Multicenter Registry of Adenomas of the Pituitary and Related Disorders: Initial Description of Cushing Disease Cohort, Surgical Outcomes, and Surgeon Characteristics.

BACKGROUND AND OBJECTIVES: To address the lack of a multicenter pituitary surgery research consortium in the United States, we established the Registry of Adenomas of the Pituitary and Related Disorders (RAPID). The goals of RAPID are to examine surgical outcomes, improve patient care, disseminate best practices, and facilitate multicenter surgery research at scale. Our initial focus is Cushing disease (CD). This study aims to describe the current RAPID patient cohort, explore surgical outcomes, and lay the foundation for future studies addressing the limitations of previous studies. METHODS: Prospectively and retrospectively obtained data from participating sites were aggregated using a cloud-based registry and analyzed retrospectively. Standard preoperative variables and outcome measures included length of stay, unplanned readmission, and remission. RESULTS: By July 2023, 528 patients with CD had been treated by 26 neurosurgeons with varying levels of experience at 9 academic pituitary centers. No surgeon treated more than 81 of 528 (15.3%) patients. The mean ± SD patient age was 43.8 ± 13.9 years, and most patients were female (82.2%, 433/527). The mean tumor diameter was 0.8 ± 2.7 cm. Most patients (76.6%, 354/462) had no prior treatment. The most common pathology was corticotroph tumor (76.8%, 381/496). The mean length of stay was 3.8 ± 2.5 days. The most common discharge destination was home (97.2%, 513/528). Two patients (0.4%, 2/528) died perioperatively. A total of 57 patients (11.0%, 57/519) required an unplanned hospital readmission within 90 days of surgery. The median actuarial disease-free survival after index surgery was 8.5 years. CONCLUSION: This study examined an evolving multicenter collaboration on patient outcomes after surgery for CD. Our results provide novel insights on surgical outcomes not possible in prior single-center studies or with national administrative data sets. This collaboration will power future studies to better advance the standard of care for patients with CD.

Multifocal Ectopic Recurrence of a C2 Chordoma.

Background Chordomas are histologically benign but locally aggressive tumors with a high propensity to recur. Our case highlights the importance of long-term vigilance in patients who have undergone chordoma resection. Case Report We report the case of a 47-year-old man with a cervical chordoma who developed multiple musculoskeletal ectopic recurrences in the left supraclavicular region, the proximal right bicep, and the left submandibular region without recurrence in the primary tumor site. Primary tumor resection was achieved via a combination of surgery, adjuvant radiation therapy, and imatinib. All recurrences were successfully resected and confirmed via pathology to be ectopic chordoma. Discussion Ectopic recurrence of cervical chordoma is rare and lung is the most common site of distant spread. Chordoma recurrence in skeletal muscle is particularly rare, with only 10 cases described in the literature. A plausible mechanism of distant metastatic disease in chordoma patients suggests that tumor cells escape the surgical tract via a combination of cytokine release, vasodilation, and microtrauma induced during resection. Conclusion Cervical chordoma with ectopic recurrence in skeletal muscle has not been previously described in the literature. Skull base surgeons should be aware of the phenomenon of chordoma ectopic recurrence in the absence of local recurrence.

Evolution of Surgical Outcomes in Endoscopic Endonasal Resection of Craniopharyngiomas.

Introduction Wide variations exist in the management of craniopharyngiomas, including pituitary stalk preservation/sacrifice. This study examines the practice patterns over 16 years using the endoscopic endonasal approach for the resection of craniopharyngiomas and it examines the effects of stalk preservation. Methods Retrospective analysis was conducted for 66 patients who underwent endoscopic transsphenoidal surgery for resection of craniopharyngiomas. Patients were stratified into three epochs: 2005 to 2009 ( N = 20), 2010 to 2015 ( N = 23), and 2016 to 2020 ( N = 20), to examine the evolution of surgical outcomes. Subgroup analysis between stalk preservation/stalk sacrifice was conducted for rate of gross total resection, anterior pituitary function preservation, and development of new permanent diabetes insipidus. Results Gross total resection rates across the first, second, and third epochs were 20, 65, and 52%, respectively ( p = 0.042). Stalk preservation across epochs were 100, 5.9, and 52.6% ( p = 0.0001). New permanent diabetes insipidus did not significantly change across epochs (37.5, 68.4, 71.4%; p = 0.078). Preservation of normal endocrine function across epochs was 25, 0, and 23.8%; ( p = 0.001). Postoperative cerebrospinal fluid (CSF) leaks significantly decreased over time (40, 4.5, and 0%; [ p = 0.0001]). Stalk preservation group retained higher normal endocrine function (40.9 vs. 0%; p = 0.001) and less normal-preoperative to postoperative panhypopituitarism (18.4 vs. 56%; p = 0.001). Stalk sacrifice group achieved higher GTR (70.8 vs. 28%, p = 0.005). At last follow-up, there was no difference in recurrence/progression rates between the two groups. Conclusion There is a continuous evolution in the management of craniopharyngiomas. Gross total resection, higher rates of pituitary stalk and hormonal preservation, and low rates of postoperative CSF leak can be achieved with increased surgical experience.

