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.

Natural History of Serviceable Hearing During Active Surveillance of Nongrowing Sporadic Vestibular Schwannoma Supports Consideration of Initial Wait-and-Scan Management.

OBJECTIVE: The treatment paradigm of vestibular schwannoma (VS) focuses on preservation of neurologic function, with small tumors increasingly managed with active surveillance. Often, tumor size and hearing outcomes are poorly correlated. The aim of the current work was to describe the natural history of hearing among patients with nongrowing VS during observational management. STUDY DESIGN: Historical cohort study. PATIENTS: Adults with sporadic VS. INTERVENTION: Wait-and-scan management. MAIN OUTCOME MEASURE: Maintenance of serviceable hearing (SH) after diagnosis. RESULTS: Among 228 patients with nongrowing VS, 157 patients had SH at diagnosis. Rates of maintaining SH (95% CI; number still at risk) at 1, 3, and 5 years after diagnosis were 94% (89-98; 118), 81% (74-89; 65), and 78% (71-87; 42), respectively. Poorer hearing at diagnosis (hazard ratio [HR] per 10 dB hearing level increase in pure-tone average of 2.51, p < 0.001; HR per 10% decrease in word recognition score of 1.70, p = 0.001) was associated with increased likelihood of developing non-SH during observation. When controlling for baseline hearing status, tumors measuring 5 mm or greater in the internal auditory canal or with cerebellopontine angle extension were associated with significantly increased risk of developing non-SH (HR, 4.87; p = 0.03). At 5 years after diagnosis, 95% of patients with nongrowing intracanalicular VS measuring less than 5 mm maintained SH. CONCLUSIONS: Hearing worsens during periods of nongrowth in sporadic VS. Patients with small (<5 mm) intracanalicular tumors demonstrate robust maintenance of SH over time, reinforcing the consideration of initial observation in this patient subset.

Rationale for the Development of a Novel Clinical Grading Scale for Postoperative Facial Nerve Function: Results of a Multidisciplinary International Working Group.

OBJECTIVE: The objective of the current study was to present the results of an international working group survey identifying perceived limitations of existing facial nerve grading scales to inform the development of a novel grading scale for assessing early postoperative facial paralysis that incorporates regional scoring and is anchored in recovery prognosis and risk of associated complications. STUDY DESIGN: Survey. SETTING: A working group of 48 multidisciplinary clinicians with expertise in skull base, cerebellopontine angle, temporal bone, or parotid gland surgery. RESULTS: House-Brackmann grade is the most widely used system to assess facial nerve function among working group members (81%), although more than half (54%) agreed that the system they currently use does not adequately estimate the risk of associated complications, such as corneal injury, and confidence in interrater and intrarater reliability is generally low. Simplicity was ranked as the most important attribute of a novel postoperative facial nerve grading system to increase the likelihood of adoption, followed by reliability and accuracy. There was widespread consensus (91%) that the eye is the most critical facial region to focus on in the early postoperative setting. CONCLUSIONS: Members were invited to submit proposed grading systems in alignment with the objectives of the working group for subsequent validation. From these data, we plan to develop a simple, clinically anchored, and reproducible staging system with regional scoring for assessing early postoperative facial nerve function after surgery of the skull base, cerebellopontine angle, temporal bone, or parotid gland.

The Effect of Age on Facial Nerve Recovery After Vestibular Schwannoma Resection.

