Gompel, Jamie J. Van

Objective:To address variance in clinical care surrounding sporadic vestibular schwannoma, a modified Delphi study was performed to establish a general framework to approach vestibular schwannoma care. A multidisciplinary panel of experts was established with deliberate representation from key stakeholder societies. External validity of the final statements was assessed through an online survey of registered attendees of the 8th Quadrennial International Conference on Vestibular Schwannoma.Study Design:Modified Delphi method.Methods:The panel consisted of 16 vestibular schwannoma experts (8 neurotology and 8 neurosurgery) and included delegates representing the AAOHNSF, AANS/CNS tumor section, ISRS, and NASBS. The modified Delphi method encompassed a four-step process, comprised of one prevoting round to establish a list of focus areas and three subsequent voting rounds to successively refine individual statements and establish levels of consensus. Thresholds for achieving moderate consensus, at ≥67% agreement, and strong consensus, at ≥80% agreement, were determined a priori. All voting was performed anonymously via the Qualtrics online survey tool and full participation from all panel members was required before procession to the next voting round.Results:Through the Delphi process, 103 items were developed encompassing hearing preservation (N = 49), tumor control and imaging surveillance (N = 20), preferred treatment (N = 24), operative considerations (N = 4), and complications (N = 6). As a result of item refinement, moderate (4%) or strong (96%) consensus was achieved in all 103 final statements. Seventy-nine conference registrants participated in the online survey to assess external validity. Among these survey respondents, moderate (N = 21, 20%) or strong (N = 73, 71%) consensus was achieved in 94 of 103 (91%) statements, and no consensus was reached in 9 (9%). Of the four items with moderate consensus by the expert panel, one had moderate consensus by the conference participants and three had no consensus.Conclusion:This modified Delphi study on sporadic vestibular schwannoma codifies 100% consensus within a multidisciplinary expert panel and is further supported by 91% consensus among an external group of clinicians who regularly provide care for patients with vestibular schwannoma. These final 103 statements address clinically pragmatic items that have direct application to everyday patient care. This document is not intended to define standard of care or drive insurance reimbursement, but rather to provide a general framework to approach vestibular schwannoma care for providers and patients.

Background: Several materials are available for cranioplasty reconstruction and consensus regarding the ideal material is lacking. The goal of this study is to present surgical and patient-reported outcomes with PEEK versus Titanium alloplastic cranioplasty. Methods: A retrospective review of all patients who underwent alloplastic cranioplasty with PEEK or Titanium from 2010 to 2017 was conducted. Patient demographics and complications were abstracted and analyzed. Information regarding patient-reported outcomes was collected through a telephone survey. Results: A total of 72 patients (median age 55 years) who underwent 77 cranioplasties were identified (38% PEEK, n = 29; 62% Titanium, n = 48). Overall complication rates were similar between the PEEK (24%, n = 7) and Titanium groups (23%, n = 11), P = 0.902. Similarly, implant failure was similar between the 2 groups (7% in PEEK (n = 2), 13% in Titanium (n = 6), P = 0.703). History of radiation was associated with increased rate of infection in patients with Titanium mesh cranioplasty (38% in radiated patients (n = 3), 3% in nonradiated patients (n = 1), P = 0.012) but not PEEK implants (0% infection rate in radiated patients (n = 0), 15% in nonradiated patients (n = 4), P = 1.000). A total of 24 patients (33% response rate) participated in the telephone survey. All PEEK cranioplasty patients who responded to our survey (n = 13) reported good to excellent satisfaction, while 72% of our titanium mesh cohort (n = 8) described good or excellent satisfaction and 27% (n = 3) reported acceptable result. Conclusion: Cranial reconstruction is associated with high satisfaction among cranioplasty patients with PEEK or Titanium showing comparable complications, failure, and patient-reported satisfaction rates. Patients with history of radiotherapy demonstrated a higher infection rate when titanium mesh was used.

AbstractBackgroundMinimal information has been reported on the effect of distant and nodal metastases at the time of diagnosis on survival in patients with sinonasal adenocarcinoma (SNAC).MethodsThe Surveillance, Epidemiology, and End Results database was utilized to compare overall survival (OS) and disease‐specific survival (DSS).ResultsOf the 325 patients with SNAC identified, 5‐year and 10‐year OS for all included patients was 64% and 58%, respectively. On multivariate analysis, the presence of distant metastases (P < .0001), maxillary and frontal sinus primary tumors (P = .0042, P = .0006), and increasing age (P = .007) were risk factors for worsened DSS. The presence of regional spread to multiple cervical nodal basins (OS RR 3.26, P = .002; DSS RR 2.51, P = .013) and a single nodal basin (DSS RR 2.19, P = .046) was associated with worsened survival compared to no regional spread.ConclusionSurvival in SNAC was significantly worsened with increasing age, tumor site of origin, and distant metastatic disease.

