The Prevalence of the Middle Clinoid Process: A Cross-Sectional Comparative Study in Patients with and without Pathology of the Sella Turcica.

Background The middle clinoid process (MCP), particularly caroticoclinoid ring (CCR) type of the MCP, is an important part of the sphenoid bone for skull base surgery. Previous studies have shown a wide range of MCP prevalence affected by various factors. However, no study has investigated the association between the MCP and the presence of sellar lesions. Objectives The main aim of this study was to evaluate the prevalence of the MCP in the Thai population and factors associated with its presence. Materials and Methods We conducted a cross-sectional study on 400 sides from 200 patients (100 with and 100 without sellar lesions) using cranial computerized tomography scans. Demographic data and MCP characteristics were collected. The association between individual variables and the presence of the MCP was determined by univariate and multivariate analysis. Results The MCP was identified in 168 of 400 sides (42%). Patients with sellar lesions had a significantly lower prevalence of the MCP compared with normal controls (29.5% versus 54.5%, p < 0.001). Of all MCP only 6% were the CCR type. Univariate and multivariate analysis showed that the absence of the sellar lesion was the only factor significantly associated with presence of the MCP (odds ratio: 2.86; 95% confidence interval: 1.90-4.32; p < 0.001). Conclusion The prevalence of the MCP was relatively high in the Thai population, while the prevalence of the CCR was relatively low compared with previous studies. The absence of sellar lesions was the only factor associated with the presence of the MCP.

Predictors for the Differentiation between Glioblastoma, Primary Central Nervous System Lymphoma, and Metastasis in Patients with a Solitary Enhancing Intracranial Mass.

Introduction Differentiation between glioblastoma (GBM), primary central nervous system lymphoma (PCNSL), and metastasis is important in decision-making before surgery. However, these malignant brain tumors have overlapping features. This study aimed to identify predictors differentiating between GBM, PCNSL, and metastasis. Materials and Methods Patients with a solitary intracranial enhancing tumor and a histopathological diagnosis of GBM, PCNSL, or metastasis were investigated. All patients with intracranial lymphoma had PCNSL without extracranial involvement. Demographic, clinical, and radiographic data were analyzed to determine their associations with the tumor types. Results The predictors associated with GBM were functional impairment ( p = 0.001), large tumor size ( p < 0.001), irregular tumor margin ( p < 0.001), heterogeneous contrast enhancement ( p < 0.001), central necrosis ( p < 0.001), intratumoral hemorrhage ( p = 0.018), abnormal flow void ( p < 0.001), and hypodensity component on noncontrast cranial computed tomography (CT) scan ( p < 0.001). The predictors associated with PCNSL comprised functional impairment ( p = 0.005), deep-seated tumor location ( p = 0.006), homogeneous contrast enhancement ( p < 0.001), absence of cystic appearance ( p = 0.008), presence of hypointensity component on precontrast cranial T1-weighted magnetic resonance imaging (MRI; p = 0.027), and presence of isodensity component on noncontrast cranial CT ( p < 0.008). Finally, the predictors for metastasis were an infratentorial ( p < 0.001) or extra-axial tumor location ( p = 0.035), smooth tumor margin ( p < 0.001), and presence of isointensity component on cranial fluid-attenuated inversion recovery MRI ( p = 0.047). Conclusion These predictors may be used to differentiate between GBM, PCNSL, and metastasis, and they are useful in clinical management.

Hydrocephalus following Brain Tumor Surgery: Factors Correlating with Occurrence of Postoperative Hydrocephalus and Predictive Scoring Model.

Hydrocephalus following brain tumor surgery is found, although cause of hydrocephalus is optimally eradicated. This study aimed to investigate factors associated with development of postoperative hydrocephalus that requires shunt procedure and generate predictive scoring model of this condition. Demographic, clinical, radiographic, treatment, laboratory, complication, and postoperative data were collected. Binary logistic regression was used to investigate final model for generating predictive scoring system of postoperative hydrocephalus. A total of 179 patients undergoing brain tumor surgery were included. Forty-five (25.1%) patients had postoperative hydrocephalus that required shunt surgery. In univariate analysis, several factors were found to be associated with postoperative hydrocephalus. Strong predictors of postoperative hydrocephalus revealed in multivariate analysis included tumor recurrence before surgery (odds ratio [OR], 4.38; 95% confidence interval [CI], 1.28-14.98; p = 0.018), preoperative hydrocephalus (OR, 6.52; 95% CI, 2.44-17.46; p < 0.001), glial tumor (OR, 3.76; 95% CI, 1.14-12.43; p = 0.030), metastasis (OR, 5.19; 95% CI, 1.72-15.69; p = 0.004), intraventricular hemorrhage (OR, 7.08; 95% CI, 1.80-27.82; p = 0.005), and residual tumor volume (OR, 1.05; 95% CI, 1.01-1.09; p = 0.007). A cutoff predictive score with the best area under curve and optimum cutoff point was utilized for discriminating patients with high risk from individuals with low risk in occurrence of postoperative hydrocephalus. This study reported predictive factors strongly associated with development of postoperative hydrocephalus. Predictive scoring system is useful for identifying patients with an increased risk of postoperative hydrocephalus. Patients classified in the high-risk group require closed surveillance of the hydrocephalus.

Germline ATM mutation and somatic PIK3CA and BCOR mutations found in an infant with aggressive orbital embryonal rhabdomyosarcoma.

PURPOSE: To report a case of aggressive infantile orbital embryonal rhabdomyosarcoma harboring germline ATM mutation and 2 somatic mutations as revealed by next-generation sequencing and the potential application for personalized therapy. OBSERVATIONS: A 7-month-old male developed a rapidly progressive left proptosis over 6 weeks due to a large medial orbital mass. Biopsy revealed embryonal rhabdomyosarcoma. After the first cycle of chemotherapy, re-imaging showed interval tumor enlargement with intracranial extension. Craniotomy, combined with orbital exenteration, was performed. Tumor specimens and blood samples were sent for 596 gene DNA sequencing panels with RNA-sequencing focused on actionable mutations as well as gene fusion detection. Sequencing revealed 3 clinically relevant mutations: a germline ATM loss-of-function (LOF) mutation, a somatic PIK3CA gain-of-function mutation, and a somatic BCOR LOF mutation. No chromosomal translocation was detected. Workup for metastasis was positive for bone marrow involvement. Despite standard high-dose adjuvant chemotherapy in combination with radiation therapy, the patient died 10 months later with metastatic diseases. CONCLUSIONS AND IMPORTANCE: This case highlights an aggressive form of embryonal rhabdomyosarcoma in an infantile orbit. The presence of germline mutation may explain the increased chemo-resistance and adverse prognosis, and may be used as the target for genomic-directed therapy.