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.

HPV-Related Multiphenotypic Sinonasal Carcinoma: A Clinicoradiological Series of 3 Cases With Full Endoscopic Surgical Outcome.

Context: Human papillomavirus (HPV)-related multiphenotypic sinonasal carcinoma (HMSC), formerly known as HPV-related carcinoma with adenoid cystic like features, is a rare tumor subtype with unusual correlation between radiological, histopathological, and surgical findings. The shared histological characteristics with other sinonasal tumors make the diagnosis challenging. Optimal surgical and oncological treatments for this rare condition remains to be clearly defined. Methods: The objective of the study was to describe the unique characteristics and endoscopic surgical treatment of this rare tumor. In this retrospective case series, all patients with an HMSC diagnosis treated in our tertiary center were selected. Results: Three HMSC cases were identified, including 2 male and 1 female patients. All cases originated from the posterior nasal cavity. One case presented with a tumor of 8.9 cm × 6.4 cm × 8.7 cm, which is the largest tumor volume described to date. All patients received exclusively endoscopic surgical treatment, followed by adjuvant radiation therapy. No patient showed clinical or radiological sign of disease recurrence, or regional or distant metastasis, with a follow-up ranging from 9 months to 4 years. In 2 cases, initial diagnoses incorrectly suggested adenoid cystic or basaloid squamous cell carcinoma. HPV-DNA testing confirmed the presence of HPV in all cases, with identification of strains 16 and 18. Conclusion and Relevance: HMSC represents a newly identified diagnosis that constitutes a significant challenge for both clinicians and pathologists. It is crucial to acknowledge its indolent clinical course and the apparent contradiction between aggressive radiological features and the noninvasive nature of surgical findings. Skull base surgeons should be aware that, despite these complexities, endoscopic treatment is achievable in the majority of cases. This understanding is essential for the effective management of HMSC.

A comprehensive review of the vertebral artery anatomy.

BACKGROUND: The vertebral arteries (VA) play a critical role by supplying nearly one-third of the brain's blood flow, predominantly contributing to the posterior circulation. These arteries may need to be exposed in a various cranial and cervical procedures and offers access to investigate or treat vascular lesions by endovascular means related to the posterior circulation. Given its complex anatomy, which is subject to numerous variations, and its role in supplying vital brain regions, a thorough understanding of the VA's anatomy is paramount for any related procedure. OBJECTIVE: To provide a comprehensive overview of vertebral artery anatomy and its relevance in contemporary clinical practice. METHODS: Dissection of the entire vertebral artery length using cadaveric specimen, combined with a comprehensive literature review. RESULTS: The vertebral artery can be subdivided into four segments. Each of these segments has its own unique topographic anatomy with its variations, anastomoses, and significance in surgery. CONCLUSION: As surgical and endovascular techniques continue to evolve with technological improvements, we are now more equipped than ever to manage complex lesions involving the VA. However, with its increasingly complexity comes the necessity for a deeper and more comprehensive understanding of the VA. Possessing the detailed knowledge of the VA is vital for the successful execution of any procedure involving it.

Development of an online calculator for the prediction of seizure freedom following pediatric hemispherectomy using the Hemispherectomy Outcome Prediction Scale (HOPS).

OBJECTIVES: Although hemispheric surgeries are among the most effective procedures for drug-resistant epilepsy (DRE) in the pediatric population, there is a large variability in seizure outcomes at the group level. A recently developed HOPS score provides individualized estimation of likelihood of seizure freedom to complement clinical judgement. The objective of this study was to develop a freely accessible online calculator that accurately predicts the probability of seizure freedom for any patient at 1-, 2-, and 5-years post-hemispherectomy. METHODS: Retrospective data of all pediatric patients with DRE and seizure outcome data from the original Hemispherectomy Outcome Prediction Scale (HOPS) study were included. The primary outcome of interest was time-to-seizure recurrence. A multivariate Cox proportional-hazards regression model was developed to predict the likelihood of post-hemispheric surgery seizure freedom at three time points (1-, 2- and 5- years) based on a combination of variables identified by clinical judgment and inferential statistics predictive of the primary outcome. The final model from this study was encoded in a publicly accessible online calculator on the International Network for Epilepsy Surgery and Treatment (iNEST) website (https://hops-calculator.com/). RESULTS: The selected variables for inclusion in the final model included the five original HOPS variables (age at seizure onset, etiologic substrate, seizure semiology, prior non-hemispheric resective surgery, and contralateral fluorodeoxyglucose-positron emission tomography [FDG-PET] hypometabolism) and three additional variables (age at surgery, history of infantile spasms, and magnetic resonance imaging [MRI] lesion). Predictors of shorter time-to-seizure recurrence included younger age at seizure onset, prior resective surgery, generalized seizure semiology, FDG-PET hypometabolism contralateral to the side of surgery, contralateral MRI lesion, non-lesional MRI, non-stroke etiologies, and a history of infantile spasms. The area under the curve (AUC) of the final model was 73.0%. SIGNIFICANCE: Online calculators are useful, cost-free tools that can assist physicians in risk estimation and inform joint decision-making processes with patients and families, potentially leading to greater satisfaction. Although the HOPS data was validated in the original analysis, the authors encourage external validation of this new calculator.