OBJECTIVE The object of this study was to ascertain outcomes of cochlear implantation (CI) following stereotactic radiosurgery (SRS) for vestibular schwannoma (VS). METHODS The authors conducted a retrospective chart review of adult patients with VS treated with SRS who underwent CI between 1990 and 2019 at a single tertiary care referral center. Patient demographics, tumor features, treatment parameters, and pre- and postimplantation audiometric and clinical outcomes are presented. RESULTS Seventeen patients (18 ears) underwent SRS and ipsilateral CI during the study period. Thirteen patients (76%) had neurofibromatosis type 2 (NF2). Median age at SRS and CI were 44 and 48 years, respectively. Median time from SRS to CI was 60 days, but notably, 4 patients underwent SRS and CI within 1 day and 5 patients underwent CI more than 7 years after SRS. Median marginal dose was 13 Gy. Median treatment volume at the time of SRS was 1400 mm 3 (range 84–6080 mm 3 , n = 15 patients). Median post-CI PTA was 28 dB HL, improved from 101 dB HL preoperatively (p < 0.001). Overall, 11 patients (12 ears) exhibited open-set speech understanding. Sentence testing was performed at a median of 10 months (range 1–143 months) post-CI. The median AzBio sentence score for patients with open-set speech understanding was 76% (range 19%–95%, n = 10 ears). Two ears exhibited Hearing in Noise Test (HINT) sentence scores of 49% and 95%, respectively. Four patients achieved environmental sound awareness without open-set speech recognition. Two had no detectable auditory percepts. CONCLUSIONS Most patients who underwent CI following SRS for VS enjoyed access to sound at near-normal levels, with the majority achieving good open-set speech understanding. Implantation can be performed immediately following SRS or in a delayed fashion, depending on hearing status as well as other factors. This strategy may be applied to cases of sporadic or NF2-associated VS.

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Objective: Definitive treatment of sporadic vestibular schwannoma (VS) following documented growth is common practice at most centers in the United States. However, as a natural extension of this paradigm, very little evidence exists surrounding the natural history of growing tumors. The primary objective of the current work was to describe the natural history of sporadic VS following documentation of initial tumor growth. Study Design: Retrospective cohort study. Setting: Tertiary referral center. Patients: Patients diagnosed with sporadic VS between January 1, 2001 and December 31, 2015 who elected continued observation despite having volumetric growth ≥20% of original tumor volume on interval magnetic resonance imaging (MRI). Main Outcome Measure: Survival free of subsequent volumetric growth. Results: Of 361 patients undergoing observation with serial imaging during the study period, 85 patients met inclusion criteria at a median age of 66 years (interquartile ranges [IQR] 55–71). Within this cohort, 40 patients demonstrated subsequent volumetric growth at a median of 1.7 years (IQR 1.0–2.6) from the date of initial MRI that documented growth. The median volumetric growth was 43% (IQR 28–57), and the median growth rate was 0.026 cm3 per year (IQR 0.009–0.107). Survival free of subsequent volumetric growth rates (95% CI; number still at risk) at 1, 2, 3, 4, and 5 years were 93% (87–99; 75), 67% (58–79; 45), 54% (43–67; 29), 44% (33–59; 19), and 41% (30–56; 12), respectively. Conclusions: In a cohort exclusively comprised of sporadic VS with documented growth, over 40% of tumors demonstrated no subsequent volumetric growth after 5 years of continued MRI surveillance. These data challenge the supposition that once growth occurs, all tumors will exhibit sustained growth. Continued observation after documented growth of sporadic VS is a reasonable consideration in appropriately selected cases.

