Seizure control by adding on other anti-seizure medication on seizure during levetiracetam administration in patients with glioma-related epilepsy.

BACKGROUND: Epilepsy is a major symptom in patients with glioma. Levetiracetam (LEV) is recognized as a first-line treatment for glioma-related epilepsy. Increasing the LEV dose is allowed into patients with seizure occurrence against its initial dose. However, the therapeutic efficacy of increasing the LEV dose in response to seizure occurrence remains unclear. METHODS: We retrospectively analyzed 236 glioma patients who were treated with antiseizure medications (ASMs) internally at our institute between September 2010 and December 2017. Of these, the analysis focused on 156 patients treated with LEV who had a clear history of administration. RESULTS: Seizure occurrences were observed in 21 of 75 patients (26.7%) who received LEV as first-line therapy and in 33 of 81 patients (40.7%) who received LEV as non-first-line treatment. The seizure control rate for seizure occurrence with LEV as first-line treatment was significantly higher in patients treated with addition of other ASMs (72.7%) than in those treated with increasing dose of LEV (20.0%) (p = 0.016). The seizure control rate for seizure occurrence with LEV as non-first-line treatment did not differ significantly between patients with addition of other ASMs (58.3%) and those treated with increasing dose of LEV (47.6%) (p = 0.554). CONCLUSIONS: Adding other ASMs was more effective than increasing the LEV dose for seizure control in patients treated with LEV as first-line treatment, but they demonstrated comparable efficacy in patients treated with LEV as non-first-line treatment.

Influence of Tumor Characteristics on Visual Field Outcomes After Pituitary Adenoma Surgery.

BACKGROUND: There were few reports about the influence of tumor characteristics on the postoperative visual field outcomes after transsphenoidal surgery for pituitary adenoma. The purpose of this study was to explore the tumor characteristics that influenced perioperative visual field changes. METHODS: Patients who underwent transsphenoidal surgery under a diagnosis of pituitary adenoma at the Kyoto University Hospital between April 2012 and December 2018 were retrospectively enrolled. Correlations among circumpapillary retinal nerve fiber layer thickness, preoperative and postoperative mean deviation (MD) of visual field, MD change after the surgery, and maximum tumor diameter were evaluated by measuring Pearson correlation coefficient. We evaluated the influences on postoperative MD using a generalized estimating equation for univariate and multivariate regression analyses. We also compared the characteristics of cystic and solid tumors. RESULTS: Thirty-two eyes of 18 patients were included in this study (9 male and 9 female patients). Postoperative MD positively correlated with maximum tumor diameter only in multivariate regression {ß = 0.22 (95% confidence interval [CI], 0.004-0.43), P = 0.046}, although maximum tumor diameter negatively correlated with postoperative MD in univariate regression (ß = -0.16 [95% CI, -0.58 to 0.26], P = 0.46). In the investigation of perioperative MD changes, eyes with cystic tumors showed significantly better improvement those with solid tumors (8.93 ± 7.85 vs 0.18 ± 6.56 dB, P = 0.007). CONCLUSIONS: Cystic and solid tumors show different characteristics of visual loss and visual field defects. The MD in eyes with cystic tumors improved significantly more than that in eyes with solid tumors.

Detecting immunoglobulin G4-related intracranial arteriopathy with magnetic resonance vessel wall imaging: a preliminary experience in two cases.

BACKGROUND: Detecting immunoglobulin G4 (IgG4)-related intracranial arteriopathy, a rare neurovascular complication of IgG4-related disease, is challenging. While magnetic resonance (MR) vessel wall imaging (VWI) can visualize various neurovascular pathologies, its application to this arteriopathy has not been reported as of this writing. CASE PRESENTATION: A 74-year-old male and a 65-year-old female manifested multiple cranial nerve palsy and neck pain, respectively. Both cases exhibited multiorgan masses with markedly elevated serum IgG4 levels and were clinically diagnosed with IgG4-related disease. Three-dimensional T1-weighted black blood VWI with and without contrast agent identified intracranial vascular lesions characterized as nearly-circumferential mural thickening with homogeneous contrast enhancement in the internal carotid and vertebral arteries; some of the lesions had been unrecognized with screening MR angiography due to expansive remodeling. The former patient underwent corticosteroid therapy, and VWI after treatment revealed decreased mural thickening and enhancement. CONCLUSION: Further studies to elucidate characteristic findings of VWI might contribute to early detection of this treatable pathology.

