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Objective To evaluate the long-term changes in sporadic vestibular schwannoma (VS) disease-specific quality-of-life (QOL) outcomes. Study Design Prospective longitudinal study using the Penn Acoustic Neuroma Quality of Life (PANQOL) Scale. Setting National survey. Patients Patients with sporadic VS who completed a baseline survey before treatment and at least one follow-up survey recruited through the authors’ center and through the Acoustic Neuroma Association. Interventions Observation, microsurgery, radiosurgery. Main Outcome Measures Changes in PANQOL scores from baseline to most recent survey. Results Among 445 eligible patients the mean duration of follow-up was 4.4 (SD, 2.3) years, including 122, 218, and 105 in the observation, microsurgery, and radiosurgery groups, respectively. Patients managed with observation (p = 0.03) or microsurgery (p < 0.001) demonstrated improvement in anxiety scores. Changes in facial function scores differed significantly by management group (p = 0.01), with patients undergoing microsurgery demonstrating a mean decline of 10 points in facial function scores compared with mean declines of 3 for those managed with observation or radiosurgery. Hearing loss scores decreased similarly over time for all three groups (p = 0.3). There were minimal changes in total PANQOL scores over time across all management groups (p = 0.5). Conclusions Long-term changes in total QOL among VS management groups are not significantly different. Microsurgery may continue to confer an advantage regarding improvement in anxiety postoperatively, but with a greater decline in facial function when compared to observation or radiosurgery. Long-term declines in hearing loss scores were not statistically significantly different among groups.

AbstractIntroductionThe International Hidradenitis Suppurativa Severity Score System (IHS4) is a validated tool that measures inflammatory lesions, including draining tunnels, in hidradenitis suppurativa (HS).ObjectiveTo evaluate secukinumab efficacy using IHS4 in patients with moderate to severe HS.MethodsData from the SUNSHINE and SUNRISE trials, which assessed subcutaneous secukinumab 300 mg every 2 (SECQ2W) and 4 (SECQ4W) weeks in adults with moderate to severe HS, were analyzed. Assessments included changes from baseline in IHS4 and severity classification up to Week 52; IHS4‐55, IHS4‐75, IHS4‐90 responses (55%, 75% and 90% reduction in IHS4) and concordance between IHS4‐55 and HS clinical response (HiSCR), at Weeks 16 and 52.ResultsIn total, 1084 patients (SECQ2W = 361; SECQ4W = 360; placebo = 363) were analyzed. At Week 16, SECQ2W and SECQ4W demonstrated a numerically higher reduction in IHS4 from baseline versus placebo (adjusted mean [95% CI]: −10.80 [−12.30 to −9.30] and −9.46 [−10.96 to −7.96] vs. −4.92 [−6.43 to −3.41]); the reduction was maintained until Week 52 in both dose regimens. A greater proportion of patients achieved IHS4‐55 with SECQ2W (43.4%) and SECQ4W (39.5%) versus placebo (31.5%) at Week 16, with further improvement at Week 52. Similar trends were observed for IHS4‐75 and IHS4‐90 responses. While no patients had mild disease based on IHS4 (80.7% had severe and 19.3% had moderate HS) at baseline, a greater proportion of patients were categorized as having mild disease at Week 16 in the SECQ2W (25.9%) and SECQ4W (24.0%) groups versus placebo (16.4%); this trend continued up to Week 52 in both dose regimens. Strong concordance (>85%) was observed between IHS4‐55 and HiSCR.ConclusionsBoth SECQ2W and SECQ4W demonstrated efficacy in improving treatment response as measured by IHS4 and reducing disease severity versus placebo at Week 16 and these improvements were sustained through Week 52. These findings support that the dynamic and dichotomous IHS4 can efficiently detect treatment response changes in clinical trial settings.Clinical Trials RegistrationSUNSHINE (NCT03713619); SUNRISE (NCT03713632).

