Significant Variability in Postoperative Thromboprophylaxis in Cushing's Disease Patients: A Survey of the North American Skull Base Society and the AANS/CNS Joint Tumor Section.

Introduction Cushing's disease (CD) is associated with hypercoagulability which is associated with an increased risk of venous thromboembolic events (VTEs) perioperatively. This risk persists even after successful transsphenoidal surgery (TSS). However, there are no current guidelines for pharmacologic thromboprophylaxis in this patient population. Objective Characterize existing thromboprophylaxis management practices in patients undergoing TSS for CD. Methods An anonymous RedCap survey comprised of questions about perioperative thromboprophylaxis in CD patients was distributed via the American Association of Neurological Surgeons (AANS)/Congress of Neurological Surgeons (CNS) Joint Tumor Section and the North American Skull Base Society (NASBS) email lists. Results The survey was distributed to 554 members of the AANS/CNS Joint Tumor Section and 1,094 members of NASBS asking that members who surgically treat CD respond. Sixty responses (3.0% response rate) were received. Fifty-two (86.7%) respondents are involved in the postoperative management of CD patients. Thirty-six (69.2%) treat all patients with postoperative VTE chemoprophylaxis, 8 (15.4%) treat some patients, while 8 (15.4%) do not use chemoprophylaxis. Preferred chemoprophylaxis varies as 26 (59.1%) administer low molecular weight heparin, 14 (31.8%) give unfractionated heparin, 1 (2.3%) give direct oral anticoagulants, and 3 (6.8%) give aspirin. Most (28, 53.8%) of the respondents perceive the VTE risk in this patient population to be 0 to 5%, 16 (30.8%) perceive the risk to be 6 to 10%, and 8 (15.4%) perceive it to be 11 to 20%. Conclusion There is great variability in VTE detection and postoperative prevention practice patterns in CD patients. This study highlights the need for prospective studies to clarify optimal pharmacologic chemoprophylaxis strategies and duration in this patient population.

Venous Thromboembolism and Prevention Strategies in Patients with Cushing's Disease: A Systematic Review.

BACKGROUND: There is a well-documented association between Cushing's syndrome (CS) and hypercoagulability. However, there is limited data on the risk of venous thromboembolic events (VTEs) after surgery for Cushing's disease (CD). There is no consensus on optimal postoperative anticoagulation strategies in this group. This review gathers information on the rates of VTE after surgery for CD, as well as reported prophylaxis strategies in this population. METHODS: A literature search was performed using Cochrane Library, EMBASE, and Ovid MEDLINE databases according to Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines. A narrative review of papers discussing mechanisms of hypercoagulability in CS was conducted. RESULTS: Twenty-five relevant papers were identified out of 944 papers yielded. Pooled postoperative VTE incidence in patients undergoing transsphenoidal surgery for CD was 2% (58 out of 2997). The pooled rate of VTE-associated mortality based on the 23 studies that reported this information was 0.2% (6 out of 2077). There were no cases of postoperative VTE in the 191 patients undergoing adrenalectomy for benign adrenocorticotropic hormone-independent CS. Ten studies reported information on perioperative thromboprophylaxis strategies in transsphenoidal surgery for CD and adrenalectomy for adrenocorticotropic hormone-independent CS. CONCLUSIONS: Postoperative VTE in CD is a source of morbidity, with a rate of 2% and mortality rate of 0.2%, highlighting that surgical resection of the corticotroph adenoma does not necessarily result in immediate resolution of hypercoagulability. Increased production of coagulation factors, impaired fibrinolysis, inflammation, and CS-associated metabolic risk factors all factor into the pathogenesis of CS-associated hypercoagulability. Further study is needed on an optimal pharmacologic prophylaxis strategy.

Implementation of a Standardized Interdisciplinary Perioperative Protocol for Patients Undergoing Transsphenoidal Surgery: Impact on Patient Outcomes.