Infrequent RAS mutation is not associated with specific histological phenotype in gliomas.

BACKGROUND: Mutations in driver genes such as IDH and BRAF have been identified in gliomas. Meanwhile, dysregulations in the p53, RB1, and MAPK and/or PI3K pathways are involved in the molecular pathogenesis of glioblastoma. RAS family genes activate MAPK through activation of RAF and PI3K to promote cell proliferation. RAS mutations are a well-known driver of mutation in many types of cancers, but knowledge of their significance for glioma is insufficient. The purpose of this study was to reveal the frequency and the clinical phenotype of RAS mutant in gliomas. METHODS: This study analysed RAS mutations and their clinical significance in 242 gliomas that were stored as unfixed or cryopreserved specimens removed at Kyoto University and Osaka National Hospital between May 2006 and October 2017. The hot spots mutation of IDH1/2, H3F3A, HIST1H3B, and TERT promoter and exon 2 and exon 3 of KRAS, HRAS, and NRAS were analysed with Sanger sequencing method, and 1p/19q codeletion was analysed with multiplex ligation-dependent probe amplification. DNA methylation array was performed in some RAS mutant tumours to improve accuracy of diagnosis. RESULTS: RAS mutations were identified in four gliomas with three KRAS mutations and one NRAS mutation in one anaplastic oligodendroglioma, two anaplastic astrocytomas (IDH wild-type in each), and one ganglioglioma. RAS-mutant gliomas were identified with various types of glioma histology. CONCLUSION: RAS mutation appears infrequent, and it is not associated with any specific histological phenotype of glioma.

Preoperative obliteration of choroidal arteries in the treatment of large hypervascular tumors in the lateral ventricle.

BACKGROUND: Removal of large hypervascular tumors in the lateral ventricle still poses a surgical challenge. These tumors are usually fed from choroidal arteries, and vascular control is typically performed late during the removal. We aimed to evaluate the clinical efficacy of our strategy for persistent preoperative obliteration of feeders from the choroidal arteries to manage large hypervascular tumors in the lateral ventricle. METHODS: We retrospectively analyzed six patients with hypervascular tumors in the lateral ventricle. We first attempted to obstruct feeders using endovascular treatment, and, if unavailable, performed initial microsurgical occlusion through the temporal horn for the staged tumor removal. RESULTS: In all patients, feeder obliteration was successfully performed; the anterior choroidal arteries were occluded by the endovascular treatment and microsurgical occlusion in one and five patients, respectively, while the lateral posterior choroidal arteries were occluded via endovascular treatment in four patients. No patients had permanent symptoms due to feeder obliteration, and tumor devascularization was achieved at the mean rate of 69.9%. During the tumor removal, the mean blood loss volume was 253 ml. No postoperative hemorrhage had occurred, and all patients scored ≤ 2 on the modified Rankin Scale at six months post-removal. CONCLUSIONS: Although further studies are warranted, persistent feeder obliteration of choroidal arteries could be an effective treatment strategy against large hypervascular tumors in the lateral ventricle.

Outpatient Cardiac Rehabilitation Suppresses Deterioration of Renal Function in Patients ≥75 Years of Age With Heart Disease.

BACKGROUND: This study investigated the effect of outpatient cardiac rehabilitation (OCR) and physical activity on the estimated glomerular filtration rate based on serum cystatin C (eGFRcys) in patients with heart disease (HD) aged ≥75 years.Methods and Results:This non-randomized prospective intervention study involved 136 patients (non-OCR group, n=66; OCR group, n=70), 55 of whom were aged ≥75 years (non-OCR group, n=29; OCR group, n=26). Renal function (eGFRcys) was evaluated at discharge and 3 months thereafter. A linear mixed model (LMM) was used to assess changes in renal function over time. The hospital readmission rate within 3 months after discharge was also evaluated. LMM analysis showed that the change in eGFRcys was -2.27 and +0.48 mL/min/1.73 m2in the non-OCR and OCR groups, respectively (F=2.960, P=0.022). Further, among patients aged ≥75 years in the non-OCR and OCR groups, the change in eGFRcys was -3.83 and -1.08 mL/min/1.73 m2, respectively (F=2.719, P=0.039). The proportion of patients aged ≥75 years who were rehospitalized due to exacerbation of HD was 16.9% (n=10) and 6.7% (n=2) in the non-OCR and OCR groups, respectively. CONCLUSIONS: Among patients with HD aged ≥75 years, participation in OCR reduces the decline in renal function and hospital readmission rates.