Multicenter Survival Analysis and Application of an Olfactory Neuroblastoma Staging Modification Incorporating Hyams Grade.

Importance: Current olfactory neuroblastoma (ONB) staging systems inadequately delineate locally advanced tumors, do not incorporate tumor grade, and poorly estimate survival and recurrence. Objective: The primary aims of this study were to (1) examine the clinical covariates associated with survival and recurrence of ONB in a modern-era multicenter cohort and (2) incorporate Hyams tumor grade into existing staging systems to assess its ability to estimate survival and recurrence. Design, Setting, and Participants: This retrospective, multicenter, case-control study included patients with ONB who underwent treatment between January 1, 2005, and December 31, 2021, at 9 North American academic medical centers. Intervention: Standard-of-care ONB treatment. Main Outcome and Measures: The main outcomes were overall survival (OS), disease-free survival (DFS), and disease-specific survival (DSS) as C statistics for model prediction. Results: A total of 256 patients with ONB (mean [SD] age, 52.0 [15.6] years; 115 female [44.9%]; 141 male [55.1%]) were included. The 5-year rate for OS was 83.5% (95% CI, 78.3%-89.1%); for DFS, 70.8% (95% CI, 64.3%-78.0%); and for DSS, 94.1% (95% CI, 90.5%-97.8%). On multivariable analysis, age, American Joint Committee on Cancer (AJCC) stage, involvement of bilateral maxillary sinuses, and positive margins were associated with OS. Only AJCC stage was associated with DFS. Only N stage was associated with DSS. When assessing the ability of staging systems to estimate OS, the best-performing model was the novel modification of the Dulguerov system (C statistic, 0.66; 95% CI, 0.59-0.76), and the Kadish system performed most poorly (C statistic, 0.57; 95% CI, 0.50-0.63). Regarding estimation of DFS, the modified Kadish system performed most poorly (C statistic, 0.55; 95% CI, 0.51-0.66), while the novel modification of the AJCC system performed the best (C statistic, 0.70; 95% CI, 0.66-0.80). Regarding estimation of DSS, the modified Kadish system was the best-performing model (C statistic, 0.79; 95% CI, 0.70-0.94), and the unmodified Kadish performed the worst (C statistic, 0.56; 95% CI, 0.51-0.68). The ability for novel ONB staging systems to estimate disease progression across stages was also assessed. In the novel Kadish staging system, patients with stage VI disease were approximately 7 times as likely to experience disease progression as patients with stage I disease (hazard ratio [HR], 6.84; 95% CI, 1.60-29.20). Results were similar for the novel modified Kadish system (HR, 8.99; 95% CI, 1.62-49.85) and the novel Dulguerov system (HR, 6.86; 95% CI, 2.74-17.18). Conclusions and Relevance: The study findings indicate that 5-year OS for ONB is favorable and that incorporation of Hyams grade into traditional ONB staging systems is associated with improved estimation of disease progression.

Hearing Rehabilitation in Patients With Neurofibromatosis Type 2: The Quebec's Experience With Auditory Implants.