INTRODUCTION: Advances in endoscopic endonasal transsphenoidal surgery have led to improved postoperative outcomes after pituitary adenoma resection, including reduced length of stay, complications and readmission rates, without compromising safety and satisfaction. METHODS: Our team implemented a perioperative protocol in January 2021 for patients undergoing endoscopic, transsphenoidal pituitary surgery. This study compares preoperative characteristics and postoperative outcomes in 279 patients between 2016 and 2022 (128 preprotocol and 151 postprotocol). Our protocol includes interdisciplinary preoperative evaluations, unified communication, cortisol thresholds for postoperative glucocorticoid replacement, and fluid restriction to prevent delayed hyponatremia. RESULTS: Median age was 54 ± 17 years with 50.8% female patients. There were 229 (82.1%) macroadenomas (>1 cm) and 50 (17.9%) microadenomas/cysts (<1 cm). Mean diameter was 18 (transverse), 18 (craniocaudal), 16 (anteroposterior) mm. Tumor types included 125 (44.8%) gonadotroph, 46 (16.4%) adrenocorticotroph, 40 (14.3%) lactotroph, 26 (9.3%) Rathke cysts, 19 (6.8%) somatotroph, 13 (4.6%) nondiagnostic, 3 (1%) somatotroph-lactotroph, 3 (1%) mammosomatotroph, 2 (0.71%) null cell, and 2 (0.7%) thyrotroph adenomas. Postprotocol, 74.2% of patients were discharged on postoperative day 1 compared with 46.1% preprotocol (P < 0.0001). Transient arginine vasopressin deficiency decreased from 10.4% (preprotocol) to 4.6% postprotocol (P = 0.101). Hyponatremia occurred in 13.3% pre-protocol and 4.6% postprotocol. Emergency department visits dropped from 9.4% to 3.9%, and readmissions decreased from 7.8% to 2.6%. Persistent arginine vasopressin deficiency affected 2.3% preprotocol and 1.3% postprotocol patients. Cerebrospinal fluid leaks occurred in 8.5% preprotocol and 7.3% postprotocol. CONCLUSIONS: Implementing an interdisciplinary, perioperative protocol for transsphenoidal endoscopic pituitary surgery improves length of stay while minimizing readmissions and surgery-related complications.

HL7 Fast Healthcare Interoperability Resources (HL7 FHIR) in digital healthcare ecosystems for chronic disease management: Scoping review.

BACKGROUND: The prevalence of chronic diseases has shifted the burden of disease from incidental acute inpatient admissions to long-term coordinated care across healthcare institutions and the patient's home. Digital healthcare ecosystems emerge to target increasing healthcare costs and invest in standard Application Programming Interfaces (API), such as HL7 Fast Healthcare Interoperability Resources (HL7 FHIR) for trusted data flows. OBJECTIVES: This scoping review assessed the role and impact of HL7 FHIR and associated Implementation Guides (IGs) in digital healthcare ecosystems focusing on chronic disease management. METHODS: To study trends and developments relevant to HL7 FHIR, a scoping review of the scientific and gray English literature from 2017 to 2023 was used. RESULTS: The selection of 93 of 524 scientific papers reviewed in English indicates that the popularity of HL7 FHIR as a robust technical interface standard for the health sector has been steadily rising since its inception in 2010, reaching a peak in 2021. Digital Health applications use HL7 FHIR in cancer (45 %), cardiovascular disease (CVD) (more than 15 %), and diabetes (almost 15 %). The scoping review revealed that references to HL7 FHIR IGs are limited to âˆ¼ 20 % of articles reviewed. HL7 FHIR R4 was most frequently referenced when the HL7 FHIR version was mentioned. In HL7 FHIR IGs registries and the internet, we found 35 HL7 FHIR IGs addressing chronic disease management, i.e., cancer (40 %), chronic disease management (25 %), and diabetes (20 %). HL7 FHIR IGs frequently complement the information in the article. CONCLUSIONS: HL7 FHIR matures with each revision of the standard as HL7 FHIR IGs are developed with validated data sets, common shared HL7 FHIR resources, and supporting tools. Referencing HL7 FHIR IGs cataloged in official registries and in scientific publications is recommended to advance data quality and facilitate mutual learning in growing digital healthcare ecosystems that nurture interoperability in digital health innovation.

Differential tractography and whole brain connectometry in primary motor area gliomas resection: A feasibility study.