OBJECTIVE: The objective of this study is to assess the influence of age on facial nerve recovery after microsurgical resection of sporadic vestibular schwannoma. STUDY DESIGN: A historical cohort study was performed. SETTING: The study was performed at a tertiary referral center. PATIENTS: The studied cohort included patients with a House-Brackmann (HB) Grade III or worse in the immediate postoperative period. INTERVENTIONS: The studied intervention was microsurgical resection. MAIN OUTCOME MEASURES: The main outcome measure was complete recovery of facial nerve function to HB Grade I at least 12 months postoperatively. RESULTS: There were six patients with intracanalicular tumors and 100 with cerebellopontine angle (CPA) tumors eligible for study. Given the few patients with intracanalicular tumors, no further analysis was pursued in this subset. For patients with CPA tumors, a multivariable analysis of several patient and tumor characteristics demonstrated age at surgery (odds ratio for 10-year increase of 0.68; 95% confidence interval [CI], 0.47-0.98; p = 0.04) and immediate postoperative HB grade (odds ratio for one-grade increase of 0.27; 95% CI, 0.15-0.50; p < 0.001) to be jointly significantly associated with complete recovery to HB Grade I, indicating that the likelihood of complete facial nerve recovery was higher for younger patients and for those with better immediate postoperative HB grades. For example, the predicted probability of complete facial nerve recovery for a 30-year-old with immediate postoperative HB Grade III was 0.76 (or 76% when expressed as a percentage), whereas the predicted probability for a 50-year-old with immediate postoperative HB Grade V was only 0.10. CONCLUSIONS: After considering immediate postoperative HB grade, younger age at surgery was independently significantly associated with complete facial nerve recovery, which can assist in intraoperative decision-making regarding extent of resection and postoperative counseling.

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.

Optimizing Quality of Life and Minimizing Morbidity through Nasal Preservation in Endoscopic Skull Base Surgery: A Contemporary Review.

Introduction Endoscopic endonasal approaches (EEAs) are increasingly utilized for intracranial pathology. As opposed to sinonasal tumors, the nasal cavity is being used as a corridor to access these intracranial tumors but is not the site of primary surgical intent. Accordingly, there has been recent interest in preserving intranasal structures not directly involved by tumor and improving postoperative sinonasal quality of life (QOL). Objectives The aim of the study is to highlight recent advances in EEA techniques focused on improving sinonasal QOL including turbinate preservation, reducing the morbidity of reconstructive techniques, and the development of alternative minimally invasive EEA corridors. Methods The method of the study involves contemporary literature review and summary of implications for clinical practice. Results Nasoseptal flap (NSF) harvest is associated with significant morbidity including septal perforation, prolonged nasal crusting, and external nasal deformities. Various grafting and local rotational flaps have demonstrated the ability to significantly limit donor site morbidity. Free mucosal grafts have re-emerged as a reliable reconstructive option for sellar defects with an excellent sinonasal morbidity profile. Middle turbinate preservation is achievable in most EEA cases and has not been shown to cause postoperative obstructive sinusitis. Recently developed minimally invasive EEA techniques such as the superior ethmoidal approach have been described to better preserve intranasal structures while allowing intracranial access to resect skull base tumors and have shown promising sinonasal QOL results. Conclusion This contemporary review discusses balancing effective skull base reconstructive techniques with associated morbidity, the role of turbinate preservation in EEA, and the development of unique EEA techniques that allow for increased nasal structure preservation.

The precuneal interhemispheric, trans-tentorial corridor to the pineal region and brainstem, surgical anatomy, and case illustration.

BACKGROUND: The pineal region and dorsal midbrain are among the most challenging surgical targets. To approach lesions in this region that harbor a superior to inferior long axis, we describe the basic steps of the precuneal, interhemispheric, trans-tentorial approach and illustrate anatomical landmarks of this established, but not so popular, surgical trajectory. METHOD: To study the anatomical landmarks and safety of this approach, the neurovascular anatomy was studied on 22 sides of 11 formalin-fixed latex-injected anatomical specimens. A step-by-step dissection of the precuneal interhemispheric trans-tentorial approach and study of the key anatomical landmarks was performed. An illustrative clinical case of a pontomesencephalic cavernous malformation (CM) resected through this approach is also detailed. RESULTS: The mean distance from the transverse sinus to the most posterior cortical vein draining into the superior sagittal sinus was 6.4 cm. The mean distance from the calcarine sulcus to the most posterior cortical vein was 5.3 cm. Key steps of the dissection are as follows: craniotomy exposing the posterior aspect of the superior sagittal sinus (SSS), durotomy and gentle retraction of the SSS edge, dissection of the interhemispheric fissure, linear incision of the tentorium that extends anteriorly to the incisura and lateral reflection of the tentorium, and arachnoidal dissection and exposure of the cerebellomesencephalic fissure. CONCLUSION: The precuneal, interhemispheric, trans-tentorial approach affords excellent access to the falcotentorial junction, splenium, pineal region, quadrigeminal cistern, and dorsal pons once the cerebellomesencephalic fissure has been dissected.