Short-lasting unilateral neuralgiform headache attacks (SUNCT/SUNA): a narrative review of interventional therapies.

Short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing (SUNCT) and short-lasting unilateral neuralgiform headache with autonomic symptoms (SUNA) are disabling primary headache disorders. The advent of advanced imaging technologies and surgical techniques has translated to a growing arsenal of interventional therapies capable of treating headache disorders. This literature review sheds light on the current evidence available for interventional therapies in medically intractable SUNCT/SUNA. PubMed and EMBASE were searched for publications between 1978 and 2022. Inclusion criteria were SUNCT/SUNA studies reporting outcomes following occipital nerve stimulation (ONS), pulsed radiofrequency (PRF) of sphenopalatine ganglion (SPG), stereotactic radiosurgery (SRS), deep brain stimulation (DBS) or microvascular decompression (MVD) of the trigeminal nerve. A greater than 50% reduction in severity or a greater than 50% reduction in the number of attacks was defined as a successful response. The rate of successful responses for the various treatment modalities were as follows: ONS 33/41 (80.5%), PRF of SPG 5/9 (55.6%), DBS of the ventral tegmental area 14/16 (86.7%), SRS to the SPG and/or trigeminal nerve 7/9 (77.8%) and MVD 56/73 (76.7%). Mean follow-up time in months was 42.5 (ONS), 24.8 (PRF), 25.3 (DBS), 20.8 (SRS) and 42.4 (MVD). A significant proportion of SUNCT/SUNA patients remain refractory to medical therapy (45%-55%). This review discusses existing literature on interventional approaches, including neuromodulation, radiofrequency ablation, gamma knife radiosurgery and MVD. The outcomes are promising, yet limited data exist, underscoring the need for further research to develop a robust surgical management algorithm.

Perioperative Outcomes of a Hydrocortisone Protocol after Endonasal Surgery for Pituitary Adenoma Resection.

Introduction In pituitary adenomas (PAs), the use of postoperative steroid supplementation remains controversial, as it reduces peritumoral edema and sinonasal complaints but disrupts the detection of adrenal insufficiency (AI). It is unclear whether postoperative cortisol supplementation has a measurable effect on improving outcomes in patients with pituitary adenoma undergoing endoscopic transsphenoidal surgery (ETS). The objective of the study was to evaluate a postoperative steroid treatment protocol on various surgical outcomes in patients with PA undergoing ETS. Methods A retrospective cohort study was performed for patients undergoing ETS from 2005 to 2020 for PA at a single tertiary academic center. Patients were divided into two groups: those managed by a routine postoperative glucocorticoid supplementation protocol (steroid protocol) and those who received supplementation based on postoperative cortisol laboratory assessment (steroid sparing protocol). Management was otherwise the same between groups. Evaluation of length of stay (LOS), sinonasal outcomes, 30-day readmission, and perioperative complications, including AI, were performed. Results Among 535 patients, 21% ( n = 111) received postoperative steroids, while the remainder ( n = 424) did not. There were no differences in mean LOS (3 vs. 3 days, p = 0.72), sinonasal complaints (27 vs. 19%, p = 0.12), 30-day readmission (5% vs. 5%, p = 0.44), and perioperative complications (5 vs. 5%, p = 0.79) between both the groups. A multivariate model supported that both groups were comparable in predicting LOS, 30-day readmission, and complications. No reduction in readmission for AI was seen. Conclusion Routine administration of postoperative glucocorticoids did not significantly improve patient outcomes in patients with PA who underwent ETS.