OBJECTIVE: Establish the evolution of the connectome before and after resection of motor area glioma using a comparison of connectome maps and high-definition differential tractography (DifT). METHODS: DifT was done using normalized quantitative anisotropy (NQA) with DSI Studio. The quantitative analysis involved obtaining mean NQA and fractional anisotropy (FA) values for the disrupted pathways tracing the corticospinal tract (CST), and white fiber network changes over time. RESULTS: We described the baseline tractography, DifT, and white matter network changes from two patients who underwent resection of an oligodendroglioma (Case 1) and an IDH mutant astrocytoma, grade 4 (Case 2). CASE 1: There was a slight decrease in the diffusion signal of the compromised CST in the immediate postop. The NQA and FA values increased at the 1-year follow-up (0.18 vs. 0.32 and 0.35 vs. 0.44, respectively). CASE 2: There was an important decrease in the immediate postop, followed by an increase in the follow-up. In the 1-year follow-up, the patient presented with radiation necrosis and tumor recurrence, increasing NQA from 0.18 in the preop to 0.29. Fiber network analysis: whole-brain connectome comparison demonstrated no significant changes in the immediate postop. However, in the 1-year follow up there was a notorious reorganization of the fibers in both cases, showing the decreased density of connections. CONCLUSIONS: Connectome studies and DifT constitute new potential tools to predict early reorganization changes in a patient's networks, showing the brain plasticity capacity, and helping to establish timelines for the progression of the tumor and treatment-induced changes.

CDK4/6 inhibitors and endocrine therapy in the treatment of metastatic breast cancer: A real-world and propensity score-adjusted comparison.

INTRODUCTION/BACKGROUND: Hormone Receptor-positive (HR+) and Human Epidermal Growth Factor Receptor 2-negative (HER2-) breast cancer is the most common subtype, predominantly treated with endocrine therapy. The efficacy of CDK4/6 inhibitors combined with endocrine therapy in this context remains to be fully evaluated. MATERIALS (OR PATIENTS) AND METHODS: This study compared the effectiveness of CDK4/6 inhibitors (palbociclib and ribociclib) in combination with an aromatase inhibitor or fulvestrant against endocrine therapy alone in patients with HR+/HER2- advanced breast cancer. The main focus was on progression-free survival (PFS) and overall survival (OS). The study involved a population treated exclusively with endocrine therapy for bone involvement, examining median OS and PFS, and adjusting for variables like stage, visceral metastasis, age, and treatment line. RESULTS: The study found no significant OS difference between treatments with palbociclib, ribociclib, and endocrine therapy alone. However, ribociclib combined with letrozole significantly improved PFS over letrozole alone. Propensity score weighting indicated a potential 50 % reduction in death risk with ribociclib compared to palbociclib, though this was not confirmed by cox regression. CONCLUSION: CDK4/6 inhibitors, particularly ribociclib in combination with letrozole, show promise in improving outcomes for HR+/HER2- breast cancer patients. While palbociclib may not be superior to traditional endocrine therapy, the results underscore the need for further research. These findings could influence future treatment protocols, emphasizing the importance of personalized therapy in this patient group.

Recurrence morbidity of olfactory neuroblastoma.

BACKGROUND: With modern treatment paradigms, olfactory neuroblastoma (ONB) has favorable overall survival (OS); however, the incidence of recurrence remains high. The primary aims of this study were to delineate the prognosis of recurrence of ONB and explore how recurrence subsites are associated with OS, disease-specific survival (DSS), and further recurrence. METHODS: A retrospective chart review of ONB cases from nine academic centers between 2005 and 2021 was completed. Tumor characteristics, recurrence subsites, timelines to recurrence, additional recurrences, and survival estimates were determined using descriptive and time-to-event analyses. RESULTS: A final cohort of 233 patients was identified, with 70 (30.0%) patients recurring within 50.4 (standard deviation ±40.9) months of diagnosis on average, consisting of local (50%), neck (36%), intracranial (9%), and distant (6%) recurrence. Compared with subjects without recurrence, patients with recurrence had significantly different primary American Joint Committee on Cancer T stage (p < 0.001), overall stage (p < 0.001), and modified Kadish scores (p < 0.001). Histopathology identified that dural involvement and positive margins were significantly greater in recurrent cases. First recurrence was significantly associated with worse 5-year DSS (hazard ratio = 5.62; p = 0.003), and subjects with neck or local recurrence had a significantly better DSS compared to intracranial or distant recurrence. CONCLUSIONS: Recurrent cases of ONB have significantly different stages and preoperative imaging factors. Patients with local or neck recurrence, however, have better DSS than those with intracranial or distant recurrence, independent of initial tumor stage or Hyams grade. Identifying specific factors that confer an increased risk of recurrence and DSS is important for patient counseling in addition to surveillance planning.

