OBJECTIVE: Due to the proximity of craniopharyngioma to the optic apparatus, one of the most common complications after surgery is visual deterioration. Intraoperative visual evoked potential (VEP), as a means of real-time visual function monitoring, has been integrated into transsphenoidal surgery for pituitary adenoma to predict postoperative visual outcome. Compared with pituitary tumor, craniopharyngioma often adheres to optic nerves, with increased risk of postoperative visual impairment. Furthermore, extended endoscopic endonasal surgery (EEES) can provide direct visualization of the surgical plane between the craniopharyngioma and the optic nerves, which contributes to analysis of the mechanism of real-time VEP changes during surgery. Therefore, VEP monitoring applied during EEES for craniopharyngioma may have more clinical value. However, only 9 patients who underwent EEES with VEP monitoring for craniopharyngioma have been sporadically reported to date. In this paper, the authors present the largest series to date analyzing the clinical value of VEP to predict postoperative visual outcome in adult patients with craniopharyngioma. METHODS: Sixty-five adult patients who underwent EEES with intraoperative VEP monitoring for primary craniopharyngioma were retrospectively reviewed. The association between changes in VEP amplitude and postoperative visual outcome was determined. In addition, other potential prognostic factors with regard to postoperative visual outcomes were included in the analysis. RESULTS: Gross-total resection was achieved in 59 patients (90.8%). Reproducible and stable VEP was recorded in 128 of 130 eyes (98.5%). During surgery, VEP remained stable in 108 eyes, 10 (9.3%) of which had new visual acuity (VA) and/or visual field (VF) defects after surgery. Transient VEP decrease was recorded in 15 eyes, 4 (26.7%) of which had visual deterioration. Of the 5 eyes with permanent VEP decrease, 3 (60%) experienced postoperative visual impairment. Permanent VEP decrease (OR 19.868, p = 0.007) and tight adhesion (OR 6.104, p = 0.040) were independent adverse factors for postoperative VA deterioration. Tight adhesion (OR 7.150, p = 0.002) and larger tumor volume (OR 1.066, p = 0.001) were significant risk factors for postoperative VF defects. CONCLUSIONS: Intraoperative VEP monitoring can serve as a real-time warning to guide surgeons to avoid postoperative visual impairment. It effectively predicted VA changes in adult patients with craniopharyngioma after EEES. Tight adhesion and larger tumor volume were also strong predictors of postoperative visual impairment.

Purpose: Chordoma is a rare bone tumor with a high recurrence rate and limited treatment options. The aim of this study was to identify molecular subtypes of chordoma that may improve clinical management. Experimental Design: We conducted RNA sequencing in 48 tumors from patients with Chinese skull-base chordoma and identified two major molecular subtypes. We then replicated the classification using a NanoString panel in 48 patients with chordoma from North America. Results: Tumors in one subtype were more likely to have somatic mutations and reduced expression in chromatin remodeling genes, such as PBRM1 and SETD2, whereas the other subtype was characterized by the upregulation of genes in epithelial–mesenchymal transition and Sonic Hedgehog pathways. IHC staining of top differentially expressed genes between the two subtypes in 312 patients with Chinese chordoma with long-term follow-up data showed that the expression of some markers such as PTCH1 was significantly associated with survival outcomes. Conclusions: Our findings may improve the understanding of subtype-specific tumorigenesis of chordoma and inform clinical prognostication and targeted options.

OBJECTIVE: In this study, the authors’ aim was to research clinical features and prognostic factors in patients harboring clival chordomas and explore the relationship between platelet-derived growth factor receptor-β (PDGFR-β) expression and tumor invasion and prognosis of clival chordoma.METHODS: A total of 242 patients were retrospectively analyzed. Clinical information, including extent of resection, Al-Mefty classification, postoperative complications, and postoperative radiotherapy, was reviewed. Kaplan-Meier analysis was used to estimate survival time. Immunohistochemical analysis, quantitative reverse transcription polymerase chain reaction, and Western blotting were used to measure the expression level of proteins or mRNA. Transwell assaying was performed to measure the invasive ability of the tumor cells.RESULTS: According to the Al-Mefty classification, there were 37, 112, and 93 type I, II, and III tumors, respectively. Gross-total resection (GTR) was achieved in 86 cases (35.5%), subtotal resection (STR) in 63 cases (26.0%), and partial resection (PR) in 93 cases (38.4%). The 5-year progression-free survival (PFS) and overall survival (OS) rates in the GTR group were significantly higher than those in the non–total resection (NTR; i.e., STR and PR) group (p < 0.001). The 5-year PFS and OS rates for patients with type I tumors were significantly higher than those for patients harboring types II and III tumors (p < 0.001). In the NTR group, the median PFS and OS of patients with lower PDGFR-β expression were significantly longer than those of patients with higher PDGFR-β expression. Reduction of PDGFR-β suppressed the invasion ability of cells in vitro. In addition, reduction of PDGFR-β can obviously downregulate the expression levels of mammalian target of rapamycin (mTOR) or phospho-mTOR.CONCLUSIONS: Extent of resection, Al-Mefty classification, primary tumor, postoperative radiotherapy, and PDGFR-β expression level are valuable prognostic factors in patients with clival chordomas. PDGFR-β could regulate invasion through the mTOR pathway in clival chordoma cells.

Purpose: Craniopharyngiomas (CPs) are benign tumors, complete tumor resection is considered to be the optimal treatment. However, although histologically benign, the local invasiveness of CPs commonly contributes to incomplete resection and a poor prognosis. At present, some advocate less aggressive surgery combined with radiotherapy as a more reasonable and effective means of protecting hypothalamus function and preventing recurrence in patients with tight tumor adhesion to the hypothalamus. Hence, if a method can be developed to predict the invasiveness of CP preoperatively, it will help in the development of a more personalized surgical strategy. The aim of the study was to report a radiomics-clinical nomogram for the individualized preoperative prediction of the invasiveness of adamantinomatous CP (ACPs) before surgery. Methods: In total, 1,874 radiomics features were extracted from whole tumors on contrast-enhanced T1-weighted images. A support vector machine trained a predictive model that was validated using receiver operating characteristic (ROC) analysis on an independent test set. Moreover, a nomogram was constructed incorporating clinical characteristics and the radiomics signature for individual prediction. Results: Eleven features associated with the invasiveness of ACPs were selected by using the least absolute shrinkage and selection operator (LASSO) method. These features yielded area under the curve (AUC) values of 79.09 and 73.5% for the training and test sets, respectively. The nomogram incorporating peritumoral edema and the radiomics signature yielded good calibration in the training and test sets with the AUCs of 84.79 and 76.48%, respectively. Conclusion: The developed model yields good performance, indicating that the invasiveness of APCs can be predicted using noninvasive radiological data. This reliable, noninvasive tool can help clinical decision making and improve patient prognosis.