Evaluation of image quality of pituitary dynamic contrast-enhanced MRI using time-resolved angiography with interleaved stochastic trajectories (TWIST) and iterative reconstruction TWIST (IT-TWIST).

BACKGROUND: Time-resolved angiography with interleaved stochastic trajectories (TWIST) is a keyhole imaging with frequent sampling of central k-space data and view-sharing for the peripheral k-space of dynamic phases. IT-TWIST is a technique to reconstruct images with a smaller temporal footprint using the same raw data obtained with TWIST by iterative reconstruction. PURPOSE: To compare image quality between TWIST and IT-TWIST in 3D pituitary DCE-MRI. STUDY TYPE: Retrospective observation study. POPULATION: Fifty-one patients (23 men, 28 women) who underwent 3D pituitary DCE-MRI using TWIST between July 2016 and April 2017. FIELD STRENGTH/SEQUENCE: 3T/TWIST and IT-TWIST. ASSESSMENT: Visual evaluation was conducted for image quality of delineation of the pituitary stalk and posterior lobe during the early arterial phase, cerebral white matter near the sella turcica, and the mass lesion. Bolus sharpness of the pituitary stalk, posterior lobe, and bilateral cavernous sinus was evaluated on the enhancement slope map calculated from TWIST and IT-TWIST. Temporal stability of intensity of the nonenhanced area was evaluated on temporal standard deviation (SD) maps calculated from TWIST and IT-TWIST. STATISTICAL TESTS: Paired t-test or Wilcoxon rank-sum test was used to test the differences between TWIST and IT-TWIST in both visual evaluation and region of interest evaluation. RESULTS: Scores of visual evaluations for IT-TWIST were significantly better than those for TWIST (P < 0.001) in all items. Enhancement slope for IT-TWIST was significantly higher than that for TWIST in posterior lobe, and right and left cavernous sinus (P < 0.001). Temporal SD for IT-TWIST was significantly lower than that for TWIST in all items, with statistical significance (P < 0.001). DATA CONCLUSION: IT-TWIST yielded better visualization, and better enhancement slope, and less temporal SD compared with TWIST in 3D pituitary DCE-MRI. Evidence Level: 4 Technical Efficacy: Stage 1 J. Magn. Reson. Imaging 2020;51:1497-1506.

Impact of collagen matrix on reconstructive material selection and postoperative complications in endoscopic endonasal skull base surgery.

OBJECTIVE: The aim of this study was to investigate the impact of collagen matrix on reconstructive material selection and postoperative complications in endoscopic endonasal skull base surgery. METHODS: The authors retrospectively reviewed the data of consecutive patients who underwent purely endoscopic endonasal skull base surgery from January 2015 to March 2023. Intraoperative CSF leakage was classified according to the Esposito grade, and skull base repair was tailored to the leakage grade. The patients were divided into two groups: before (group A) and after (group B) collagen matrix implementation. The rates of autologous graft harvesting (fat, fascia, and nasoseptal flap), postoperative CSF leakage, and donor-site complications were compared between the two groups. RESULTS: In total, 270 patients were included. Group A included 159 patients and group B included 111 patients. There were no differences in patient characteristics, including age, pathology, and Esposito grade, between the two groups. The overall fat usage rate was significantly higher in group A (63.5%) than in group B (39.6%) (p = 0.0001), and the fascia usage rate was also significantly higher in group A (25.8%) than in group B (4.5%) (p < 0.0001). The nasoseptal flap usage rate did not differ between group A (32.7%) and group B (30.6%) (p = 0.79). Postoperative CSF leakage was similar between the two groups (0.63% in group A vs 1.8% in group B, p = 0.57), and the overall rate of CSF leakage was 1.1%. Donor-site complications occurred in 3 patients in group A, including 1 abdominal hematoma, 1 delayed abdominal infection, and 1 fluid collection after fascia lata harvesting. CONCLUSIONS: Collagen matrix implementation significantly decreased autologous graft harvesting without increasing postoperative CSF leakage, contributing to less invasive surgery.

