Search

Purpose: The objective of this study was to characterize the clinical characteristics and factors predictive of biochemical remission in patients with symptomatic acromegaly undergoing transsphenoidal surgery (TSS) at an academic tertiary care center, as defined by the 2022 Acromegaly Consensus Conference guidelines., Methods: In this single institution, longitudinal, retrospective study, a large cohort of 158 patients with a preoperative diagnosis of acromegaly undergoing surgery at a large, academic, tertiary care center were examined. We excluded 38 patients as IGF-1 testing was performed less than 12 weeks postoperatively., Results: The majority of tumors were intrasellar macroadenomas (75%), receiving endoscopic surgery (98.3%). Patients who failed remission appeared to have higher raw IGF-1 levels preoperatively (732 ± 313 ng/mL) compared to those who attained remission (278 ± 313 ng/mL), and trended towards higher rates of GH hypersecretion (93.1% vs. 78.4%). Patients failing remission had higher GH levels and IGF-1 levels postoperatively and experienced a lower percentage reduction in raw IGF-1 levels. Multivariable logistic regression demonstrated that the magnitude of preoperative IGF-1 (OR: 1.001, 95% CI: 1.00, 1.003) and the percentage change in IGF-1 (OR: 1.021, 95% CI: 1.01, 1.04) were predictive of remission failure. Radiographic characteristics such as tumor size, suprasellar extension, and location were not necessarily predictive of worse postoperative outcomes., Conclusions: Lesions which failed to achieve biochemical remission appeared to display distinctive preoperative endocrinological characteristics, with preoperative IGF-1 levels and percentage changes in IGF-1 levels being predictive of biochemical remission status., Competing Interests: Declarations. Competing interests: The authors declare no competing interests., (© 2024. This is a U.S. Government work and not under copyright protection in the US; foreign copyright protection may apply.)

Context and Objective: The genetic profile of prolactinomas remains poorly understood. Our objective is to identify somatic genetic alterations associated with prolactinomas and to report the identification of an activating ESR1 mutation (ESR1Y537S) in an aggressive prolactinoma., Setting: Brigham and Women's Hospital., Design: Massively parallel-sequencing panel (OncoPanel) was performed in a cohort of patients with prolactinomas to identify mutations and copy number variation (CNV)., Results: Twenty subjects (mean age 38.6 years; 12 women and 8 men) were included in this study. A somatic ESR1Y537S mutation was identified in an aggressive prolactinoma in a post-menopausal woman. No SF3B1 or other somatic mutations were identified. The median number of CNV events identified in our samples was 46; the prolactinoma with ESR1Y537S had the highest number with 233 events. In breast cancer, ESR1Y537S has been shown to activate estrogen receptor alpha independent of ligand binding. In patients with resistant breast cancer and ESR1Y537S, elacestrant, a second-line ER degrader, improves progression-free survival. Therefore, given the lack of response to multimodality therapies, elacestrant was initiated in this patient after the third cycle of radiotherapy. Elacestrant, along with radiotherapy, controlled tumor growth and significantly reduced prolactin levels., Conclusion: Molecular profiling allowed the identification of ESR1Y537S, in an aggressive prolactinoma. ESR1Y537S was not detected early in the course of the disease and is likely conferring tumor aggressiveness. This finding emphasizes the significance of estrogen receptor signaling in prolactinomas. It also allowed the use of targeted therapy with successful control of disease progression., (© The Author(s) 2024. Published by Oxford University Press on behalf of the Endocrine Society. All rights reserved. For commercial re-use, please contact reprints@oup.com for reprints and translation rights for reprints. All other permissions can be obtained through our RightsLink service via the Permissions link on the article page on our site—for further information please contact journals.permissions@oup.com. See the journal About page for additional terms.)