Defining the Minimal Clinically Important Difference for Patients With Vestibular Schwannoma: Are all Quality-of-Life Scores Significant?

BACKGROUND: The diagnosis of vestibular schwannomas (VS) is associated with reduced patient quality of life (QOL). Minimal clinically important difference (MCID) was introduced as the lowest improvement in a patient-reported outcome (PRO) score discerned as significant by the patient. We formerly presented an MCID for the Penn Acoustic Neuroma QOL (PANQOL) battery based on cross-sectional data from 2 tertiary referral centers. OBJECTIVE: To validate the PANQOL MCID values using prospective data. METHODS: A prospective registry capturing QOL was queried, comprising patients treated at the authors' institution and Acoustic Neuroma Association members. Anchor- and distribution-based techniques were utilized to determine the MCID for domain and total scores. We only included anchors with Spearman's correlation coefficient larger than 0.3 in the MCID threshold calculations. Most domains had multiple anchors with which to estimate the MCID. RESULTS: A total of 1254 patients (mean age: 57.4 yr, 65% females) were analyzed. Anchor-based methods produced a span of MCID values (median, 25th-75th percentile) for each PANQOL domain and the total score: hearing (13.1, 13-16 points), balance (14, 14-19 points), pain (21, 20-28 points), face (25, 16-36 points), energy (16, 15-18 points), anxiety (16 [1 estimate]), general (13 [1 estimate]), and total (12.5, 10-15 points). CONCLUSION: Current findings corroborate our formerly shared experience using multi-institutional, cross-sectional information. These MCID thresholds can serve as a pertinent outcome when deciphering the clinical magnitude of VS QOL endpoints in cross-sectional and longitudinal studies.

Quality of Life Within the First 6 Months of Vestibular Schwannoma Diagnosis With Implications for Patient Counseling.

OBJECTIVE: To evaluate quality-of-life (QOL) in patients with sporadic vestibular schwannoma (VS) with particular focus on those recently diagnosed. STUDY DESIGN: Cross-sectional survey, Penn Acoustic Neuroma Quality of Life (PANQOL) instrument. SETTING: Acoustic Neuroma Association and a single tertiary referral center. PATIENTS: Patients with sporadic VS. MAIN OUTCOME MEASURES: Domain-specific and total PANQOL scores comparing treatment modalities after adjusting for baseline covariates of interest. RESULTS: Among all 1,288 respondents there were 229 (18%) who were recently diagnosed and had not yet selected a treatment modality, 303 (24%) who were observed, 185 (14%) who underwent radiosurgery alone, 507 (39%) who underwent microsurgery alone, and 64 (5%) who underwent radiosurgery and microsurgery. After adjusting for covariates of interest, total PANQOL scores were highest for the observation cohort (65; 95% CI 62-68), lowest for patients treated with multimodality therapy (56; 51-61) and those recently diagnosed (58; 55-62), and intermediate for those who received microsurgery alone (60; 58-62) and radiosurgery alone (61; 57-64) (global comparison, p = 0.001). When comparing groups that received single-modality therapy, there were no statistically significant differences in total PANQOL scores at short (0-5 yr), intermediate (6-10 yr), or long-term (≥11 yr) follow-up after adjusting for baseline covariates (all, p > 0.05). CONCLUSION: A new diagnosis of VS frequently imparts significant anxiety on the patient and leads to a temporary reduction in QOL. Notably, anxiety domain QOL scores among patients with newly diagnosed VS are poorer than patients who have observed their tumor for 6 months or longer. When feasible, an initial period of observation may afford patients valuable time to reconcile with the new diagnosis, to learn more about treatment options, and to discover whether there is tumor growth. In turn, this valuable information can be used to inform sound decision-making regarding tumor management.