Thin-slice reverse encoding distortion correction DWI facilitates visualization of non-functioning pituitary neuroendocrine tumor (PitNET)/pituitary adenoma and surrounding normal structures.

BACKGROUND: To evaluate the clinical usefulness of thin-slice echo-planar imaging (EPI)-based diffusion-weighted imaging (DWI) with an on-console distortion correction technique, termed reverse encoding distortion correction DWI (RDC-DWI), in patients with non-functioning pituitary neuroendocrine tumor (PitNET)/pituitary adenoma. METHODS: Patients with non-functioning PitNET/pituitary adenoma who underwent 3-T RDC-DWI between December 2021 and September 2022 were retrospectively enrolled. Image quality was compared among RDC-DWI, DWI with correction for distortion induced by B0 inhomogeneity alone (B0-corrected-DWI), and original EPI-based DWI with anterior-posterior phase-encoding direction (AP-DWI). Susceptibility artifact, anatomical visualization of cranial nerves, overall tumor visualization, and visualization of cavernous sinus invasion were assessed qualitatively. Quantitative assessment of geometric distortion was performed by evaluation of anterior and posterior displacement between each DWI and the corresponding three-dimensional T2-weighted imaging. Signal-to-noise ratio (SNR), contrast-to-noise ratio (CNR), and apparent diffusion coefficient values were measured. RESULTS: Sixty-four patients (age 70.8 ± 9.9 years [mean ± standard deviation]; 33 females) with non-functioning PitNET/pituitary adenoma were evaluated. In terms of susceptibility artifacts in the frontal and temporal lobes, visualization of left trigeminal nerve, overall tumor visualization, and anterior displacement, RDC-DWI performed the best and B0-corrected-DWI performed better than AP-DWI. The right oculomotor and right trigeminal nerves were better visualized by RDC-DWI than by B0-corrected-DWI and AP-DWI. Visualization of cavernous sinus invasion and posterior displacement were better by RDC-DWI and B0-corrected-DWI than by AP-DWI. SNR and CNR were the highest for RDC-DWI. CONCLUSIONS: RDC-DWI achieved excellent image quality regarding susceptibility artifact, geometric distortion, and tumor visualization in patients with non-functioning PitNET/pituitary adenoma. RELEVANCE STATEMENT: RDC-DWI facilitates excellent visualization of the pituitary region and surrounding normal structures, and its on-console distortion correction technique is convenient. RDC-DWI can clearly depict cavernous sinus invasion of PitNET/pituitary adenoma even without contrast medium. KEY POINTS: • RDC-DWI is an EPI-based DWI technique with a novel on-console distortion correction technique. • RDC-DWI corrects distortion due to B0 field inhomogeneity and eddy current. • We evaluated the usefulness of thin-slice RDC-DWI in non-functioning PitNET/pituitary adenoma. • RDC-DWI exhibited excellent visualization in the pituitary region and surrounding structures. • In addition, the on-console distortion correction of RDC-DWI is clinically convenient.

High intratumoral susceptibility signal grade on susceptibility-weighted imaging: a risk factor for hemorrhage after stereotactic biopsy.