OBJECTIVE: To review a single-center experience with hearing rehabilitation in patients with neurofibromatosis type 2 (NF2) and to describe the auditory outcomes of cochlear implants (CIs) and auditory brainstem implants (ABI) in this population. STUDY DESIGN: Retrospective case series. SETTING: Tertiary referral center. PATIENTS: Twelve adult patients with NF2 who received a CI (n = 10) and/or an ABI (n = 5) between 2000 and 2021. INTERVENTIONS: Insertion of a CI and/or an ABI in NF2 patients with bilateral vestibular schwannomas (VSs). MAIN OUTCOME MEASURES: Patients' demographic data, treatment history, hearing evolution, hearing rehabilitation methods, implant details, and auditory outcomes after implantation. RESULTS: Among those who received a CI, five patients had a stable untreated VS, one patient underwent a cochlear nerve preserving surgery, and four patients received radiotherapy treatments. Six patients became regular users of their device. The median open-set sentence recognition scores were as follows: 0.5% preoperatively, 60% at 1 year postoperatively, and 80% on the most recent audiological evaluation. All patients with an ABI were implanted concomitantly with VS surgical excision, and three of them also received radiotherapy treatments. The median open-set sentence recognition scores were as follows: 4% preoperatively, 26% at 1 year postoperatively, and 0% on the most recent evaluation. Three patients became regular ABI users. CONCLUSION: Despite major technological advances, auditory outcomes with ABIs remain deceiving. Considering the overall improvement in postoperative auditory performances provided by CIs compared with ABIs, cochlear implantation standouts as a primary mean of hearing rehabilitation in NF2 patients.

SARS-CoV-2 Infection, A Risk Factor for Pituitary Apoplexy? A Case Series and Literature Review.

Introduction: Pituitary apoplexy (PA) is a rare phenomenon, characterized by a hemorrhagic or ischemic event of the pituitary gland, most often in association with a pituitary lesion. Severe acute respiratory syndrome coronavirus-2 (SARS-CoV-2) is the strain of virus responsible for the internationally recognized global pandemic COVID-19. Multiple clinical manifestations associated with this virus have been described, ranging from asymptomatic, mild flu symptoms to acute respiratory distress syndrome, end-organ failure leading to death. Cases of patients with concomitant COVID-19 infections and PA are being further recognized in the literature, but the causal association between the 2 entities remains speculative. Objectives: The objectives of this case series are 3-fold: to describe additional cases of patients with concomitant COVID-19 infection and PA (1), to review the current evidence regarding this potential complication associated with a COVID-19 infection (2), and to discuss physiopathological hypotheses, treatments, and prognoses of this newly recognized association (3). Method: We conducted an electronic chart review of patients treated for PA with concomitant COVID-19 infection from March 2020 to December 2021. A literature review was performed using MEDLINE, Web of Science, and Embase databases to identify other cases of COVID-19-associated PA. Results: From March 2020 to December 2021, 3 patients presented to our center with PA following a symptomatic COVID-19 infection. Two of these patients developed PA symptoms days following the viral infection, whereas the third patient developed PA after a 2-month period. The 2 first patients were managed surgically because of persistent visual symptoms. Results from our literature review yielded 12 other cases of COVID-19-associated PAs. Conclusions: The association between COVID-19 infection and PA has been increasingly reported in the literature. With the addition of the 3 cases described in our article, a total of 15 cases have been published. Many contributing mechanisms may lead to PA following COVID-19 infection. Coagulopathy is probable major contributing cause responsible for hemorrhage or infarction of the pituitary gland. Our case series provides further arguments that PA may be a direct manifestation of a COVID-19 infection.

Metastatic Seeding From a Gastrointestinal Neoplasia in a Pituitary Adenoma: A Case Report and Literature Review.

Pituitary composite tumors consisting of metastasis within an adenoma are rare and aggressive entities. We present a case of esophageal adenocarcinoma metastasis at a prolactinoma presenting in a unique fashion and highlight how this case could contribute to a better understanding and early recognition of this condition. The patient was a 65-year-old male who presented with partial palsy of the third and sixth cranial nerves. He had a history of treated esophageal adenocarcinoma. He also had a known small parasellar lesion, with an elevated prolactin. Investigations showed a rapid progression of the parasellar lesion and normalization of prolactin. Partial surgical resection was performed, and pathology confirmed metastasis of the known digestive tract neoplasia. Although extremely rare, dual pathology of pituitary metastasis within adenomas should be considered in the differential diagnosis of sellar masses. Atypical behavior of benign adenomas, including rapid growth, spontaneous normalization of prolactin, or progression despite medical treatment should prompt medical teams to reconsider their diagnosis.

