Superior orbital fissure narrowing and tumor-associated pain in spheno-orbital meningiomas.

INTRODUCTION: Spheno-orbital meningiomas (SOMs) represent a distinct subtype of meningioma characterized by their unique multi-compartmental invasion pattern. Previous studies have investigated correlations between SOMs and visual manifestations. However, our comprehension of pain associated with SOMs remains limited. This study aims to provide insight into the pathophysiology underlying SOM-related pain through measurements of tumor volume and superior orbital fissure (SOF) narrowing. METHODS: This retrospective study included patients who underwent surgical resection of a SOM between 2000 and 2022. Preoperative CT and/or MRI scans were analyzed, and the tumor volume of each segment was measured. Bony 3D reconstructions were used to measure the area of the SOF, and SOF narrowing was calculated. RESULTS: The study cohort included 66 patients diagnosed with SOMs, among which 25.8% (n = 17) presented with pain. Postoperatively, 14/17 (82.4%) of patients reported pain improvement. There was no significant correlation between the total volume or the volume of tumor within each compartment and the presence of pain on presentation (p > 0.05). The median SOF narrowing was significantly different between patients presenting with and without tumor-associated pain with median of 11 mm2 (IQR 2.8-22.3) and 2 mm2 (IQR 0-6), respectively (p = 0.005). Using logistic regression, a significant correlation between the degree of SOF narrowing and the presence of SOM-associated pain on presentation was identified, with an aOR of 1.2 (95% CI 1.12-1.3, p = 0.02). CONCLUSION: While the exact cause of tumor-associated pain remains unclear, SOF narrowing seems to play a role in pain among SOM patients. Based on the radiological characteristics, SOF neurovascular decompression is recommended in SOM patients.

Volumetric Analysis of Spheno-Orbital Meningiomas: Prognostic Correlation and a Compartmentalized Approach.

BACKGROUND AND OBJECTIVES: Spheno-orbital meningiomas arise from the arachnoid villi cap cells at the sphenoid ridge and have the ability to spread through soft tissue extension and cranial bone invasion. Owing to their orbital hyperostosis and intraorbital soft tissue extension, they commonly present with ophthalmologic manifestations. This study aims to investigate the correlation between tumor volume with the presenting symptoms and postoperative outcomes. METHODS: This retrospective study analyzed patients who underwent surgical resection of spheno-orbital meningiomas. Tumor volumes in different compartments were measured using preoperative and postoperative imaging. Linear and logistic regression analyses were used to identify correlations between tumor volumes and presenting symptoms preoperatively and postoperative outcomes. RESULTS: Sixty-six patients were included in this study, of whom 86.4% had proptosis, 80.3% had decreased visual acuity (VA), 30.3% had visual field defects, and 13.6% had periorbital edema. Preoperatively, proptosis linearly correlated with intraosseous tumor volume (coefficient = 0.6, P < .001), while the decrease in baseline VA correlated with the intraorbital tumor volume (coefficient = 0.3, P = .01). The odds of periorbital edema were found to increase with an increase in intraosseous tumor volume with an adjusted odds ratio of 1.4 (95% CI, 1.1-1.7, P = .003), while the odds of visual field defects were found to increase with an increase in intraorbital tumor volume with an adjusted odds ratio of 2.7 (95% CI, 1.3-5.6, P = .01). Postoperatively, the volume of intraosseous tumor resected linearly correlated with the improvement in proptosis (coefficient = 0.7, P < .001), while the volume of intraorbital tumor resected linearly correlated with improvement in VA (coefficient = 0.5, P < .001) and with a larger effect size in patients presenting with moderate-to-severe decrease in VA preoperatively (coefficient = 0.8). CONCLUSION: Underscoring the importance of each tumor compartment relative to the patient's symptomatology serves as a valuable guide in implementing a compartmentalized resection approach tailored to the surgical objectives.

Sellar atypical teratoid/rhabdoid tumor in adults: survival analysis of treatment strategies. Illustrative case.