BACKGROUND: Defining qualitative grades of echocardiographic metrics of right heart chamber size and function is critical for screening, clinical assessment, and measurement of therapeutic response in individuals with pulmonary vascular disease (PVD). In a population enriched for PVD, we sought to establish qualitative grades and prognostic value of right heart chamber size and function. METHODS: We investigated 1053 study participants in PVDOMICS (Redefining Pulmonary Hypertension Through the PVD Phenomics Program) to determine clinical and echocardiographic differences associated with increasing pulmonary vascular resistance severity. Right heart chamber size and function were qualitatively assessed using a percentile-based approach above the median values to create a clinical grading system for right heart adaptation. The relationship between echocardiographic categories and all-cause mortality was examined using survival analyses adjusted for potential confounders. RESULTS: A stepwise increase in adverse right heart remodeling was observed with a concomitant decrease in functional parameters by pulmonary vascular resistance strata ( P <0.001 for all). Mild, moderate, and severe categories of right heart chamber size and dysfunction were defined using a percentile-based approach across the spectrum of PVD. During a median follow-up of 2.07 years (interquartile range, 1.23–3.01 years), 130 participants died (11.4%). Progressive pulmonary vascular resistance increase and 2DE evidence of right heart dysfunction inclusive of fractional area change, and right ventricular global longitudinal strain were independently associated with increased all-cause mortality risk in multivariate analysis adjusted for age, disease duration, and male sex. CONCLUSIONS: In this well-characterized sample of adults with diverse etiologies and varying PVD severity, we define categories of abnormal right heart chamber size and function. Further, we demonstrate a stepwise relationship between these categories of abnormal morphology and function and all-cause mortality. Defining grades of right ventricular dysfunction in individuals with known PVD has important clinical implications for monitoring disease progression and response to therapies.

OBJECTIVE Many patients recruited in the Treatment of Brain Arteriovenous Malformations Study (TOBAS) are managed conservatively. The aim of this study was to monitor what happened to those patients. METHODS TOBAS comprises two randomized controlled trials and multiple prospective registries. All patients with brain arteriovenous malformations (AVMs) can participate. This report concerns patients selected for conservative management. The primary trial outcome measure is related death or dependency (modified Rankin Scale [mRS] score > 2) at 10 years. Secondary outcomes include intracranial hemorrhages, nonhemorrhagic neurological events, and serious adverse events (SAEs). For this report, outcome results are presented using patient-years, Kaplan-Meier survival curves, and Cox log-rank tests. There was no blinding. RESULTS From June 2014 to May 2021, 1010 patients were recruited, of whom 498 (49%) were proposed the prospective observation registry. After exclusions, 434 (87%) patients remained for analysis. The majority of patients had unruptured AVMs (378/434 [87%]), of which 195 (52%) were low grade (Spetzler-Martin grade I or II). During a mean follow-up period of 3.2 years (total 1368 patient-years), the primary outcome occurred in 23 of 434 (5%) patients, corresponding to an incidence of 1.7 (95% CI 1.1–2.5) per 100 patient-years. For unruptured AVMs the incidence was 1.1 (95% CI 0.7–1.9) per 100 patient-years, and for low-grade unruptured AVMs it was 0.6 (95% CI 0.2–1.7) per 100 patient-years. Poor outcomes were more frequent in patients with a history of rupture (HR 5.6 [95% CI 2.4–13.0], p < 0.001), infratentorial AVMs (HR 2.9 [95% CI 1.1–7.3], p = 0.027), and age ≥ 55 years (HR 3.2 [95% CI 1.4–7.6], p = 0.007). Major intracranial hemorrhage occurred in 35 of 434 (8%) patients (incidence of 2.6 [95% CI 1.9–3.6] per 100 patient-years; 2.0 [95% CI 1.3–2.9] per 100 patient-years for unruptured AVMs and 1.3 [95% CI 0.6–2.6] per 100 patient-years for low-grade unruptured AVMs). Major AVM hemorrhages were more frequent in ruptured (HR 4.4 [95% CI 2.1–8.9], p < 0.001), large (HR 2.6 [95% CI 1.1–6.6], p = 0.039), and high-grade (HR 2.5 [95% CI 1.2–5.3], p = 0.013) AVMs and those with deep venous drainage (HR 2.1 [95% CI 1.1–4.2], p = 0.032). SAEs occurred in 48 of 434 (11%) patients (incidence of 3.6 [95% CI 2.7–4.8] per 100 patient-years). For unruptured AVMs the incidence was 2.8 (95% CI 2.0–4.0) per 100 patient-years, and for low-grade unruptured AVMs it was 1.8 (95% CI 1.0–3.2) per 100 patient-years. CONCLUSIONS Nearly half of TOBAS participants were observed. Rates of untoward neurological events were within expected boundaries.