OBJECTIVE: This study aimed to examine the association of preoperative intratumoral susceptibility signal (ITSS) grade with hemorrhage after stereotactic biopsy (STB). METHODS: The authors retrospectively reviewed 66 patients who underwent STB in their institution. Preoperative factors including age, sex, platelet count, prothrombin time-international normalized ratio, activated thromboplastin time, antiplatelet agent use, history of diabetes mellitus and hypertension, target location, anesthesia type, and ITSS data were recorded. ITSS was defined as a dot-like or fine linear low signal within a tumor on susceptibility-weighted imaging (SWI) and was graded using a 3-point scale: grade 1, no ITSS within the lesion; grade 2, 1-10 ITSSs; and grade 3, ≥ 11 ITSSs. Postoperative final tumor pathology was also reviewed. The association between preoperative variables and the size of postoperative hemorrhage was examined. RESULTS: Thirty-four patients were men and 32 were women. The mean age was 66.6 years. The most common tumor location was the frontal lobe (27.3%, n = 18). The diagnostic yield of STB was 93.9%. The most common pathology was lymphoma (36.4%, n = 24). The ITSS was grade 1 in 37 patients (56.1%), grade 2 in 14 patients (21.2%), and grade 3 in 15 patients (22.7%). Interobserver agreement for ITSS was almost perfect (weighted kappa = 0.87; 95% CI 0.77-0.98). Age was significantly associated with ITSS (p = 0.0075). Postoperative hemorrhage occurred in 17 patients (25.8%). Maximum hemorrhage diameter (mean ± SD) was 1.78 ± 1.35 mm in grade 1 lesions, 2.98 ± 2.2 mm in grade 2 lesions, and 9.51 ± 2.11 mm in grade 3 lesions (p = 0.01). Hemorrhage > 10 mm in diameter occurred in 10 patients (15.2%), being symptomatic in 3 of them. Four of 6 patients with grade 3 ITSS glioblastomas (66.7%) had postoperative hemorrhages > 10 mm in diameter. After adjusting for age, ITSS grade was the only factor significantly associated with hemorrhage > 10 mm (p = 0.029). Compared with patients with grade 1 ITSS, the odds of postoperative hemorrhage > 10 mm in diameter were 2.57 times higher in patients with grade 2 ITSS (95% CI 0.31-21.1) and 9.73 times higher in patients with grade 3 ITSS (95% CI 1.57-60.5). CONCLUSIONS: ITSS grade on SWI is associated with size of postoperative hemorrhage after STB.

Long-term survival outcomes and recurrence patterns of olfactory neuroblastoma: A 13-year experience at a single institution.

OBJECTIVES: Olfactory neuroblastoma (ONB), also known as esthesioneuroblastoma, is a rare malignant neoplasm of the nasal vault and anterior skull base. The results of treatment for ONB are relatively good; however, regional and distant metastases can develop several years after definitive treatment. This study aimed to validate the treatment modality of ONB for oncological outcomes, especially for regional recurrence. METHODS: We retrospectively reviewed the medical records of 22 patients diagnosed with ONB at Kyoto University Hospital between 2009 and 2020. Descriptive statistics were calculated, and Kaplan-Meier curves were used. RESULTS: The median follow-up time was 58.2 months. One (4.5%) patient was clinically node positive, (cN+) and the remaining 21 (95.5%) were clinically node negative (cN0) at presentation. Eighteen patients underwent an endoscopic endonasal approach (EEA) for primary resection, and the remaining four patients underwent a combined EEA and transcranial approach. Elective neck dissection was not performed for 21 patients with cN0 ONB, whereas unilateral neck dissection with removal of ipsilateral lateral retropharyngeal node was performed for one patient with cN+ ONB. Postoperative radiotherapy without concurrent chemotherapy was performed only at the primary tumor bed for 21 patients with cN0 ONB, and at the primary tumor bed and bilateral neck for one patient with cN+ ONB. The 5-year overall, disease-specific, and disease-free survival rates were 94.1%, 100%, and 69.6%, respectively. No patients developed local recurrence, but 6 (27.2%) patients experienced recurrence with a median time to recurrence of 36.4 months, including four and two patients who initially developed regional recurrences and bone metastases, respectively. Five (22.7%) patients had delayed neck recurrence. The salvage rate was only 60.0% in the five patients who had delayed neck recurrence. Regarding the level of delayed neck recurrence, 4 (18.2%) patients had lateral retropharyngeal lymph node metastases. CONCLUSION: Patients with ONB have excellent survival outcomes after endoscopic surgical resection of the primary lesion with postoperative radiotherapy only to the primary tumor bed. Despite excellent survival, delayed neck recurrence, including the lateral retropharyngeal lymph node, remains high. Because salvage surgery for lateral retropharyngeal lymph node recurrence is sometimes technically difficult, it may be better to extend the field of postoperative radiotherapy from the primary tumor bed only to include bilateral lateral retropharyngeal lymph node regions in patients with clinically N0 ONB. Further prospective studies with a large number of patients are needed to determine the extent of postoperative radiotherapy.