Radiographic predictors of occult intracranial involvement in olfactory neuroblastoma patients.

BACKGROUND: Traditional management of olfactory neuroblastoma (ONB) includes margin-negative resection with removal of cribriform plate, dura, and olfactory bulb, regardless of intracranial disease. This approach may be overtreating certain patients. Our investigation examines risk factors associated with occult intracranial disease to optimize therapeutic outcomes. METHODS: This retrospective, multi-institutional cohort study examined clinical covariates associated with occult intracranial involvement. Patient demographics, staging, Hyam's grade, and pathologic involvement of dura, olfactory bulb/tract, and brain were collected. Diagnostic imaging was reviewed. Positive and negative predictive value (NPV) were estimated along with effect size estimates. Cox hazard regression examined associations with overall survival (OS) and disease-free survival (DFS). RESULTS: A total of 224 subjects with new diagnoses of ONB (2005-2021) were identified. Skull base bone involvement on computed tomography (CT) had the highest NPV for pathologic dura (88.0%), olfactory bulb (88%), and brain involvement (97.3%). Hyam's grade category was significantly associated with dural involvement (φC  = 0.26; 95% confidence interval [CI]: 0.16, 0.42). Subjects without radiologic skull base involvement (n = 66) had pathologic positivity of 12.1%. Within this subgroup, Hyam's grade was clinically significant for dural positivity (φ = 0.34; 95% CI: -0.12, 0.71) with 28.6% involvement in high grade tumors. Neither clinical nor pathologic positivity of intracranial structures were associated with significantly different OS or DFS. CONCLUSIONS: Both CT and magnetic resonance imaging (MRI) had reasonably good NPV for involvement of dura and olfactory bulb. Higher Hyam's grade was associated with dural involvement. Patients with low-grade tumors not involving the skull base may be suitable for avoiding skull base resection; however, further investigation is warranted.

The Evolution of Endoscopic Endonasal Approach for Olfactory Groove Meningiomas.

BACKGROUND: There is controversy over the choice of optimal approach for olfactory groove meningiomas (OGM). The endoscopic endonasal approach (EEA) is one of the most recently described corridors, which provides direct access to the tumor base. OBJECTIVE: To detail the evolution of the endoscopic endonasal technique over the past 2 decades and demonstrate the impact of this evolution on patient outcomes. METHODS: This is a retrospective study based on a prospectively maintained database of consecutive cases of OGM operated on at our institution via EEA. For analysis, the whole cohort has been divided into 3 equal "eras" according to the time period during which the surgery happened, creating early, middle, and contemporary groups. RESULTS: Seventy-five patients were included, 25 in each group. The rate of postoperative cerebrospinal fluid leak significantly decreased over time (28%, 32%, and 8% in the early, middle, and contemporary groups, respectively, P = .020). The rate of postoperative encephalomalacia was significantly lower in more recent groups (24%, 16%, and 0% in the early, middle, and contemporary groups, respectively, P = .029), as was the time to resolution of postoperative brain edema (103.9 months, 87.3 months, and 16.8 months in the early, middle, and contemporary groups, respectively, P = .020). CONCLUSION: The endoscopic endonasal approach for OGM significantly evolved over time, achieving lower cerebrospinal fluid leak rates while providing a high rate of Simpson grade 1 resection. Technical improvements minimized the frontal lobe impact. Current data support EEA as a safe and effective corridor to treat OGM.

How I do it: cerebellopontine angle epidermoid cyst removal with endoscopic assistance.

BACKGROUND: Epidermoid cyst in the cerebellopontine angle (CPA) can involve numerous critical structures. Endoscopic assistance following microscopic removal via a retrosigmoid approach can expand the surgical corridor, allowing for more complete resection. METHOD: We describe in a stepwise fashion the surgical steps for the microscopic removal of an epidermoid cyst of the CPA with endoscopic assistance. CONCLUSION: Endoscopically assisted microscopic removal for CPA epidermoid cysts provides wide access to the CPA and its adjacent structures and shows to be an effective option in selected cases.

Risk Factors in a Pediatric Population for Postoperative Intracranial Infection Following Endoscopic Endonasal Skull Base Surgery and the Role of Antibiotic Prophylaxis.