Performance of intraoperative neurocognitive tests during awake surgery for patients with diffuse low-grade glioma.

Despite great advancements and the diffusion of awake surgery for brain tumors, the literature shows that the tests applied during the procedure are heterogeneous and non-standardized. This prospective, observational, descriptive study collected data on intraoperative brain mapping and the performance of multiple neurocognitive tests in 51 awake surgeries for diffuse low-grade glioma. Frequency of use and rate of intraoperative findings of different neurocognitive tests were analyzed. Patients mean age at the time of surgery was 35.1 (20-57) years. We performed 26 (51.0%) surgeries on the left hemisphere (LH) and 25 (49.0%) on the right hemisphere (RH). Significant differences were observed between the total number of functional findings (cortical and subcortical) identified in the LH and RH (p = 0.004). In subcortical findings alone, the differences remained significant (p = 0.0004). The RH subcortical region showed the lowest number of intraoperative findings, and this was correlated with functional outcome: Karnofsky performance scale at five days (p = 0.022), three months (p = 0.002) and one year (p = 0.002) post-surgery. On average, more tests were used to map the RH, with a lower frequency of both cortical and subcortical functional findings. Even though subcortical findings were less frequent than cortical findings, they were crucial to defining the resection margins. Based on the intraoperative findings, frequency of use, and rate of findings per use of the tests analyzed, the most relevant tests for each hemisphere for awake brain mapping were identified.

Sustainable approach towards alternatives for the use of iron ore tailings in the construction sector using Data Envelopment Analysis methodology.

Iron ore tailings (IOTs) need to be properly managed to mitigate the environmental, social, and economic impacts of mining activities. To cope with this issue, we use data envelopment analysis (DEA) to evaluate alternatives for using IOT in the construction sector. The classical and weight restriction output-oriented DEA models were used in this analysis. The results show that the ranking of alternatives depends on the aspect being evaluated. Concrete block is the most environmentally friendly alternative when analysing both models. For both social and economic aspects, ceramics produced better results in the classical model, whereas Portland cement showed better outcomes in the weight restriction model. In this sense, the results suggest great potential for the use of IOT in the construction sector, enabling the reduction of risks and social and environmental impacts of tailings dams.

Endoscopic Endonasal Transcavernous Hemipituitary Transposition for Resection of Invasive Prolactinoma: 2-Dimensional Operative Video.

Invasive prolactinomas often require multimodal management including medical and surgical interventions. Here, we present the case of a 34-year-old man with a history of progressive visual disturbances. MRI unveiled a sella lesion with suprasellar and retrosellar extensions. Elevated prolactin levels (6125 ng/mL) confirmed the diagnosis of prolactinoma, leading to initiation of medical treatment, with gradual escalation to maximum dosing. The patient achieved only partial hormonal response and incomplete improvement of symptoms, and therefore, surgical intervention was pursued with objective of maximum safe resection. The patient consented to the procedure. An endonasal endoscopic approach was selected. Surgical procedures encompassed transsellar, transtuberculum, and transplanum approaches, extended laterally to expose the right parasellar carotid and the anterior wall of the cavernous sinus. Subsequent steps involved opening the sellar and suprasellar dura mater, anterior wall of the cavernous sinus, and transcavernous hemipituitary transposition for access to the retrosellar region.1-5 Debulking of the lesion was performed, followed by dissection of the retrosellar space and resection of tumor component within the interpeduncular cistern. Reconstruction employed dura substitute and vascularized nasoseptal flap. Histopathology confirmed diagnosis of prolactinoma. Postoperative MRI findings and significantly improved prolactin levels (50 ng/mL) were compatible with near total resection. The patient's postoperative course was uneventful, resulting in discharge on the second postoperative day. The patient was additionally started on cabergoline 0.5 mg 2x/week to achieve hormonal control. This case demonstrates the application of surgical anatomy and its translation in modern surgical techniques that allow improved resection of such complex tumors while ensuring optimal clinical outcomes.