Surgical Outcomes of Endoscopic Transsphenoidal Pituitary Adenoma Resection in Elderly Versus Younger Patients.

Introduction Pituitary adenomas (PAs) are one of the most common types of intracranial neoplasm with increased incidence in elderly patients. The outcomes of endoscopic transsphenoidal surgery (ETS) specifically on elderly patients remain unclear. Methods We performed a retrospective cohort study to compare elderly patients (age ≥65 years) with nonelderly patients (age <65 years) who underwent ETS for PA from January 2005 to December 2020. Surgical outcomes, including extent of resection, complication profile, length of stay, and endocrinopathy rates, were compared between elderly and nonelderly patients. Results A total of 690 patients were included, with 197 (29%) being elderly patients. Elderly patients showed higher rates of hypertension ( p < 0.05), myocardial infarction ( p < 0.01), and atrial fibrillation ( p = 0.01) but not other comorbidities. Elderly patients also had more frequent optic nerve involvement (72 vs. 61% of cases, p = 0.01). Tumor characteristics and other patient variables were otherwise similar between younger and elderly patients. Postoperative cerebrospinal fluid (CSF) leaks (2 vs. 2%, p = 0.8), 30-day readmission, reoperation, postoperative complications, and postoperative endocrinopathies were similar between younger and older patients. Subdividing patients into age <65, 65 to 79, and >80 years also did not demonstrate a worsening of surgical outcomes with age. Conclusion For well-selected elderly patients in experienced endoscopic skull base centers, good surgical outcomes similarly to younger patients may be achieved.

The use of telemedicine in pre-surgical evaluation: a retrospective cohort study of a neurosurgical oncology practice.

PURPOSE: To determine if there was a discrepancy between telemedicine versus in-person New Patient Visits (NPVs) regarding the conversion rate to operative and radiosurgery cases at a tertiary surgical neuro-oncology practice. METHODS: A retrospective analysis was performed of patients who had an outpatient encounter with a neurosurgeon from the Tumor Division at our institution's Department of Neurosurgery between February 1, 2021 and April 30, 2021. NPVs during this period were registered as either telemedicine or in-person appointments. The primary endpoint of the study was to compare the rate at which telemedicine NPVs and in-person NPVs underwent surgery or radiosurgery, reported as the surgical conversion rate. RESULTS: A total of 206 patients were included in this study. Of them, 119 (57.8%) were seen using telemedicine and 87 (42.2%) were seen in clinic via an in-person visit. A total of 70 (34%) of all patients underwent surgery or radiosurgery. Of the 119 patients seen via telemedicine, 40 (33.6%) underwent surgery or radiosurgery; during the same period, 87 NPVs were conducted in person and 30 (34.5%, p = 1.0) received an intervention. Further stratification revealed no differences between the two groups across measured criteria including diagnosis, number of pre-operative visits, elapsed time from appointment to surgery, follow-up visits, and distance from home address to neurosurgical clinic. CONCLUSION: Telemedicine NPVs did not differ significantly from in-person NPVs when evaluating the likelihood of a new patient committing to surgical treatment. This study provides quantifiable evidence that telemedicine is an effective means of meeting new patients and planning complex neurosurgical interventions.

Radiation of meningioma dural tail may not improve tumor control rates.

Introduction: Dural tails are thickened contrast-enhancing portions of dura associated with some meningiomas. Prior studies have demonstrated the presence of tumor cells within the dural tail, however their inclusion in radiation treatment fields remains controversial. We evaluated the role of including the dural tail when treating a meningioma with stereotactic radiation and the impact on tumor recurrence. Methods: This is a retrospective, single-institution, cohort study of patients with intracranial World Health Organization (WHO) grade 1 meningioma and identified dural tail who were treated with stereotactic radiosurgery (SRS) or fractionated stereotactic radiotherapy (FSRT) from January 2012 to December 2018. SRS and FSRT subgroups were categorized based on coverage or non-coverage of the dural tail by the radiation fields, as determined independently by a radiation oncologist and a neurosurgeon. Demographics, tumor characteristics, radiation plans, and outcomes were evaluated. High grade tumors were analyzed separately. Results: A total of 187 WHO grade 1 tumors from 177 patients were included in the study (median age: 62 years, median follow-up: 40 months, 78.1% female) with 104 receiving SRS and 83 receiving FSRT. The dural tail was covered in 141 (75.4%) of treatment plans. There was no difference in recurrence rates (RR) or time to recurrence (TTR) between non-coverage or coverage of dural tails (RR: 2.2% vs 3.5%, P = 1.0; TTR: 34 vs 36 months, P = 1.00). There was no difference in the rate of radiation side effects between dural tail coverage or non-coverage groups. These associations remained stable when SRS and FSRT subgroups were considered separately, as well as in a high grade cohort of 16 tumors. Conclusion: Inclusion of the dural tail in the SRS or FSRT volumes for meningioma treatment does not seem to reduce recurrence rate. Improved understanding of dural tail pathophysiology, tumor grade, tumor spread, and radiation response is needed to better predict the response of meningiomas to radiotherapy.