Abstract Purpose Many women are employed at the time of breast cancer (BC) diagnosis. Pre-diagnosis psychological distress is prevalent among early-stage BC patients. We prospectively evaluated return to work (RTW) in BC patients with and without reported pre-diagnosis psychological distress over a 15-month period to determine if such distress affects workforce participation and mental health status after curative treatment. Methods 188 patients with newly diagnosed stage 0-III BC and working 35-plus hours/week participated at Mayo Clinic in Minnesota and Florida. Patients received an email survey at diagnosis and 3 and 15 months later. Incidence of severe psychological distress (SPD) per the Kessler Psychological Distress Scale (K6, score ≥ 13) was assessed at each timepoint. Results 77/188 survey respondents (41%) reported pre-diagnosis psychological distress, with 23.5% noting job-related stress. 59/188 patients (31%) had SPD at diagnosis, with similar incidence in the group that had pre-diagnosis distress (+ stress) and the non-stressed group (no-stress). The sample was highly educated and well salaried. The + stress group worked more hours on average at diagnosis (41.3 vs. 39.3 hours, p = 0.034). At 3 months, 83/141 respondents (60.1%) reported taking time off work after diagnosis, with 46.6% returning within 2 weeks and 42.5% requiring > 4 weeks’ break. More + stress patients reported RTW to maintain health insurance (13/56 vs. 6/85, p = 0.006), although this difference resolved by 15 months. SPD rates decreased over time but affected 17/104 respondents (16.3%) at 15 months. Conclusion Job-related stress is a primary driver of pre-diagnosis psychological distress. Three months after BC diagnosis, many women with pre-diagnosis psychological distress were motivated to RTW to keep employer-linked health insurance. SPD is prominent in BC patients; while SPD declined over time, it did not resolve completely.

Transgender and gender-diverse (TGD) persons represent a small but growing population in the United States. Accessing inclusive, equitable, and evidence-based healthcare remains a challenge for this patient population. Many TGD persons seek gender-affirming care, including gender-affirming hormonal therapy (GAHT) and gender-affirming surgery (GAS), to help ameliorate the physical and mental aspects of their gender incongruence. Both GAHT and GAS induce clinically important histopathologic and anatomic changes in breast tissue. Consequently, breast care in TGD persons has become an increasingly recognized topic of importance in gender-affirming care. However, there remains a scarce but growing base of literature specifically addressing the unique healthcare needs of breast care in TGD patients. This article will review how to establish trusting patient–provider relationships for TGD patients, gender inclusivity in breast clinics and imaging centers, the influence of GAHT and GAS on breast tissue, breast cancer screening recommendations and barriers, and breast cancer risk and treatment considerations in TGD persons.

Abstract Women at increased risk for breast cancer may benefit from taking risk-reducing medication (RRM) with tamoxifen (tam). Historical uptake of tam in women who qualify has been low. Recent studies have shown low-dose tam to have similar efficacy to standard dosing, with lower risk for adverse events. In this study, we aimed to evaluate uptake, adherence, and tolerability of low-dose tam in women at increased risk for breast cancer and those with ductal carcinoma in situ (DCIS). In this two-site prospective study, women who qualified for breast cancer RRM were offered participation and received consultation with a breast specialist for discussion of RRM rationale, benefits, side effects, and risks. Patients received baseline and 1-year follow-up surveys. A total of 41 patients consented for participation, and 31 completed 1-year follow-up. After initial consultation, 90% (n = 37) reported good/complete understanding of breast cancer risk. Of patients included in 1-year follow-up, 5 had DCIS, 13 had high-risk intraepithelial lesion, and 13 qualified based on Breast Cancer Risk Assessment Tool/International Breast Intervention Study calculation. Furthermore, 74% (n = 23) of patients reported that they took low-dose tam after consultation, with 78.2% (n = 18) of those still taking medication at 1 year. Patients who continued medication had higher estimated breast cancer risk compared with those who discontinued (International Breast Intervention Study 10-year risk, 12.7% vs. 7.6%; P = 0.027). All patients with DCIS initiated low-dose tam, and only one patient with DCIS had discontinued at 1 year. Uptake of low-dose tam after informed discussion is high. Adherence and tolerability at 1-year follow-up improved compared with those with traditional dosing of tam. Prevention Relevance: tam has been used extensively for breast cancer prevention in high-risk women. Historical uptake has been low because of concern for side effects and poor tolerability. Herein, we demonstrate that in the clinical setting, effective patient education and offering of a low-dose option can improve uptake in this high-risk population. See related Spotlight, p. 545