Evaluation of isocitrate dehydrogenase mutation in 2021 world health organization classification grade 3 and 4 glioma adult-type diffuse gliomas with 18F-fluoromisonidazole PET.

PURPOSE: This study aimed to investigate the uptake characteristics of 18F-fluoromisonidazole (FMISO), in mutant-type isocitrate dehydrogenase (IDH-mutant, grade 3 and 4) and wild-type IDH (IDH-wildtype, grade 4) 2021 WHO classification adult-type diffuse gliomas. MATERIALS AND METHODS: Patients with grade 3 and 4 adult-type diffuse gliomas (n = 35) were included in this prospective study. After registering 18F-FMISO PET and MR images, standardized uptake value (SUV) and apparent diffusion coefficient (ADC) were evaluated in hyperintense areas on fluid-attenuated inversion recovery (FLAIR) imaging (HIA), and in contrast-enhanced tumors (CET) by manually placing 3D volumes of interest. Relative SUVmax (rSUVmax) and SUVmean (rSUVmean), 10th percentile of ADC (ADC10pct), mean ADC (ADCmean) were measured in HIA and CET, respectively. RESULTS: rSUVmean in HIA and rSUVmean in CET were significantly higher in IDH-wildtype than in IDH-mutant (P = 0.0496 and 0.03, respectively). The combination of FMISO rSUVmean in HIA and ADC10pct in CET, that of rSUVmax and ADC10pct in CET, that of rSUVmean in HIA and ADCmean in CET, were able to differentiate IDH-mutant from IDH-wildtype (AUC 0.80). When confined to astrocytic tumors except for oligodendroglioma, rSUVmax, rSUVmean in HIA and rSUVmean in CET were higher for IDH-wildtype than for IDH-mutant, but not significantly (P = 0.23, 0.13 and 0.14, respectively). The combination of FMISO rSUVmean in HIA and ADC10pct in CET was able to differentiate IDH-mutant (AUC 0.81). CONCLUSION: PET using 18F-FMISO and ADC might provide a valuable tool for differentiating between IDH mutation status of 2021 WHO classification grade 3 and 4 adult-type diffuse gliomas.

Multicenter Study of Surgical Ablation for Atrial Fibrillation in Aortic Valve Replacement.

There is controversy regarding appropriate surgical ablation procedures concomitant with nonmitral valve surgery. We retrospectively investigated the impact of surgical ablation for atrial fibrillation during aortic valve replacement between 2010 and 2015 in 16 institutions registered through the Japanese Society for Arrhythmia Surgery. Clinical data of 171 patients with paroxysmal and nonparoxysmal atrial fibrillation undergoing aortic valve replacement were collected and classified into full maze operation (n = 79), pulmonary vein isolation (PVI) (n = 56), and no surgical ablation (n = 36) groups. All patients were followed up and electrocardiograms were recorded in 68% at 2 years. The myocardial ischemia time was significantly longer in the maze group than the others during isolated aortic valve replacement (p ≤ 0.01), but there were no significant differences in 30-day or 2-year mortality rates between groups. The ratios of sinus rhythm at 2 years in paroxysmal and nonparoxysmal atrial fibrillation in the maze group versus PVI group were 87% versus 97%, respectively (p = 0.24) and 53% versus 42%, respectively (p = 0.47). No patients with nonparoxysmal atrial fibrillation in the no surgical ablation group maintained sinus rhythm at 2 years. In conclusion, both maze and PVI during aortic valve replacement are valuable strategies to restore sinus rhythm at 2 years and result in favorable early and midterm survival rates.

Central nervous system mature teratoma producing carbohydrate antigen 19-9: illustrative case.