Targeted Therapy in the Management of Modern Craniopharyngiomas.

BACKGROUND: The proximity of craniopharyngiomas (CPs) to critical neurovascular structures can lead to a host of neurologic and endocrine complications that lead to difficulty with surgical management. In this review, we examine the molecular and genetic markers implicated in CP, their involvement in tumorigenic pathways, and their impact on CP prognosis and treatment. METHODS: We undertook a focused review of relevant articles, clinical trials, and molecular summaries regarding CP. RESULTS: Genetic and immunological markers show variable expression in different types of CP. BRAF is implicated in tumorigenesis in papillary CP (pCP), whereas CTNNB1 and EGFR are often overexpressed in adamantinomatous CP (aCP) and VEGF is overexpressed in aCP and recurrent CP. Targeted treatment modalities inhibiting these pathways can shrink or halt progression of CP. In addition, EGFR inhibitors may sensitize tumors to radiation therapy. These drugs show promise in medical management and neoadjuvant therapy for CP. Immunotherapy, including anti-interleukin-6 (IL-6) drugs and interferon treatment, are also effective in managing tumor growth. Ongoing clinical trials in CP are limited but are testing BRAF/MET inhibitors and IL-6 monoclonal antibodies. CONCLUSIONS: Genetic and immunological markers show variable expression in different subtypes of CP. Several current molecular treatments have shown some success in the management of this disease. Additional clinical trials and targeted therapies will be important to improve CP patient outcomes.

Telemedicine in Neurosurgery During the COVID-19 Outbreak: Where We Are 1 Year Later.

BACKGROUND: Telemedicine use skyrocketed in March 2020 on implementation of shelter-in-place measures owing to the coronavirus disease 2019 (COVID-19) pandemic. Within the past year, shelter-in-place measures were lifted and the COVID-19 vaccine was released, resulting in many neurosurgeons returning to in-person outpatient clinics. This study aimed to determine the extent of usage of telemedicine in neurosurgery 1 year into the COVID-19 pandemic. METHODS: A retrospective cohort study of patients who received neurosurgical care at a single institution from February 1 to April 18 of the years 2020 and 2021 was performed. The inclusion criteria were met by 11,592 patients. During the 2 study periods, 1465 patients underwent surgery, 7083 were seen in clinic via an in-person meeting, and 3044 were assessed via telemedicine. RESULTS: At 1 year after the COVID-19 outbreak, telemedicine usage was at 81.3% of the initial volume on implementation of shelter-in-place measures. In-person outpatient visits increased 40.2% from the early pandemic volume. Among the 4 neurosurgery divisions, telemedicine usage remained high in tumor and functional neurosurgery, significantly increased in vascular neurosurgery, and decreased in spine neurosurgery. CONCLUSIONS: Telemedicine use in neurosurgery clinics continues 1 year after the COVID-19 outbreak. Even after the lifting of shelter-in-place measures, many neurosurgeons still use telemedicine, while the operative volume remains stable. Owing to the limited physical examination that can be performed via current telemedicine platforms, telemedicine use in spine neurosurgery is lower than peak use during the early pandemic, while use has remained high among tumor, vascular, and functional neurosurgery.

Delayed symptomatic hyponatremia in transsphenoidal surgery: Systematic review and meta-analysis of its incidence and prevention with water restriction.