BACKGROUND: Atypical teratoid/rhabdoid tumor (ATRT) is a rare, highly aggressive central nervous system tumor predominantly found in children. Limited information exists on ATRT in adults, posing challenges in diagnosis and treatment. This study presents the case of an adult patient with ATRT in the sellar region and explores the impact of different treatment regimens on patient survival. OBSERVATIONS: A 60-year-old female with an ATRT underwent resection of the tumor, followed by adjuvant chemoradiotherapy. Molecular genetic analysis revealed compound heterozygous SMARCB1 point mutations. Survival analysis was performed on previously published adult ATRT cases, comparing treatment approaches. The cohort's overall median survival was 6 months, with patients receiving combined chemoradiotherapy showing the longest median survival of 23.5 months. Statistical analysis demonstrated a significant difference in survival between patients treated with surgery alone and those receiving surgery followed by chemoradiotherapy (p < 0.001). However, no significant difference was observed between patients treated with surgery alone and those with postoperative radiotherapy (p = 0.105). LESSONS: Early initiation of adjuvant chemoradiotherapy following surgery improves survival outcomes in adult patients with ATRT. Because of limited data on standardized treatment protocols for adults with ATRT, further research and larger-scale studies are needed to establish effective treatment guidelines for this population.

Therapeutic response to pazopanib: case report and literature review on molecular abnormalities of aggressive prolactinomas.

Introduction: Aggressive prolactinomas (APRLs) pose a significant clinical challenge due to their high rate of regrowth and potentially life-threatening complications. In this study, we present a case of a patient with an APRL who had a trial of multiple therapeutic modalities with the aim to provide a review of molecular abnormalities and management of APRLs by corroborating our experience with previous literature. Methods: A total of 268 articles were reviewed and 46 were included. Case reports and series, and studies that investigated the molecular and/or genetic analysis of APRLs were included. Special care was taken to include studies describing prolactinomas that would fall under the APRL subtype according to the European Society of Endocrinology guidelines; however, the author did not label the tumor as "aggressive" or "atypical". Addiontionally, we present a case report of a 56-year-old man presented with an invasive APRL that was resistant to multiple treatment modalities. Results: Literature review revealed multiple molecular abnormalities of APRLs including mutations in and/or deregulation of ADAMTS6, MMP-9, PITX1, VEGF, POU6F2, CDKN2A, and Rb genes. Mismatch repair genes, downregulation of microRNAs, and hypermethylation of specific genes including RASSF1A, p27, and MGMT were found to be directly associated with the aggressiveness of prolactinomas. APRL receptor analysis showed that low levels of estrogen receptor (ER) and an increase in somatostatin receptors (SSTR5) and epidermal growth factor receptors (EGFR) were associated with increased invasiveness and higher proliferation activity. Our patient had positive immunohistochemistry staining for PD-L1, MSH2, and MSH6, while microarray analysis revealed mutations in the CDKN2A and POU6F2 genes. Despite undergoing two surgical resections, radiotherapy, and taking dopamine agonists, the tumor continued to progress. The patient was administered pazopanib, which resulted in a positive response and the patient remained progression-free for six months. However, subsequent observations revealed tumor progression. The patient was started on PD-L1 inhibitor pembrolizumab, yet the tumor continued to progress. Conclusion: APRLs are complex tumors that require a multidisciplinary management approach. Knowledge of the molecular underpinnings of these tumors is critical for understanding their pathogenesis and identifying potential targets for precision medical therapy.

Symptomatic intracranial tumors in pregnancy: an updated management algorithm. Illustrative case.

BACKGROUND: Intracranial tumors are infrequently encountered during pregnancy, and their diagnosis and management require a multidisciplinary approach to ensure the best possible outcomes for the mother and fetus. The pathophysiology and manifestations of these tumors are influenced by hormonal changes, hemodynamic modifications, and alterations in immunological tolerance that occur during pregnancy. Despite the complexity of this condition, no standardized guidelines exist. This study aims to highlight the key points of this presentation, along with the discussion of a possible management algorithm. OBSERVATIONS: The authors report the case of a 35-year-old woman who presented during the third trimester of pregnancy with severe signs of increased intracranial pressure (ICP) due to a posterior cranial fossa mass. The decision was made to stabilize the patient by placing an external ventricular drain to temporize her increased ICPs until the baby could be safely delivered via cesarean section. The mass was resected via suboccipital craniectomy 1 week postpartum. LESSONS: In considering treatment modalities and their timing in patients presenting with intracranial tumors during pregnancy, each patient should be managed on the basis of an individual treatment algorithm. Symptoms, prognosis, and gestational age should be taken into account to optimize the surgical and perioperative outcomes of both the mother and fetus.

Thermal Analysis of an Ultrasonic Aspirator Micro Claw Tool Compared With Standard High-Speed Drilling During Internal Auditory Canal Opening in a Cadaveric Model.

