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Abstract BACKGROUND Endoscopic endonasal approaches pose the potential risk of olfactory loss. Loss of olfaction and potentially taste can be permanent and greatly affect patients’ quality of life. Treatments for olfactory loss have had limited success. Omega-3 supplementation may be a therapeutic option with its effect on wound healing and nerve regeneration. OBJECTIVE To evaluate the impact on olfaction in patients treated with omega-3 supplementation following endoscopic skull base tumor resection. METHODS In this multi-institutional, prospective, randomized controlled trial, 110 patients with sellar or parasellar tumors undergoing endoscopic resection were randomized to nasal saline irrigations or nasal saline irrigations plus omega-3 supplementation. The University of Pennsylvania Smell Identification Test (UPSIT) was administered preoperatively and at 6 wk, 3 mo, and 6 mo postoperatively. RESULTS Eighty-seven patients completed all 6 mo of follow-up (41 control arm, 46 omega-3 arm). At 6 wk postoperatively, 25% of patients in both groups experienced a clinically significant loss in olfaction. At 3 and 6 mo, patients receiving omega-3 demonstrated significantly less persistent olfactory loss compared to patients without supplementation (P = .02 and P = .01, respectively). After controlling for multiple confounding variables, omega-3 supplementation was found to be protective against olfactory loss (odds ratio [OR] 0.05, 95% CI 0.003-0.81, P = .03). Tumor functionality was a significant independent predictor for olfactory loss (OR 32.7, 95% CI 1.15-929.5, P = .04). CONCLUSION Omega-3 supplementation appears to be protective for the olfactory system during the healing period in patients who undergo endoscopic resection of sellar and parasellar masses.

Purpose A subset of skull base meningiomas (SBM) may show early progression/recurrence (P/R) as a result of incomplete resection. The purpose of this study is the implementation of MR radiomics to predict P/R in SBM. Methods From October 2006 to December 2017, 60 patients diagnosed with pathologically confirmed SBM (WHO grade I, 56; grade II, 3; grade III, 1) were included in this study. Preoperative MRI including T2WI, diffusion-weighted imaging (DWI), and contrast-enhanced T1WI were analyzed. On each imaging modality, 13 histogram parameters and 20 textural gray level co-occurrence matrix (GLCM) features were extracted. Random forest algorithms were utilized to evaluate the importance of these parameters, and the most significant three parameters were selected to build a decision tree for prediction of P/R in SBM. Furthermore, ADC values obtained from manually placed ROI in tumor were also used to predict P/R in SBM for comparison. Results Gross-total resection (Simpson Grades I–III) was performed in 33 (33/60, 55%) patients, and 27 patients received subtotal resection. Twenty-one patients had P/R (21/60, 35%) after a postoperative follow-up period of at least 12 months. The three most significant parameters included in the final radiomics model were T1 max probability, T1 cluster shade, and ADC correlation. In the radiomics model, the accuracy for prediction of P/R was 90%; by comparison, the accuracy was 83% using ADC values measured from manually placed tumor ROI. Conclusions The results show that the radiomics approach in preoperative MRI offer objective and valuable clinical information for treatment planning in SBM.

A 53 year old female presented with a six-year history of right-sided slow deterioration in hearing and a feeling of pressure in the right ear. The patient had not experienced any pain but reported some paresthesia of the right half of the tongue, whereas no further other cranial nerve deficits were evident. The otoscopy was unremarkable as well as the rest of the clinical ENT examination except for a slight asymptomatic swelling of the right cheek. Imaging findings showed an expansive tumor infiltrating and destroying the right lateral skull base. The tumor was partially composed of cystic/regressive lesions with high contrast media uptake. The tumor had high-signal intensity with water-sensitive sequences (T2w) and was hypointense on T1w images. We performed a tumor resection via a transparotideal-infratemporal approach. Histologically, the tumor was composed of granular variably calcified chondroid matrix with extensive regressive changes and granulation-like tissue reaction associated with calcinosis and crystal deposition. Molecular analysis of the tumor via the TruSight- RNA-Fusion panel detected a fusion involving FN1::FGFR2, consistent with “calcified chondroid mesenchymal neoplasm” (CCMN), a rare tumor entity recently defined by Liu et al 2021. In regular follow-up care no residual tumor has been detected in imaging studies (MRI and CT) within 2 years and 4 months. The biology and consequently the radio sensitivity cannot be defined precisely since long term results are missing due to the first description of this entity in 2021. As a consequence, surgical resection is recommended as the treatment of choice. Thorough clinical and radiological follow-up is mandatory as local recurrences are to be expected due to the infiltrative behavior. In case of a loco regional recurrence the fusion with FGFR2 may represent a therapeutic option for a targeted therapy on molecular level.