BACKGROUND: Delayed symptomatic hyponatremia (DSH) is an unpredictable postoperative complication after transsphenoidal pituitary surgery. Universal postoperative sodium screening and water restriction are two strategies to detect or prevent the development of DSH. We performed a meta-analysis of studies characterizing the rate of DSH using sodium screening and water restriction protocols. METHODS: Literature search was done using MEDLINE/PUBMED, EMBASE, and Cochrane databases. Inclusion criteria are (1) development of DSH after endoscopic or microscopic transsphenoidal, sellar surgery, and (2) reporting of a standardized postoperative sodium screening protocol for monitoring or prevention of DSH. RESULTS: A total of 23 publications fulfilled the inclusion criteria resulted in a total of 5870 patients. Two meta-analyses were conducted. Of the 19 studies (N = 4488 patients) examining rate of DSH after sodium screening, DSH rates ranged from 0% to 19.7%. In the first meta-analysis, using a random-effect estimate of the combined proportions, the overall rate of DSH was 5.60% (4.0%-7.1%, I2 = 96.54%, T2 = 0.0007). In the second meta-analysis, a fixed-effect model of four studies consisted of 1382 patients. Eight hundred fifty-two patients were included prior to and 530 were included after water restriction protocol. Meta-analysis showed an odds ratio (OR) of 5.02 (95% CI: 2.16-11.65) favoring water restriction. CONCLUSION: This meta-analysis summarized rates of DSH with sodium screening protocol to be 5.60% (4.0%-7.1%) and showed a decreased risk of DSH after implementation of a water restriction protocol. The results are limited due to few studies examining fluid restriction (N = 4) and heterogeneity in water restriction protocols. No adverse events were seen with fluid restriction protocol. Prospective and multicenter studies should be conducted to further investigate the utility of water restriction following transsphenoidal pituitary surgery.

Evaluation of early postoperative day 1 discharge after endoscopic endonasal pituitary adenoma resection.

OBJECTIVE: While multiple studies have evaluated the length of stay after endonasal transsphenoidal surgery (ETS) for pituitary adenoma, the potential for early discharge on postoperative day 1 (POD 1) remains unclear. The authors compared patients discharged on POD 1 with patients discharged on POD > 1 to better characterize factors that facilitate early discharge after ETS. METHODS: A retrospective chart review was performed for patients undergoing ETS for pituitary adenoma at a single tertiary care academic center from February 2005 to February 2020. Discharge on POD 1 was defined as a discharge within 24 hours of surgery. RESULTS: A total of 726 patients (mean age 55 years, 52% male) were identified, of whom 178 (24.5%) patients were discharged on POD 1. These patients were more likely to have pituitary incidentaloma (p = 0.001), require dural substitutes and DuraSeal (p = 0.0001), have fewer intraoperative CSF leaks (p = 0.02), and have lower postoperative complication rates (p = 0.006) compared with patients discharged on POD > 1. POD 1 patients also showed higher rates of macroadenomas (96.1% vs 91.4%, p = 0.03) and lower rates of functional tumors (p = 0.02). POD > 1 patients were more likely to have readmission within 30 days (p = 0.002), readmission after 30 days (p = 0.0001), nasal synechiae on follow-up (p = 0.003), diabetes insipidus (DI; 1.7% vs 9.8%, p = 0.0001), postoperative hypocortisolism (21.8% vs 12.1%, p = 0.01), and postoperative steroid usage (44.6% vs 59.7%, p = 0.003). The number of patients discharged on POD 1 significantly increased during each subsequent time epoch: 2005-2010, 2011-2015, and 2016-2020 (p = 0.0001). On multivariate analysis, DI (OR 7.02, 95% CI 2.01-24.57; p = 0.002) and intraoperative leak (OR 2.02, 95% CI 1.25-3.28; p = 0.004) were associated with increased risk for POD > 1 discharge, while operation epoch (OR 0.46, 95% CI 0.3-0.71; p = 0.0001) was associated with POD 1 discharge. CONCLUSIONS: This study demonstrates that discharge on POD 1 after ETS for pituitary adenomas was safe and feasible and without increased risk of 30-day readmission. On multivariate analysis, surgical epoch was associated with decreased risk of prolonged length of stay, while factors associated with increased risk of prolonged length of stay included DI and intraoperative CSF leak. These findings may help in selecting patients who are deemed reasonable for safe, early discharge after pituitary adenoma resection.