OBJECTIVE Cushing’s disease arises from functioning adrenocorticotropic hormone (ACTH)–secreting pituitary adenomas. These tumors can be very small and evade detection by MRI. Empty sella syndrome is a phenomenon by which an arachnoid outpouching of CSF into the sella leads to compression of the pituitary, likely due to intracranial hypertension (a common issue in Cushing’s disease), further leading to difficulty in visualizing the pituitary gland that may contribute to difficulty in finding a tumor on MRI, so-called MRI-negative Cushing’s disease. The authors sought to examine the association between empty sella syndrome and MRI-negative Cushing’s disease. METHODS A single-institution database of Cushing’s disease cases from 2000 to 2017 was reviewed, and 197 cases were included in the analysis. One hundred eighty patients had a tissue diagnosis of Cushing’s disease and 17 had remission with surgery, but no definitive tissue diagnosis was obtained. Macroadenomas (tumors > 1 cm) were excluded. The degree of empty sella syndrome was graded on the degree of CSF visualized in the sella on midline sagittal T1-weighted MRI. RESULTS Of the 197 cases identified, 40 (20%) presented with MRI-negative disease, and empty sella syndrome was present in 49 cases (25%). MRI-negative disease was found in 18 (37%) of 49 empty sella cases versus 22 (15%) of 148 cases without empty sella syndrome present. Empty sella syndrome was significantly associated with MRI-negative disease (OR 3.32, 95% CI 1.61–6.74, p = 0.0018). Decreased thickness of the pituitary gland was also associated with MRI-negative disease (mean thickness 5.6 vs 6.8 mm, p = 0.0002). CONCLUSIONS Empty sella syndrome is associated with an increased rate of MRI-negative Cushing’s disease. Pituitary compression causing a relative reduction in the volume of the pituitary for imaging is a plausible cause for not detecting the tumor mass with MRI.

OBJECTIVE Multiple endocrine neoplasia type 1 (MEN1) is a rare, autosomal-dominant tumor disorder characterized by the development of pituitary tumors and other endocrine neoplasms. Diagnosis is made clinically based on the development of 2 or more canonical lesions (parathyroid gland, anterior pituitary, and enteropancreatic tumors) or in family members of a patient with a clinical diagnosis of MEN1 and the occurrence of one of the MEN1-associated tumors. The goal of this study was to characterize pituitary tumors arising in the setting of MEN1 at a single institution. The probability of tumor progression and the likelihood of surgical intervention in patients with asymptomatic nonfunctional pituitary adenomas were also analyzed. METHODS A retrospective review of a prospectively maintained institutional database was performed for patients with MEN1 diagnosed from 1970 to 2017. Data included patient demographics, tumor characteristics, treatment strategies, and outcomes. RESULTS A review of the database identified 268 patients diagnosed with MEN1, of whom 158 (59%) were female. Among the 268 patients, 139 (51.8%) had pituitary adenomas. There was a higher prevalence in women than in men (65% vs 35%, p < 0.005). Functional adenomas (57%) were more common. Prolactin-secreting adenomas were the most common functional tumors. Macroadenomas were seen in 27% of patients and were more likely to be symptomatic and locally aggressive (p < 0.001). Forty-nine patients (35%) underwent transsphenoidal resection at some point during their disease course. In 52 patients who were initially observed with MEN1 asymptomatic nonfunctional adenomas, only 5 (10%) progressed to need surgery. In MEN1 patients, an initial parathyroid lesion is most likely followed in order by pituitary, pancreatic, adrenal, and, finally, rare carcinoid tumors. CONCLUSIONS Asymptomatic nonfunctional pituitary adenomas in patients with MEN1 may be followed safely with MRI. In this series, parathyroid tumors developed at the lowest median age of all cardinal tumors, and development of additional cardinal MEN1 lesions followed a predictable pattern. This pattern of disease progression could have significant implications for disease surveillance in clinical practice and may help to target clinical resources to the lesions most likely to develop next. This may aid with early detection and treatment and warrants further study.

Abstract Background Consensus in timing of radiotherapy is yet to be established in esthesioneuroblastoma (ENB). Objective This study was aimed to investigate if planned adjuvant radiotherapy improves tumor control after complete margin negative resection of low Hyams' grade (1 or 2) ENB. Methods A retrospective review of patients with pathologically confirmed negative margin resection of Kadish's stage B or C and Hyams' grade 1 and 2 ENBs was conducted. Seventeen patients meeting the criteria were divided into the following two groups for cohort study: (1) those who underwent planned immediate postoperative adjuvant radiotherapy (IR group) and (2) those who did not (delayed radiotherapy [DR] group). Results The IR group included nine patients (Kadish's stage B in one and stage C in eight; Hyams' grade 1 in two and grade 2 in seven). Mean follow-up was 140.8 months. Seven patients (78%) had disease progression (DP) at a median of 88 months (four with cervical lymph node metastasis [CLNM], one with distant metastasis, and two with both local recurrence and CLNM). One patient experienced frontal lobe abscess. The DR group included eight patients (Kadish's stage B in six and stage C in two; all Hyams' grade 2). Mean follow-up was 123.3 months. Four (50%) patients who developed DP (all local recurrence) were salvaged with surgery and adjuvant radiotherapy at a median of 37.5 months. There was no statistically significant difference in DP rate (p = 0.23), time to DP (p = 0.26), or the local tumor control rate (p = 0.23). Conclusion In our limited cohort, immediate postoperative radiotherapy did not demonstrate superiority in tumor control, although risk of radiotherapy toxicity appears low.