BACKGROUND: Central nervous system (CNS) mature teratoma is a rare disease with symptoms that can vary according to tumor location. Most lesions are benign; rarely, malignancy can develop in any of the somatic components. Elevated levels of tumor markers such as α-fetoprotein and ß-human chorionic gonadotropin are not usually found in patients with CNS mature teratoma, and no reports have described an association with carbohydrate antigen 19-9 (CA19-9). OBSERVATIONS: A 64-year-old woman with headache was found to have a mass lesion in the anterior cranial fossa. Magnetic resonance imaging of the brain suggested a mature teratoma. Serum and cerebrospinal fluid (CSF) tests showed significant CA19-9 elevations (2,770 U/mL and 4,387 U/mL, respectively). Other examinations, including whole-body 18F-fluorodeoxyglucose positron emission tomography, did not detect the origin of elevated CA19-9, suggesting that the high CA19-9 levels were caused by intracranial tumor. The patient underwent tumor removal. The histopathological diagnosis was mature teratoma with positive CA19-9 staining. CA19-9 levels in serum and CSF decreased significantly after tumor removal. LESSONS: The histopathological findings and postoperative decreased CA19-9 levels established the diagnosis of CA19-9-producing CNS mature teratoma. CNS mature teratoma can cause elevations in CA19-9 in cases with absence of neoplasms in the trunk.

Malignant transformation of central neurocytoma with dissemination 17 years after initial treatment: illustrative case.

BACKGROUND: Central neurocytomas usually have a favorable clinical course, and gross total resection (GTR) results in long-term survival. Recurrences of central neurocytomas are usually local, and dissemination is extremely rare. OBSERVATIONS: A 24-year-old man who presented with vomiting was found to have a mass in the right lateral ventricle. After GTR, he received whole-brain irradiation and chemotherapy and had remained disease-free on follow-up for years. The review of the initial tumor revealed central neurocytoma. Seventeen years later, he presented with deterioration of memory, and magnetic resonance imaging showed an enhanced lesion in the left hippocampus. The enhanced lesion was resected, and the histological examination revealed that the tumor was a disseminated atypical central neurocytoma with frequent mitoses. Although he was treated with chemotherapy, the disseminated tumor slowly grew and invaded the brain. Massive brain invasion occurred without enhanced lesions, and he died 27 months after the tumor recurrence. LESSONS: In this patient, a central neurocytoma disseminated after an extremely long period of time. Once neurocytomas disseminate and show aggressive behavior, patients usually follow a poor course. Patients with central neurocytomas should be followed up for a long time.

Whole-genome sequencing analysis of an atypical teratoid/rhabdoid tumor in a patient with Phelan-McDermid syndrome: a case report and systematic review.

Atypical teratoid/rhabdoid tumor (AT/RT) is a rare pediatric brain tumor with abnormalities in SMARCB1 located in 22q11.2. We report a case of AT/RT associated with Phelan-McDermid syndrome (PMS) characterized by congenital developmental disorder, mental retardation, and ring chromosome 22 with 22q13.3-qter depletion, for which we performed whole-genome sequencing (WGS). A 4-year-old girl with a developmental disability was referred to our hospital due to dysphoria. Brain magnetic resonance imaging showed a 5-cm well-demarcated mass that extended bilaterally in the frontal lobes. G-banding was performed preoperatively due to a history of developmental retardation. Ring chromosome 22 and deletion of 22q13.3-qter were observed, and she was diagnosed with PMS. She underwent gross total resection of the tumor, and the pathological diagnosis was AT/RT. WGS showed somatic SMARCB1 mutation (p.R201X) and somatic loss of the entire chromosome 22 in the tumor, but not in the blood sample. WGS confirmed previously unreported BRCA2 mutations, 6q loss, and 14q acquisition during tumor progression, but no other significant findings associated with tumor progression. The present case is discussed with reference to a systematic review of previous reports of AT/RT associated with PMS. PMS patients with ring chromosome 22 should be carefully followed up for AT/RT occurrence.

Intraoperative Cerebrospinal Fluid Leak Graded by Esposito Grade Is a Predictor for Diabetes Insipidus After Endoscopic Endonasal Pituitary Adenoma Resection.

