 cells/mm3; 95% of the cells were eosinophilic granulocytes (EGs), suggesting eosinophilic meningitis. Laboratory examination showed the patient to have various elevated allergy indicators. The administration of corticosteroids failed to improve his condition. Despite the insertion of a lumbar drain his symptoms failed to improve. He underwent a second surgical intervention to remove the Gliadel wafers. Histologically, EGs had assembled around the wafers. Eosinophilic infiltrate was present in the brain parenchyma around small vessels. After ventriculoperitoneal shunting his course was favorable. A drug lymphocyte stimulation test against the Gliadel wafers failed to demonstrate a positive reaction; polifeprosan, the wafer matrix without 1,3-bis(2-chloroethyl)-1-nitrosourea, yielded a positive reaction. These findings strongly suggest that although extremely rare, polifeprosan (the wafer matrix) can elicit an allergic reaction. When eosinophilic meningitis is suspected after the implantation of Gliadel wafers, their immediate removal should be considered.

An epidermoid cyst is a benign tumor, accounting for 0.2-1.8% of all brain tumors. Histologically, an epidermoid cyst has a wall consisting of squamous epithelium with keratinization and without skin components. An epidermoid cyst tends to develop in the cerebellopontine angle(CPA), middle cranial fossa, pituitary fossa, spinal cord, and other locations. We describe a rare case of malignant transformation of an epidermoid cyst with cerebrospinal fluid(CSF)dissemination. A 54-year-old man was referred to our hospital with chief complaints of dysesthesia of the right leg, persisting for 6 months, and dizziness and dysarthria for 2 months. Magnetic resonance(MR)imaging showed multiple neoplastic lesions with lobulated margins in the left frontal and temporal lobes, left cerebellum, right brainstem, and right CPA. Diffusion-weighted imaging showed a hyperintense cord-like lesion in the right CPA. Blood examination revealed squamous cell carcinoma(SCC)antigen positivity;however, systemic examination revealed no neoplastic lesion, except for the head lesion. Intracranial lesions were considered unlikely to be metastatic brain tumors. We performed surgical removal of the tumor in the left CPA via a left lateral suboccipital craniotomy. The histopathological diagnosis was SCC. MR imaging for the headache was taken at another hospital 2 years before the surgery, revealed an epidermoid cyst in the right CPA. The current MR imaging showed enhancement in part of the epidermoid cyst in the right CPA, strongly suggesting that the epidermoid cyst in the right CPA had disseminated to the left frontal and temporal lobes, left cerebellum, and right brainstem via the CSF.

A 74-year-old woman presented with central nervous system (CNS) lymphomatoid granulomatosis (LYG) that spontaneously regressed and then regrew shortly thereafter. Initial magnetic resonance imaging studies showed a well demarcated, round, enhanced lesion with perifocal edema in the left temporal lobe. The enhanced lesion and perifocal edema had drastically regressed without treatment at follow-up examination. Two months later, the lesion reappeared and was larger, so was completely removed via left fronto-temporal craniotomy. The histological diagnosis was CNS LYG. CNS LYG should be considered in the differential diagnosis of spontaneously regressing brain tumors.

A 74-year-old woman presented with central nervous system (CNS) lymphomatoid granulomatosis (LYG) that spontaneously regressed and then regrew shortly thereafter. Initial magnetic resonance imaging studies showed a well demarcated, round, enhanced lesion with perifocal edema in the left temporal lobe. The enhanced lesion and perifocal edema had drastically regressed without treatment at follow-up examination. Two months later, the lesion reappeared and was larger, so was completely removed via left fronto-temporal craniotomy. The histological diagnosis was CNS LYG. CNS LYG should be considered in the differential diagnosis of spontaneously regressing brain tumors.

Gangliogliomas are rare tumors of the central nervous system, usually containing neoplastic ganglion cells and astrocytic components. Few cases of ganglioglioma containing only oligodendrocytic tissue have been reported to date. We present a case of a 40-year-old woman with ganglioglioma consisting mostly of oligodendroglial components. Magnetic resonance imaging showed a well-demarcated cystic lesion with slight perifocal edema in the right parietal lobe. The wall of the cyst was not enhanced after administration of Gd-DTPA contrast media. The mass was totally resected. Histological examination showed a mixture of two distinct components: oligodendroglioma and dysplastic ganglions. The first component was diffusely proliferated cells with round nuclei and perinuclear halo; the second showed marked nucleoli and basophilic cytoplasm containing Nissl bodies. Immunohistochemical study of the oligodendroglial component was positive for OLIG 2 and NKX2.2 but negative for synaptophysin. In addition, LOH of 1p/19q was detected by FISH. Although no adjuvant therapy was carried out, follow-up MRI showed no recurrence of the tumor 41 months after the operation.