BACKGROUND: Endoscopic endonasal skull base surgery (EESBS) is performed through a non-sterile corridor. Intracranial infection rates in a pediatric population have not been extensively studied and the exact effect of antibiotic prophylaxis on rates of infection after EESBS in pediatric patients remains unclear. OBJECTIVE: The purpose of our study is to investigate risk factors for postoperative intracranial infection during EESBS in a pediatric population and help elucidate the role of antibiotic prophylaxis. METHODS: We conducted a retrospective chart review of 99 consecutive patients under the age of 18 who underwent EESBS at our institution from 2013 to 2021. Centers for Disease Control and Prevention/National Healthcare Safety Network criteria for diagnosis of meningitis were used to identify postoperative intracranial infections. RESULTS: The average age was 12.3 years (range 1.6-18) with 66 male patients and 33 female patients. 49 patients had an intraoperative cerebrospinal fluid (CSF) leak, of which 4 had a postoperative CSF leak. We identified 3 postoperative intracranial infections (3%), which were all meningitis cases. The infection rate was 6% (3/49) among those with intraoperative CSF leaks. All patients with meningitis had a postoperative CSF leak. All infections were transclival approaches (2 chordoma and 1 neurenteric cyst). CONCLUSION: This investigation represents one of the largest pediatric endoscopic skull base surgery cohorts. EESBS is safe to perform in pediatric populations, but transclival approaches and postoperative CSF leaks are risk factors for postoperative meningitis.

Management strategies in clival and craniovertebral junction chordomas: a 29-year experience.

OBJECTIVE: Chordomas represent one of the most challenging subsets of skull base and craniovertebral junction (CVJ) tumors to treat. Despite extensive resection followed by proton-beam radiation therapy, the recurrence rate remains high, highlighting the importance of developing efficient treatment strategies. In this study, the authors present their experience in treating clival and CVJ chordomas over a 29-year period. METHODS: The authors conducted a retrospective study of clival and CVJ chordomas that were surgically treated at their institution from 1991 to 2020. This study focuses on three aspects of the management of these tumors: the factors influencing the extent of resection (EOR), the predictors of survival, and the outcomes of the endoscopic endonasal approaches (EEAs) compared with open approaches (OAs). RESULTS: A total of 265 surgical procedures were performed in 210 patients, including 123 OAs (46.4%) and 142 EEAs (53.6%). Tumors that had an intradural extension (p = 0.03), brainstem contact (p = 0.005), cavernous sinus extension (p = 0.004), major artery encasement (p = 0.01), petrous apex extension (p = 0.003), or high volume (p = 0.0003) were significantly associated with a lower EOR. The 5-year progression-free survival (PFS) and overall survival (OS) rates were 52.1% and 75.1%, respectively. Gross-total resection and Ki-67 labeling index < 6% were considered to be independent prognostic factors of longer PFS (p = 0.0005 and p = 0.003, respectively) and OS (p = 0.02 and p = 0.03, respectively). Postoperative radiation therapy correlated independently with a longer PFS (p = 0.006). Previous surgical treatment was associated with a lower EOR (p = 0.01) and a higher rate of CSF leakage after EEAs (p = 0.02) but did not have significantly lower PFS and OS compared with primary surgery. Previously radiation therapy correlated with a worse outcome, with lower PFS and OS (p = 0.001 and p = 0.007, respectively). EEAs were more frequently used in patients with upper and middle clival tumors (p = 0.002 and p < 0.0001, respectively), had a better rate of EOR (p = 0.003), and had a lower risk of de novo neurological deficit (p < 0.0001) compared with OAs. The overall rate of postoperative CSF leakage after EEAs was 14.8%. CONCLUSIONS: This large study showed that gross-total resection should be attempted in a multidisciplinary skull base center before providing radiation therapy. EEAs should be considered as the gold-standard approach for upper/middle clival lesions based on the satisfactory surgical outcome, but OAs remain important tools for large complex chordomas.

Endoscopic Endonasal Anterior Clinoidectomy: Volumetric Assessment and Feasibility.