A Rare Case of Pulmonary Artery Sarcoma.

Pulmonary artery sarcoma (PAS) is a rare and aggressive mesenchymal tumor with an overall poor prognosis1-5. Due to similar clinical and radiologic findings, PAS is often misdiagnosed as a pulmonary embolism (PE) frequently leading to prolonged anticoagulation therapy, which delays the correct diagnosis 1-3. By presenting this clinical case our objective is to emphasize characteristic CT findings that favour a neoplastic origin of a pulmonary intravascular filling defect. PET-CT and MRI have also an important potential role in its diagnosis and therapeutical management.

Spontaneous rhinorrhea and idiopathic intracranial hypertension: a complex and challenging association.

INTRODUCTION: Spontaneous CSF leak is a known complication of idiopathic intracranial hypertension (IIH). Patients with CSF rhinorrhea present a unique challenge within the IIH population, as the occurrence of a leak can mask the typical IIH symptoms and signs, complicating the diagnosis. Treatment of leaks in this population can also be challenging, with the risk of rhinorrhea recurrence if intracranial hypertension is not adequately treated. OBJECTIVE: The aim of this narrative review was to examine current literature on the association between spontaneous CSF rhinorrhea leaks and IIH, focusing on key clinical features, diagnostic approaches, management strategies, and outcomes. MATERIAL AND METHODS: A literature search was executed using the PubMed and Scopus databases. The search was confined to articles published between January 1985 and August 2023; extracted data was then analysed to form the foundation of the narrative review. RESULTS: This search yielded 26 articles, comprising 943 patients. Average age was 46.8 ± 6.5 years, and average body mass index was 35.8 ± 4.8. Most of the patients were female (74.33%). Presenting symptoms were rhinorrhea, headaches and meningitis. The most common imaging findings were empty sella and encephalocele. The standard treatment approach was endoscopic endonasal approach for correction of CSF rhinorrhea leak, and shunt placement was also performed in 128 (13%) patients. Recurrences were observed in 10% of cases. CONCLUSIONS: The complex relationship between spontaneous CSF leaks and IIH is a challenge that benefits from multidisciplinary evaluation and management for successful treatment. Treatments such as endoscopic repair, acetazolamide, and VP/ /LP shunts reduce complications and recurrence. Personalised plans addressing elevated intracranial pressure are crucial for successful outcomes.

Repeated surgical resections for management of high-grade glioma and its impact on quality of life.

PURPOSE: High-grade gliomas (HGG) are aggressive cancers, and their recurrence is inevitable, despite advances in treatment options. While repeated tumor resection has been shown to increase survival rate, its impact on quality of life is not clearly defined. To address this gap, we compared quality of life (QoL) changes in HGG patients who underwent first-time (FTR) versus repeat surgical resections (RSR) for management of recurrence. METHODS: Forty-four adults with HGG who underwent tumor resection were included in this study and classified into either the FTR group (n = 23) or the RSR group (n = 21). All patients completed comprehensive neuropsychological evaluations that included the Functional Assessment of Cancer Therapy-General (FACT-G) and Functional Assessment of Cancer Therapy-Cognitive Function (FACT-Cog) scales, pre-operatively and at two weeks post-operatively. RESULTS: There was no difference between the FTR and RSR groups in any of the QoL indices (all p > .05), except for improved emotional well-being and worsened social well-being, suggesting minimal detrimental effects of repeat surgeries on QoL in comparison to first time surgery. CONCLUSIONS: These results suggest that repeated resection is a viable strategy in certain cases for management of HGG recurrence, with similar impact on QoL as observed in patients undergoing first time surgery. These encouraging outcomes provide useful insight to guide treatment strategies and patient and clinician decision making to optimize surgical and functional outcomes.

Awake Craniotomy Program Implementation.