BACKGROUND: Diabetes insipidus (DI) is a well-known complication of transsphenoidal surgery. However, the risk factors for DI remain controversial. METHODS: We conducted a retrospective study of patients who underwent endoscopic transsphenoidal surgery for pituitary adenoma at our institution during a 5-year period. The patients were divided into a DI group and a non-DI group. Logistic regression analyses were used to identify risk factors for postoperative DI. In subgroup analysis, the DI group was divided into transient DI and permanent DI groups, and perioperative factors were compared between groups. RESULTS: Of 101 patients, 58 were in the non-DI group (57.4%) and 43 were in the DI group (42.6%). Permanent DI occurred in 7 patients (6.9%). In univariate analyses, statistically significant risk factors were suprasellar extension, tumor functionality, and intraoperative cerebrospinal fluid leaks by Esposito grade. In multivariate logistic regression analysis, Esposito grade was the only statistically significant risk factor (P = 0.015). The frequency of DI increased as the Esposito grade increased (P = 0.0002 for the trend). In subgroup analysis, postoperative nadir sodium concentration was lower in the permanent DI group (128.1 ± 2.78 mmol/L) than in the transient DI group (135 ± 1.22 mmol/L; P = 0.035), and the optimal cutoff value was 124.5 mmol/L, with a sensitivity of 57.1% and a specificity of 91.7% (area under the curve = 0.76, P = 0.034). CONCLUSIONS: Intraoperative cerebrospinal fluid leak by Esposito grade is associated with postoperative DI. These data can be applied to help identify high-risk patients who need more aggressive follow-up and fluid management.

The association of ectopic craniopharyngioma in the fourth ventricle with familial adenomatous polyposis: illustrative case.

BACKGROUND: Craniopharyngioma (CP) often arises in the sellar and suprasellar areas; ectopic CP in the posterior fossa is rare. Familial adenomatous polyposis (FAP) is a genetic disorder involving the formation of numerous adenomatous polyps in the gastrointestinal tract, and it is associated with other extraintestinal manifestations. OBSERVATIONS: The authors reported the case of a 63-year-old woman with FAP who presented with headache and harbored a growing mass in the fourth ventricle. Magnetic resonance imaging (MRI) findings revealed a well-circumscribed mass with high intensity on T1-weighted images and low intensity on T2-weighted images and exhibited no contrast enhancement. Gross total resection was performed and histopathology revealed an adamantinomatous CP (aCP). The authors also reviewed the previous reports of ectopic CP in the posterior fossa and found a high percentage of FAP cases among the ectopic CP group, thus suggesting a possible association between the two diseases. LESSONS: An ectopic CP may be reasonably included in the differential diagnosis in patients with FAP who present with well-circumscribed tumors in the posterior fossa.

Papillary glioneuronal tumor growing slowly for 26 years: illustrative case.

BACKGROUND: Papillary glioneuronal tumors (PGNTs) are classified as a type of World Health Organization grade I mixed neuronal-glial tumor. Most PGNTs involve cystic formations with mural nodules and solid components in the cerebral hemispheres, and PGNTs occur mainly in young adults. The long-term prognosis of PGNTs remains unclear. OBSERVATIONS: A 38-year-old male had been diagnosed with an arachnoid cyst associated with epilepsy in a local hospital. The initial magnetic resonance imaging (MRI) study showed the tumor as a heterogeneously enhanced nodule in the left postcentral gyrus. Subsequent MRI studies showed slow growth of the tumor for 26 years. He underwent gross total resection to control his epilepsy. The histopathological findings revealed pseudopapillary structures involving hyalinized blood vessels with a single or pseudostratified layer of cuboidal glial cells with round nuclei and scant cytoplasm. At the periphery of the lesion, Rosenthal fibers and acidophilic granule bodies were observed in the gliotic brain tissue. Immunohistochemically, some interpapillary cells were positive for NeuN. On the basis of these findings, the tumor was diagnosed as a PGNT. LESSONS: This PGNT showed slow growth for 26 years. When recognizing a slowly growing tumor in the cerebral hemispheres of relatively young people that is associated with epileptic seizures, PGNT should be considered as a differential diagnosis.