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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.

PurposeNon-vestibular schwannomas (NVSs) of the skull base occur in several sites, and few previous studies have evaluated the usefulness of the endoscopic endonasal transmaxillary-pterygoid approach (EETMPA) to resect these lesions. We aimed to evaluate the characteristics and clinical outcomes of patients who underwent EETMPA for skull-base NVSs and to investigate the efficacy, safety, and indications for the procedure.MethodsWe retrospectively reviewed the clinical data of 10 consecutive patients (mean age, 45 ± 17) who underwent EETMPA for skull-base NVSs at the University of Tsukuba hospital between 2013 and 2020. We also calculated the total tumor volume and the size of the corridor to the tumor for EEA (SCEEA) in nine patients who underwent EEA for NVSs adjacent to the Meckel’s cave or cavernous sinus.ResultsNine patients (9/10), including five women (5/10), underwent primary surgery. Gross total resection and subtotal resection were achieved in five patients each (5/10). Postoperatively, one patient showed a new and mild cranial nerve V sensory deficit and one patient showed slight worsening of abducens nerve palsy. The greater palatine nerve was amputated in two patients; however, permanent perception loss in the soft palate was observed in one patient. The Vidian nerve was sacrificed in four patients, and new dry eye occurred in one patient. None of the patients experienced postoperative tumor recurrence or regrowth during the follow-up period of 40 ± 28 months.ConclusionEETMPA is safe and effective for excising skull-base NVSs which are not eligible for radiosurgery leading to a high rate of successful resection and a high rate of but mild neurological sequela. The EEA is appropriate when the tumor extends to the paranasal sinus with sufficient SCEEA.

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.

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.

PurposeThis study defines the specific areas that connect the surgical corridors of the endoscopic endonasal (EEA) and transorbital approach (TOA) to identify adequate clinical applications and perspectives of this combined multiportal approach.MethodsConsecutive patients who underwent combined EEA and TOA procedures for various pathologies involving multiple compartments of the skull base were enrolled.ResultsA total of eight patients (2 chondrosarcomas, 2 meningiomas, 2 schwannomas, 1 glioma, and 1 traumatic optic neuropathy) were included between August 2016 and April 2021. The cavernous sinus (CS) was targeted as the connection area of the combined approach in four patients with tumors infiltrating the middle cranial fossa (MCF) and central skull base through the CS. For two patients with MCF tumors extending into the infratemporal fossa (ITF), the horizontal portion of the greater sphenoid wing and the foramen ovale were utilized as the connection area. In the remaining 2 patients, connection was achieved through the optic canal (OC). Gross total and near total resection was achieved in 5 patients with tumors, and circumferential removal of bone composing the OC was performed in one patient with traumatic compressive optic neuropathy. Postoperative complications included one cardiac arrest due to underlying cardiovascular disease and one case of oculomotor nerve palsy.ConclusionsThe combined EEA and TOA procedure is a useful strategy for complex lesions involving multiple compartments of the skull base. Herein, we identified the specific areas connecting the two surgical approaches, allowing a common path for EEA and TOA procedures.

Abstract Chordomas are rare tumors of the axial skeleton that are refractory to conventional therapy. Few studies have compared the morphological and molecular characteristics of chordomas according to the skull base and sacral locations. Histopathological data and changes revealed by array comparative genomic hybridization (CGH) and next-generation sequencing (NGS) of cell cycle regulation genes were analyzed for 28 skull base (SBCs) and 15 sacral (SC) chordomas. All cases were conventional chordomas. SBCs were significantly more frequent in patients aged <40 years and SCs predominated in patients aged >60 years. Mitotic indices ≥2 mitoses/10 high-power fields were correlated with high degrees of nuclear atypia and Ki67 labeling indices ≥6%. We identified 321 genomic positions, and copy number variation losses were more frequent than gain. Moreover, we report a panel of 85 genetic variants of cell cycle genes and the presence of molecular clusters for chordoma as well in CGH as in NGS. These new data strengthen the view that the chordoma should not be considered as a single molecular entity.

Abstract There are a myriad of head and neck pathologies that extend from the extracranial to the intracranial compartment, traversing the skull base, and knowledge of the imaging appearance of this pathology is critical to practicing neurosurgeons. This article reviews some of the important inflammatory or acquired head and neck pathology along the skull base, neoplastic skull base lesions, and the intracranial extension of head and neck malignancy. Focus will be on the relevant anatomy, appropriate imaging protocols to evaluate these processes, as well as the differentiating imaging findings on computed tomography and magnetic resonance imaging.

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.