Importance: Implementing multidisciplinary teams for treatment of complex brain tumors needing awake craniotomies is associated with significant costs. To date, there is a paucity of analysis on the cost utility of introducing advanced multidisciplinary standardized teams to enable awake craniotomies. Objective: To assess the cost utility of introducing a standardized program of awake craniotomies. Design, Setting, and Participants: A retrospective economic evaluation was conducted at Mayo Clinic Florida. All patients with single, unilateral lesions who underwent elective awake craniotomies between January 2016 and December 2021 were considered eligible for inclusion. The economic perspective of the health care institution and a time horizon of 1 year were considered. Data were analyzed from October 2022 to May 2023. Exposure: Treatment with an awake craniotomy before standardization (2016-2018) compared with treatment with awake craniotomy after standardization (2018-2021). Main Outcomes and Measures: Patient demographics, perioperative, and postoperative outcomes, including length of stay, intensive care (ICU) admission, extent of resection, readmission rates, and 1-year mortality were compared between patients undergoing surgery before and after standardization. Direct medical costs were estimated from Medicare reimbursement rates for all billed procedures. A cost-utility analysis was performed considering differences in direct medical costs and in 1-year mortality within the periods before and after standardization of procedures. Uncertainty was explored in probability sensitivity analysis. Results: A total of 164 patients (mean [SD] age, 49.9 [15.7] years; 98 [60%] male patients) were included in the study. Of those, 56 underwent surgery before and 108 after implementation of procedure standardization. Procedure standardization was associated with reductions in length of stay from a mean (SD) of 3.34 (1.79) to 2.46 (1.61) days (difference, 0.88 days; 95% CI, 0.33-1.42 days; P = .002), length of stay in ICU from a mean (SD) of 1.32 (0.69) to 0.99 (0.90) nights (difference, 0.33 nights; 95% CI, 0.06-0.60 nights; P = .02), 30-day readmission rate from 14% (8 patients) in the prestandardization cohort to 5% (5 patients) (difference, 9%; 95% CI, 19.6%-0.3%; P = .03), while extent of resection and intraoperative complication rates were similar between both cohorts. The standardized protocol was associated with mean (SD) savings of $7088.80 ($12 389.50) and decreases in 1-year mortality (dominant intervention). This protocol was found to be cost saving in 75.5% of all simulations in probability sensitivity analysis. Conclusions and Relevance: In this economic evaluation of standardization of awake craniotomy, there was a generalized reduction in length of stay, ICU admission time, and direct medical costs with implementation of an optimized protocol. This was achieved without compromising patient outcomes and with similar extent of resection, complication rates, and reduced readmission rates.

A Prospective, Multicenter, Observational Study of Surgical vs Nonsurgical Management for Pituitary Apoplexy.

CONTEXT: Pituitary apoplexy (PA) has been traditionally considered a neurosurgical emergency, yet retrospective single-institution studies suggest similar outcomes among patients managed medically. OBJECTIVE: We established a multicenter, international prospective registry to compare presentation and outcomes in PA patients treated with surgery or medical management alone. METHODS: A centralized database captured demographics, comorbidities, clinical presentation, visual findings, hormonal status, and imaging features at admission. Treatment was determined independently by each site. Key outcomes included visual, oculomotor, and hormonal recovery, complications, and hospital length of stay. Outcomes were also compared based on time from symptom onset to surgery, and from admission or transfer to the treating center. Statistical testing compared treatment groups based on 2-sided hypotheses and P less than .05. RESULTS: A total of 100 consecutive PA patients from 12 hospitals were enrolled, and 97 (67 surgical and 30 medical) were evaluable. Demographics, clinical features, presenting symptoms, hormonal deficits, and imaging findings were similar between groups. Severe temporal visual field deficit was more common in surgical patients. At 3 and 6 months, hormonal, visual, and oculomotor outcomes were similar. Stratifying based on severity of visual fields demonstrated no difference in any outcome at 3 months. Timing of surgery did not affect outcomes. CONCLUSION: We found that medical and surgical management of PA yield similar 3-month outcomes. Although patients undergoing surgery had more severe visual field deficits, we could not clearly demonstrate that surgery led to better outcomes. Even without surgery, apoplectic tumor volumes regress substantially within 2 to 3 months, indicating that surgery is not always needed to reduce mass effect.

Extent of Surgical Resection as a Predictor of Tumor Progression in Skull Base Chordomas: A Multicenter Volumetric Analysis.