BACKGROUND: Transcranial removal of the anterior clinoid process (ACP) provides access to the clinoidal segment of the internal carotid artery as well as superolateral decompression of the optic canal. Endoscopic endonasal approaches (EEAs) can access the entire medial and inferior portions of the optic canal, but no data exist to support what proportion of the anterior clinoid could be safely resected via an EEA. METHODS: A cadaveric anatomical study was performed with removal of the ACP in 3 major steps in order of difficulty and risk. At each step, the removal was stopped when no more bone of the ACP could be seen without traction on neural structures. After each step, a CT scan was performed to allow volumetric measurement of the remaining ACP. RESULTS: Twenty ACPs in 10 cadaveric heads were removed to various degrees using the described stepwise technique. The mean portion of the ACP resected by each step was 21%, 46%, and 27%, respectively. Cumulated ACP removal at the end of step 3 was 94%, with complete removal achieved in 35% of the specimens. CONCLUSIONS: Using the safe route above the optic canal, removal of 21% of the ACP can be achieved via EEA. Although substantially more of the ACP can be drilled by accessing the optic strut, the benefits of pursuing additional removal must be weighed against the significant risks of drilling in this narrow corridor bordered by the internal carotid artery, the third cranial nerve, and the optic nerve.

Potential Benefit of Add-on Δ9-Tetrahydrocannabinol in Pediatric Drug-Resistant Epilepsy: A Case Series.

We present five cases of pediatric drug-resistant epilepsy (DRE) that failed management using high cannabidiol (CBD) doses, but had significant reduction in seizure frequency with reintroduction or increasing doses of tetrahydrocannabinol (THC). There is growing evidence supporting the use of whole-plant CBD-rich extracts (containing THC and other cannabinoids) in the treatment of pediatric DRE. Based on our experiences and reports in the literature, we propose that, in patients who fail management with an initial trial of high-dose CBD-focused therapy, there may be a role for add-on THC-focused formulations.

Superparamagnetic iron oxide nanoparticles-based detection of neuronal activity.

Precise detection of brain regions harboring heightened electrical activity plays a central role in the understanding and treatment of diseases such as epilepsy. Superparamagnetic iron oxide nanoparticles (SPIONs) react to magnetic fields by aggregating and represent interesting candidates as new sensors for neuronal magnetic activity. We hypothesized that SPIONs in aqueous solution close to active brain tissue would aggregate proportionally to neuronal activity. We tested this hypothesis using an in vitro model of rat brain slice with different levels of activity. Aggregation was assessed with dynamic light scattering (DLS) and magnetic resonance imaging (MRI). We found that increasing brain slice activity was associated with higher levels of aggregation as measured by DLS and MRI, suggesting that the magnetic fields from neuronal tissue could induce aggregation in nearby SPIONs in solution. MRI signal change induced by SPIONs aggregation could serve as a powerful new tool for detection of brain electrical activity.

Comparison of the real-world effectiveness of vertical versus lateral functional hemispherotomy techniques for pediatric drug-resistant epilepsy: A post hoc analysis of the HOPS study.

OBJECTIVE: This study was undertaken to determine whether the vertical parasagittal approach or the lateral peri-insular/peri-Sylvian approach to hemispheric surgery is the superior technique in achieving long-term seizure freedom. METHODS: We conducted a post hoc subgroup analysis of the HOPS (Hemispheric Surgery Outcome Prediction Scale) study, an international, multicenter, retrospective cohort study that identified predictors of seizure freedom through logistic regression modeling. Only patients undergoing vertical parasagittal, lateral peri-insular/peri-Sylvian, or lateral trans-Sylvian hemispherotomy were included in this post hoc analysis. Differences in seizure freedom rates were assessed using a time-to-event method and calculated using the Kaplan-Meier survival method. RESULTS: Data for 672 participants across 23 centers were collected on the specific hemispherotomy approach. Of these, 72 (10.7%) underwent vertical parasagittal hemispherotomy and 600 (89.3%) underwent lateral peri-insular/peri-Sylvian or trans-Sylvian hemispherotomy. Seizure freedom was obtained in 62.4% (95% confidence interval [CI] = 53.5%-70.2%) of the entire cohort at 10-year follow-up. Seizure freedom was 88.8% (95% CI = 78.9%-94.3%) at 1-year follow-up and persisted at 85.5% (95% CI = 74.7%-92.0%) across 5- and 10-year follow-up in the vertical subgroup. In contrast, seizure freedom decreased from 89.2% (95% CI = 86.3%-91.5%) at 1-year to 72.1% (95% CI = 66.9%-76.7%) at 5-year to 57.2% (95% CI = 46.6%-66.4%) at 10-year follow-up for the lateral subgroup. Log-rank test found that vertical hemispherotomy was associated with durable seizure-free progression compared to the lateral approach (p = .01). Patients undergoing the lateral hemispherotomy technique had a shorter time-to-seizure recurrence (hazard ratio = 2.56, 95% CI = 1.08-6.04, p = .03) and increased seizure recurrence odds (odds ratio = 3.67, 95% CI = 1.05-12.86, p = .04) compared to those undergoing the vertical hemispherotomy technique. SIGNIFICANCE: This pilot study demonstrated more durable seizure freedom of the vertical technique compared to lateral hemispherotomy techniques. Further studies, such as prospective expertise-based observational studies or a randomized clinical trial, are required to determine whether a vertical approach to hemispheric surgery provides superior long-term seizure outcomes.