Background Subacute oculomotor dysfunction encompasses a broad differential diagnosis, including autoimmune, infectious, vascular, and neoplastic etiologies. Primary skull base diffuse large B‐cell lymphoma (DLBCL) is a rare but treatable cause of cranial nerve dysfunction and may present subtly on conventional neuroimaging. Methods We report an 80‐year‐old man with subacute diplopia, dysfunction of multiple cranial nerves, left‐sided ptosis, and headache. Diagnostic workup included cranial imaging, cerebrospinal fluid (CSF) analysis with cytology and flow cytometry, neurophysiological studies, serum diagnostics, and dedicated MRI of the skull base. Transsphenoidal biopsy was performed following identification of an infiltrative lesion. Results Initial investigations including cranial CT, CSF analysis, and serum diagnostics were unremarkable. Dedicated skull base MRI revealed a diffuse infiltrating mass involving the clivus, petrous bone, occipital condyles, and perisellar/posterior pituitary region, with anatomical correlation to multiple cranial nerves. Mildly elevated prolactin levels suggested a stalk effect. Histological analysis confirmed DLBCL (NOS, GCB type). Treatment with corticosteroids followed by dose‐adjusted R‐CHOP resulted in complete clinical remission and radiologically confirmed tumor regression. Conclusions This case illustrates a rare presentation of primary bony skull base DLBCL with perisellar infiltration, highlighting the diagnostic challenges of complex oculomotor dysfunction. Dedicated skull base MRI is essential in the workup of unexplained cranial neuropathy. CSF‐based molecular markers may complement the diagnostic approach in similar presentations.

Skull base chondrosarcoma is a rare tumour usually treated by surgery and proton therapy. However, as mortality rate is very low and treatment complications are frequent, a less aggressive therapeutic strategy could be considered. The objective of this study was to compare the results of surgery only vs surgery and adjuvant proton therapy, in terms of survival and treatment adverse effects, based on a retrospective series. Monocentric retrospective study at a tertiary care centre. All patients treated for a skull base grade I and II chondrosarcoma were included. We collected data concerning surgical and proton therapy treatment and up-to-date follow-up, including Common Terminology Criteria for Adverse Events (CTCAE) scores. 47 patients (23M/24F) were operated on between 2002 and 2015; mean age at diagnosis was 47 years-old (10-85). Petroclival and anterior skull base locations were found in 34 and 13 patients, respectively. Gross total resection was achieved in 17 cases (36%) and partial in 30 (64%). Adjuvant proton therapy (mean total dose 70 GyRBE,1.8 GyRBE/day) was administered in 23 cases. Overall mean follow-up was 91 months (7-182). Of the patients treated by surgery only, 8 (34%) experienced residual tumour progression (mean delay 51 months) and 5 received second-line proton therapy. Adjuvant proton therapy was associated with a significantly lower rate of relapse (11%; p = 0.01). There was no significant difference in 10-year disease specific survival between patients initially treated with or without adjuvant proton therapy (100% vs 89.8%, p = 0.14). Difference in high-grade toxicity was not statistically significant between patients in both groups (25% (7) vs 11% (5), p = 0.10). The most frequent adverse effect of proton therapy was sensorineural hearing loss (39%). Long-term disease specific survival was not significantly lower in patients without adjuvant proton therapy, but they experienced less adverse effects. We believe a surgery only strategy could be discussed, delaying as much as possible proton therapy in cases of relapse. Further prospective studies are needed to validate this more conservative strategy in skull base chondrosarcoma.