INTRODUCTION: Skull-base chordomas are aggressive tumors with a propensity for recurrence/progression. Even with standard of care (SoC), 5-year recurrence rates are variable (19%-54%). This high recurrence/progression rate correlates with increased morbidity and mortality. We sought to analyze a multicenter cohort of skull base chordomas to identify predictors of progression in patients receiving SoC. METHODS: The [Blinded]-Neurosurgery data registry was queried for skull base chordomas treated from 2008-2020. Patients with the histopathologic diagnosis of chordoma were included. The cohort was composed of patients with preoperative and postoperative magnetic resonance imaging. Tumor volume and radiologic characteristics were obtained from axial T2 sequences using a Digital Imaging and Communications in Medicine viewer. Survival analysis was performed using Kaplan-Meier method, and time-to-event multivariate regression was performed to identify independent predictors of progression. RESULTS: The cohort included 195 patients, of which 66 patients met inclusion criteria; median age was 44, and 28 (42%) were females. Fifty-four (82%) received SoC, 7 (11%) resection only, and 5 (8%) radiotherapy only. Median preoperative and postoperative tumor volumes were 11.55 cm3 (0.33-54.89) and 0.34 cm3 (0-42.52), respectively. Recurrence rate with SoC was 37%. Postoperative tumor volume (P = 0.010) correlated with progression. A postoperative volume of >4.9 cm3 (P = 0.044), ≤81.3% of tumor resection (P = 0.02), and lower-clivus location (P < 0.005) correlated with decreased time to progression. CONCLUSIONS: Skull base chordomas can be challenging to resect. Even though maximal resection and radiotherapy improve rate of tumor progression, many of these lesions eventually recur. We have identified a postoperative tumor volume of ≥4.9 cm3 and extent of resection of ≤81.3% in this cohort as predictors of progression in patients receiving SoC.

Secondary (additional) findings from the 100,000 Genomes Project: Disease manifestation, health care outcomes, and costs of disclosure.

PURPOSE: The UK 100,000 Genomes Project offered participants screening for additional findings (AFs) in genes associated with familial hypercholesterolemia (FH) or hereditary cancer syndromes including breast/ovarian cancer (HBOC), Lynch, familial adenomatous polyposis, MYH-associated polyposis, multiple endocrine neoplasia (MEN), and von Hippel-Lindau. Here, we report disclosure processes, manifestation of AF-related disease, outcomes, and costs. METHODS: An observational study in an area representing one-fifth of England. RESULTS: Data were collected from 89 adult AF recipients. At disclosure, among 57 recipients of a cancer-predisposition-associated AF and 32 recipients of an FH-associated AF, 35% and 88%, respectively, had personal and/or family history evidence of AF-related disease. During post-disclosure investigations, 4 cancer-AF recipients had evidence of disease, including 1 medullary thyroid cancer. Six women with an HBOC AF, 3 women with a Lynch syndrome AF, and 2 individuals with a MEN AF elected for risk-reducing surgery. New hyperlipidemia diagnoses were made in 6 FH-AF recipients and treatment (re-)initiated for 7 with prior hyperlipidemia. Generating and disclosing AFs in this region cost £1.4m; £8680 per clinically significant AF. CONCLUSION: Generation and disclosure of AFs identifies individuals with and without personal or familial evidence of disease and prompts appropriate clinical interventions. Results can inform policy toward secondary findings.

Intradural Pituitary Hemitransposition: Technical Note and Case Series Illustration.