Intracranial Meningiomas Decrease in Volume on Magnetic Resonance Imaging After Discontinuing Progestin.

BACKGROUND: The behavior of meningiomas under influence of progestin therapy remains unclear. OBJECTIVE: To investigate the relationship between growth kinetics of intracranial meningiomas and usage of the progestin cyproterone acetate (PCA). METHODS: This study prospectively followed 108 women with 262 intracranial meningiomas and documented PCA use. A per-meningioma analysis was conducted. Changes in meningioma volumes over time, and meningioma growth velocities, were measured on magnetic resonance imaging (MRI) after stopping PCA treatment. RESULTS: Mean follow-up time was 30 (standard deviation [SD] 29) mo. Ten (4%) meningiomas were treated surgically at presentation. The other 252 meningiomas were followed after stopping PCA treatment. Overall, followed meningiomas decreased their volumes by 33% on average (SD 28%). A total of 188 (72%) meningiomas decreased, 51 (20%) meningiomas remained stable, and 13 (4%) increased in volume of which 3 (1%) were surgically treated because of radiological progression during follow-up after PCA withdrawal. In total, 239 of 262 (91%) meningiomas regressed or stabilized during follow-up. Subgroup analysis in 7 women with 19 meningiomas with follow-up before and after PCA withdrawal demonstrated that meningioma growth velocity changed statistically significantly (P = .02). Meningiomas grew (average velocity of 0.25 mm3/day) while patients were using PCA and shrank (average velocity of -0.54 mm3/day) after discontinuation of PCA. CONCLUSION: Ninety-one percent of intracranial meningiomas in female patients with long-term PCA use decrease or stabilize on MRI after stopping PCA treatment. Meningioma growth kinetics change significantly from growth during PCA usage to shrinkage after PCA withdrawal.

Tumor Growth Rate as a New Predictor of Progression-Free Survival After Chordoma Surgery.

BACKGROUND: Currently, different postoperative predictors of chordoma recurrence have been identified. Tumor growth rate (TGR) is an image-based calculation that provides quantitative information of tumor's volume changing over time and has been shown to predict progression-free survival (PFS) in other tumor types. OBJECTIVE: To explore the usefulness of TGR as a new preoperative radiological marker for chordoma recurrence. METHODS: A retrospective single-institution study was carried out including patients reflecting these criteria: confirmed diagnosis of chordoma on pathological analysis, no history of previous radiation, and at least 2 preoperative thin-slice magnetic resonance images available to measure TGR. TGR was calculated for all patients, showing the percentage change in tumor size over 1 mo. RESULTS: A total of 32 patients were retained for analysis. Patients with a TGR ≥ 10.12%/m had a statistically significantly lower mean PFS (P < .0001). TGR ≥ 10.12%/m (odds ratio = 26, P = .001) was observed more frequently in recurrent chordoma. In a subgroup analysis, we found that the association of Ki-67 labeling index ≥ 6% and TGR ≥ 10.12%/m was correlated with recurrence (P = .0008). CONCLUSION: TGR may be considered as a preoperative radiological indicator of tumor proliferation and seems to preoperatively identify more aggressive tumors with a higher tendency to recur. Our findings suggest that the therapeutic strategy and clinical-radiological follow-up of patients with chordoma can be adapted also according to this new parameter.