BACKGROUND: The ultrasonic aspirator micro claw tool (UAmCT) can be used to remove the bone of the internal auditory canal (IAC) during vestibular schwannoma resection via the retrosigmoid approach (RSA) without the risk of a spinning drill shaft. However, the thermal profile of the UAmCT during IAC removal has not been reported. OBJECTIVE: To compare the thermal profile of the UAmCT during access of the IAC to that of a conventional high-speed drill (HSD) and to present an illustrative case of this application. METHODS: IAC opening via RSA was performed in 5 embalmed cadaveric specimens using the UAmCT with 3, 8, and 15 mL/min irrigation on the left and the HSD at 75 000 revolutions per minute and 0%, 14%, and 22% irrigation on the right. Peak bone surface temperatures were measured 4 times in 20-second intervals, and statistical analyses were performed using SPSS software. An illustrative case of a vestibular schwannoma resected via an RSA using the UAmCT to access the IAC is presented. RESULTS: The IAC was opened in all 5 specimens using both the UAmCT and HSD without complication. The mean peak bone surface temperatures were significantly lower with the UAmCT compared with the HSD ( P < .001). The UAmCT did not meaningfully prolong the operating time in the illustrative case, and the IAC was accessed without complication. CONCLUSION: The UAmCT may be a safe and effective alternative to HSD for IAC opening during vestibular schwannoma resection via the RSA. Larger studies under clinical conditions are required to further validate these findings.

Radial Forearm Free Flap with Parapharyngeal Pedicle Corridor for Reconstruction of Anterior Skull Base Defects.

BACKGROUND: The radial forearm free flap (RFFF) is an indispensable tool for the reconstruction of a range of defects, including those of the skull base. Several options for routing of the RFFF pedicle have been described, and the parapharyngeal corridor (PC) has been proposed as an option and employed for a nasopharyngeal defect. However, there are no reports of its use for the reconstruction of anterior skull base defects. The objective of this study is to describe the technique for free tissue reconstruction of anterior skull base defects using the RFFF and routing of the pedicle via the PC. METHODS: Relevant neurovascular landmarks and critical surgical steps for reconstruction of anterior skull base defects with an RFFF and routing of the pedicle via the PC are reported using an illustrative clinical case and cadaveric dissections. RESULTS: We present a case of a 70-year-old man who underwent endoscopic transcribriform resection of cT4N0 sinonasal squamous cell carcinoma and was left with a large anterior skull base defect that persisted despite several repair attempts. An RFFF was utilized to repair the defect. This report is the first description of the clinical use of the PC for free tissue repair of an anterior skull base defect. CONCLUSIONS: The PC is an option for routing the pedicle during the reconstruction of anterior skull base defects. When the corridor is prepared as described in this case, a direct route exists from the anterior skull base to cervical vessels, simultaneously maximizing the reach of the pedicle and minimizing the risk of kinking.

A Cost-Effective and Reproducible Cadaveric Training Model for Internal Carotid Artery Injury Management During Endoscopic Endonasal Surgery: The Submersible Peristaltic Pump.

BACKGROUND: Internal carotid artery injury (ICAI) represents one of the most challenging complications in endoscopic endonasal neurosurgery and its rarity results in limited opportunities for trainees and surgeons to achieve proficiency in its management. Currently, available models for ICAI have employed costly systems that prevent their widespread use. The objective of this study is to validate an affordable submersible peristaltic pump (SPP)-based model as a reproducible and realistic paradigm for ICAI management training. METHODS: A laceration of the left parasellar internal carotid artery was purposely carried out in 2 human cadaveric heads. A blood substitute was perfused to ensure a perfusion flow of 1 L/min using an affordable SPP. A cohort of 20 neurosurgery and otolaryngology residents, fellows, and attendings were enrolled to evaluate the realism and content validity of the model using a validated 5-grade questionnaire. RESULTS: The model proved to mimic a real intraoperative scenario of ICAI with an expected output flow of 1 L/min. Questionnaire responses reported a realistic experience and the impact of this model on improving trainee surgical coordination and capability to rehearse the most accepted repair technique. The use of a fixed noninjected head allowed the reproducibility of the training session without the additional cost of new fresh-frozen heads. The affordable SPP allowed an impactful reduction of ICAI model training expenses maintaining high realism. CONCLUSIONS: The SPP-based ICAI model with noninjected cadaveric specimens is an affordable and cost-effective system that allows reproducibility and realism. These qualities favor greater adoption in neurosurgery and otolaryngology training curricula.