Background Skull base chordoma is a rare and aggressive bone tumor with a poor prognosis. The basement membrane (BM) plays an pivotal role in tumor progression. However, the involvement of BM-related genes in assessing the prognosis and influencing the biological behavior of skull base chordomas remains unclear. Methods Patients with skull base chordoma undergoing endoscopic endonasal surgery were included in the study (77 patients for bulk transcriptome sequencing and 6 patients for single-cell RNA sequencing). A BM-related genes signature was established and validated using bulk transcriptome data. Additionally, we investigated the oncogenic potential of a key BM-related gene in chordoma cells in vitro. Results A prognostic signature consisting of five BM-related genes was identified through LASSO Cox regression analysis. The accuracy and reliability of this signature were validated by the validation cohort. Multivariate Cox analysis and a nomogram demonstrated that the risk score serves as an independent and reliable prognostic factor for skull base chordoma. Moreover, the BM-related gene signature was significantly associated with the immune microenvironment, immune checkpoint expression, and drug sensitivity. Single-cell RNA sequencing analysis revealed both the chordoma tumor cell and the fibroblast contributed to the overall BM signature. Finally, in vitro experiments demonstrated that the knockdown of ITGB3, the hub gene in the signature, inhibited the proliferation and migration of chordoma cells via the PI3K-Akt pathway. Conclusion This study explored the critical role of BM-related genes in skull base chordoma, which affected postoperative recurrence and maligant behavior of chordoma via the PI3K-Akt signaling pathway.

Background Precise delineation of gross tumor volume (GTV) is fundamental for effective radiation therapy in low-grade skull base meningiomas. Magnetic resonance imaging (MRI) serves as the primary imaging tool but may not fully represent tumor extent. This study investigates the additional value of incorporating Somatostatin receptor (SSTR)-directed PET/CT in radiation therapy planning. Methods A retrospective analysis was conducted with four experienced radiation oncologists contouring GTVs for skull base meningiomas using MRI alone (GTV_MRI), PET/CT alone (GTV_PET/CT), and both modalities combined (GTV_ALL). Consensus ground truth volumes were generated for each modality through a STAPLE algorithm. Agreement between modalities, excluding observer variability, was assessed using statistical metrics including Dice Similarity Coefficient (DSC), Jaccard Index (JCI), Hausdorff distance (HD95), Geographical Miss Index (GMI), sensitivity, and kappa statistics. Results The study included 25 patients (15 females, 10 males; median age 56 years (range: 23–74 years), with 96% achieving local control post-radiotherapy over a median follow-up of 64 months (range: 28–135 months). Substantial interobserver agreement was observed, with median kappa values of 0.74 for GTV_MRI, 0.68 for GTV_PET/CT, and 0.77 for GTV_ALL. Median consensus volumes were 6.65 cc (MRI STAPLE ), 7.21 cc (PET STAPLE ), and 6.73 cc (ALL STAPLE ). The median GMI for MRI STAPLE compared to ALL STAPLE was 0.18 (IQR: 0.11–0.39), and 0.21 (IQR: 0.15–0.28) for PET STAPLE compared to ALL STAPLE . The DSC indicated the lowest concordance between MRI STAPLE and PET STAPLE with a median of 0.75 (IQR: 0.59–0.82), followed by PET STAPLE versus ALL STAPLE with a median DSC of 0.84 (IQR: 0.79–0.89), and MRI STAPLE versus ALL STAPLE with a median DSC of 0.89 (IQR: 0.76–0.92). The integration of PET/CT with MRI significantly enhanced concordance metrics. Conclusion Combining MRI and PET/CT improves GTV delineation in low-grade skull base meningiomas, as PET/CT can reveal regions missed by MRI, which may slightly underestimate tumor size. This multimodal imaging approach enhances consensus and supports its role in radiotherapy planning. Standardized protocols and technical integration remain key future goals.