Abstract Disclosure: A. Verma: None. F. Elli: None. S. Rao: None. M. Smith: None. A. Shapiro: None. C. Moshe: None. R. Chadha: None. Introduction: Pheochromocytomas are rare, neuroendocrine tumors derived from chromaffin cells of the neural crest, typically within the adrenal medulla. The pathophysiologic effect of primary concern is the uncontrolled release of catecholamines into the bloodstream, which carry risk for episodic hypertension, palpitations, and life-threatening crises. Preoperative preparation with drugs that block adrenergic receptors is utilized to improve hemodynamic oscillation force and surgical outcomes. Widely used premedication include alpha-blockers, beta-blockers, and calcium channel blockers. Despite adequate pretreatment, hemodynamic instability continues to evoke massive risk. The objective of this study was to assess the incidence of intraoperative hypertensive episodes in patients undergoing robotic assisted laparoscopic adrenalectomy. Methods: This study is an observational retrospective analysis of patients who underwent elective laparoscopic robotic-assisted adrenalectomies from February 2019 to April 2023. 17 patients total patients were included. Preoperative information included age, BMI, ASA physical classification, preoperative blockade regiment, and urine metanephrines. Intraoperative information included operation duration, tumor size, intraoperative anti-hypertensives, and hypertensive spikes defined as a SBP greater than 175 mmHg for 5 consecutive minutes. Data analysis was performed to obtain frequency counts (%), means (standard deviations), and median (range) as appropriate. Results: The mean age at the time of surgery was 57.12 ± 17.2 years. The majority of patients (76%) were ASA status class III. 7 of the 17 patients did not have active pheochromocytoma based on symptomatology, the incidental diagnoses being made by imaging and workup. The average tumor diameter was 3.90 ± 1.8, with a range of 2.0 - 8.0. Mean operative time was 153.76 ± 53.9 minutes. Preoperative urinary metanephrines were observed to have a mean of 1,248.44 ± 2,866.9 pg/mL. In terms of preoperative preparation, 65% of patients received Doxazosin, and 29% of patients received an alternative anti-hypertensive. Yet, 64.7% of patients were found to have at least 1 intraoperative hypertensive spike Conclusion: Despite premedication with anti-hypertensives, a significant percentage of patients experienced intraoperative hemodynamic instability. This emphasizes the importance of anesthesiologist preparation for intraoperative hypertension management to aggressively prevent hypertensive crises. In addition, it questions the routine use of alpha blockade for all cases preoperatively as has been suggested in certain studies, and whether or not it should be driven by other patient characteristics. Presentation: 6/1/2024

A 59‐year‐old female developed symptoms of graft‐versus‐host disease 3½ weeks after liver transplantation for primary biliary cirrhosis. Symptoms of graft‐versus‐host disease included fever, erythroderma with bullae formation, and mucosal surface ulceration. Donor lymphocytes were present in the peripheral blood. Despite treatment with equine antilymphoblast globulin, steroids and, eventually, cyclosporine withdrawal, she developed neutropenia and succumbed to sepsis. At autopsy, she had marked lymphocyte depletion involving the bone marrow and both native and donor origin lymph nodes. As expected, the liver was normal. The clinical and pathological features were remarkably similar to those of the few other reported cases of graft‐versus‐host disease after liver transplantation and transfusion‐associated graft‐versus‐host disease; therapy is usually ineffective, and progression of disease leads to pancytopenia and death from sepsis.