BACKGROUND AND OBJECTIVES: Lesions located in the retrosellar region, interpeduncular cistern, and petroclival region are among the most difficult to access in neurosurgery. Transcranial approaches are useful; however, the large distance between the surgeon and the lesion as well as the presence of major neurovascular structures surrounding the lesion may limit surgical exposure. A midline transsphenoidal route avoids transgression of the neurovascular plane and provides direct access to the interpeduncular cistern. To safely access the interpeduncular fossa, it requires mobilization of the pituitary gland. The pituitary hemitransposition technique permits mobilization of the gland, while preserving its venous drainage and arterial supply to the gland on one of its sides, preserving gland function. The authors aim to describe the intradural pituitary hemitransposition technique and to demonstrate its safe application for resection of skull base tumors in the retrosellar space. METHODS: The authors describe the surgical technique and illustrate its application in 5 cases of different types of skull base tumors, including a video demonstrating all the steps to perform this approach. In addition, the authors discuss the advantages and limitations of this technique compared with other approaches to the retrosellar space. RESULTS: The intradural pituitary hemitransposition technique was used to safely resect a chondrosarcoma, chordoma, craniopharyngioma, teratoma, and meningioma involving the parasellar and retrosellar spaces, while minimizing endocrine morbidity. We had one patient with mild, albeit permanent hyperprolactinemia and hypothyroidism after surgery. No other patients had permanent dysfunction related to surgery. CONCLUSION: The endonasal endoscopic intradural pituitary hemitransposition approach is an effective technique for resection of lesions located within the retrosellar and petroclival regions, allowing adequate exposure while potentially optimizing the preservation of the pituitary function.

Are we ready to stratify BI-RADS 4 lesions observed on magnetic resonance imaging? A real-world noninferiority/equivalence analysis / Estamos prontos para estratificar lesões BI-RADS 4 na ressonância magnética? Uma análise de não inferioridade/equivalência do mundo real

Abstract Objective: To demonstrate that positive predictive values (PPVs) for suspicious (category 4) magnetic resonance imaging (MRI) findings that have been stratified are equivalent to those stipulated in the American College of Radiology Breast Imaging Reporting and Data System (BI-RADS) for mammography and ultrasound. Materials and Methods: This retrospective analysis of electronic medical records generated between January 4, 2016 and December 29, 2021 provided 365 patients in which 419 suspicious (BI-RADS category 4) findings were subcategorized as BI-RADS 4A, 4B or 4C. Malignant and nonmalignant outcomes were determined by pathologic analyses, follow-up, or both. For each subcategory, the level 2 PPV (PPV2) was calculated and tested for equivalence/noninferiority against the established benchmarks. Results: Of the 419 findings evaluated, 168 (40.1%) were categorized as malignant and 251 (59.9%) were categorized as nonmalignant. The PPV2 for subcategory 4A was 14.2% (95% CI: 9.3-20.4%), whereas it was 41.2% (95% CI: 32.8-49.9%) for subcategory 4B and 77.2% (95% CI: 68.4-84.5%) for subcategory 4C. Multivariate analysis showed a significantly different cancer yield for each subcategory (p < 0.001). Conclusion: We found that stratification of suspicious findings by MRI criteria is feasible, and malignancy probabilities for sub-categories 4B and 4C are equivalent to the values established for the other imaging methods in the BI-RADS. Nevertheless, low suspicion (4A) findings might show slightly higher malignancy rates.

Resumo Objetivo: Demonstrar que os valores preditivos positivos (VPPs) para lesões suspeitas (categoria 4) identificadas por ressonância magnética (RM) são equivalentes aos estipulados no ACR BI-RADS para mamografia e ultrassonografia. Materiais e Métodos: Análise retrospectiva de dados em prontuário eletrônico, entre 4 de janeiro de 2016 e 29 de dezembro de 2021, resultou em 365 pacientes elegíveis, com 419 lesões classificadas como BI-RADS 4A, 4B ou 4C. Desfechos malignos e não malignos foram determinados por estudo patológico e/ou acompanhamento. Realizamos o cálculo do VPP nível 2 (VPP2) para cada subcategoria e testamos para não inferioridade/equivalência em relação aos valores de referência. Resultados: Dos 419 achados, 168 (40,1%) foram malignos e 251 (59,9%), não malignos. O VPP2 para subcategoria 4A foi 14,2% (IC 95%: 9,3-20,4%), para 4B foi 41,2% (IC 95%:, 32,8-49,9%) e para 4C foi 77,2% (IC 95%: 68,4-84,5%). Análise multivariada demonstrou diferenças estatisticamente significantes entre as subcategorias (p < 0,001). Conclusão: A estratificação de achados suspeitos por RM é factível, sendo que a probabilidade de malignidade das subcategorias 4B e 4C é equivalente à estabelecida para outros métodos de imagem pelo BI-RADS. Contudo, lesões de baixa suspeição (4A) podem apresentar taxas mais altas de malignidade.