IDH-mutant astrocytomas (AIDHmut) in the posterior cranial fossa (PCF) are rare and present substantial diagnostic and therapeutic challenges due to their location. We analyzed patients with PCF AIDHmut from our institutions, treated between December 2021 and September 2024. Additionally, we conducted a systematic literature review (from January 2021 to September 2024) using PubMed, Ovid MEDLINE, and Ovid EMBASE to identify cases of PCF AIDHmut. We identified a total of 19 cases, including one institutional case. Most patients were young adults, with a male predominance (15 males, 4 females). Tumors primarily originated from the brainstem (94.7%), with only one case involving the cerebellum. Clinical presentations frequently included cranial nerve deficits, with diplopia being the most common symptom (47.4%). Adjuvant radiotherapy (IMRT, DT 54 Gy/27 fractions, 78.9%) and chemotherapy (temozolomide, 68.4%) formed the mainstays of treatment. Tumor grading revealed 63.2% (12/19) were WHO grade 2, 21% (4/19) were WHO grade 3, and 15.8% (3/19) were grade 4. The mean follow-up period was 45 months. PCF AIDHmut are rare but pose significant treatment challenges due to their location and infiltrative nature. Multimodal treatment—comprising surgery, radiotherapy, and chemotherapy—is essential for achieving long-term disease control. Subtotal resection followed by adjuvant therapies provides a favorable balance between tumor control and functional preservation.

Objective This is a case report of a dermoid cyst located in the infratemporal fossa and its surgical removal using infratemporal fossa type B approach. Case report A 15-year-old male was referred from a local clinic after an incidental finding of a mass lesion in the skull base area on computed tomography (CT). Pre-operative magnetic resonance imaging showed a large cystic mass lesion, expanding to the foramen ovale with fat component in the right infratemporal fossa region. The lesion was completely excised using an infratemporal fossa type B approach. Conclusion An extremely rare case of dermoid cysts of the infratemporal fossa was managed with infratemporal fossa type B approach without severe complication.

Purpose In patients with subtotal resection (STR) of WHO grade I skull base meningiomas, treatment strategies of adjuvant radiation versus observation with salvage radiation, if necessary, were compared using progression-free survival (PFS) and radiation failure-free survival (RFFS). Methods Patients with newly diagnosed WHO grade I skull base meningioma who underwent radiographically confirmed STR between 1995 and 2021 were included. PFS was measured from last treatment date. RFFS was measured from surgery date to first radiation failure. Multivariable Cox regression, adjusted for propensity score (PS) and inverse probability treatment weighted (IPTW), was performed. Results Of 179 patients, 25 (14.0%) received adjuvant radiation. Among 154 observed patients, 90 (58.4%) experienced tumor progression and 64 (71.1%) received salvage radiation. Observation after STR had PFS at 3, 5, and 10 years of 60.6%, 47.5%, and 26.8%, respectively. Adjuvant radiation had PFS/RFFS at 3, 5, and 10 years of 84.2%, 77.2%, and 77.2%. Salvage radiation had PFS at 3, 5, and 10 years of 96.0%, 85.0%, and 80.0%. RFFS after observation with salvage radiation, if needed, at 3, 5, and 10 years was 100%, 97.7%, and 92.8%. PS and IPTW Cox regression models, controlling for residual tumor volume, demonstrated that observation with salvage radiation significantly prolonged RFFS (HR = 0.06, p  = 0.013; HR 0.08, p  = 0.026, respectively) compared to adjuvant radiation. Median follow-up was 77.5 months. Conclusion Most patients will have tumor progression within 10 years of STR. Our data suggests that appropriately selected patients can be observed with close follow-up, reserving radiation for progression.