Background and Objectives: Intrasellar arachnoid diverticulae can often be identified on preoperative imaging in patients undergoing endoscopic transsphenoidal surgery. The objective of this study was to characterize arachnoid diverticulae both qualitatively and quantitatively in a large institutional cohort of patients with pituitary tumors and to evaluate its association with intraoperative cerebrospinal fluid (CSF) leak., Methods: Preoperative imaging studies of 530 patients who underwent primary endoscopic transsphenoidal resection of pituitary tumors were examined both quantitatively and qualitatively for the presence of an intrasellar arachnoid diverticulum. A matched cohort analysis was performed to compare patients with a "significant" (>50% sellar depth) diverticulum with those with nonsignificant/no diverticulum. Morphologically, diverticulae were separately classified as Type 1 (ventral CSF cleft with no tumor/gland tissue between sellar face and infundibulum) or Type 2 (central CSF cleft with tumor/gland tissue between sellar face and infundibulum)., Results: Arachnoid diverticulae were noted in 40.2% of cases, and diverticulum depth was linearly correlated with tumor size and body mass index. A significant diverticulum was identified in 66 cases (12.5%) and was significantly associated with the functional tumor subtype ( P = .005) and intraoperative CSF leak ( P < .001). Type 1 clefts were associated with nonfunctional pathology ( P = .034) and the presence of suprasellar extension ( P = .035) and tended to be deeper than Type 2 clefts ( P < .001), with a higher incidence of intraoperative CSF leak ( P = .093). On logistic regression analysis, only the presence of a significant diverticulum was independently associated with intraoperative CSF leak (odds ratio 4.545; 95% CI 2.418-8.544; P < .001)., Conclusion: The presence of an intrasellar arachnoid diverticulum should alert the surgeon to an elevated risk of intraoperative CSF leak during transsphenoidal surgery for pituitary tumors. A relatively limited surgical exposure tailored to the craniocaudal extent of the sellar pathology should be considered in these patients., (Copyright © Congress of Neurological Surgeons 2024. All rights reserved.)

Background: Emerging research expands our understanding of the cerebellum beyond motor control to include cognitive, emotional, and autonomic functions. This review examines the cerebellum's complex role, spotlighting Schmahmann's syndrome, or cerebellar cognitive affective syndrome (CCAS), which impairs executive functions, language, and spatial processing. It emphasizes advancements in diagnosing CCAS and the imperative of developing superior diagnostic tools for managing cerebellar pathologies effectively., Methods: A comprehensive literature search was performed using databases such as PubMed, OVID Embase, and OVID Medline. Using the keywords "cerebellar cognitive, affective syndrome" and "Schmahmann syndrome," the search was conducted in accordance with the Preferred Reporting Items for Systematic Reviews and Meta-Analyses 2020 guidelines for systemic review, in which the selection process narrowed down an initial set of 54 articles to 12, focusing on the impact of the CCAS scale on diagnosing and understanding Schmahmann's syndrome., Results: The review's analysis confirms the cerebellum's roles in motor and cognitive functions and underscores the CCAS scale as a significant advancement in detecting cerebellar deficits, surpassing traditional assessments such as the mini-mental state examination and Montreal cognitive assessment., Conclusion: This review emphasizes the importance of understanding the cerebellum's involvement in cognition and emotion and the crucial role of the CCAS scale for identifying cerebellar impairments. It calls for better diagnostic tools to assess CCAS accurately and suggests enhancing the CCAS Scale to reflect cultural and educational diversity. This will improve the diagnosis and treatment of cerebellar disorders, promoting a comprehensive neurological perspective on the cerebellum's functions., Competing Interests: There are no conflicts of interest, (Copyright: © 2024 Surgical Neurology International.)

Purpose: Pituitary adenomas are the most common tumor of the pituitary gland and comprise nearly 15% of all intracranial masses. These tumors are stratified into functional or silent categories based on their pattern of hormone expression and secretion. Preliminary evidence supports differential clinical outcomes between some functional pituitary adenoma (FPA) subtypes and silent pituitary adenoma (SPA) subtypes., Methods: We collected and analyzed the medical records of all patients undergoing resection of SPAs or FPAs from a single high-volume neurosurgeon between 2007 and 2018 at Brigham and Women's Hospital. Descriptive statistics and the Mantel-Cox log-rank test were used to identify differences in outcomes between these cohorts, and multivariate logistic regression was used to identify predictors of radiographic recurrence for SPAs., Results: Our cohort included 88 SPAs and 200 FPAs. The majority of patients in both cohorts were female (48.9% of SPAs and 63.5% of FPAs). SPAs were larger in median diameter than FPAs (2.1 cm vs. 1.2 cm, p < 0.001). The most frequent subtypes of SPA were gonadotrophs (55.7%) and corticotrophs (30.7%). Gross total resection (GTR) was achieved in 70.1% of SPA resections and 86.0% of FPA resections (p < 0.001). SPAs had a higher likelihood of recurring (hazard ratio [HR] 3.2, 95% confidence interval [95%CI] 1.6-7.2) and a higher likelihood of requiring retreatment for recurrence (HR 2.5; 95%CI 1.0-6.1). Subset analyses revealed that recurrence and retreatment were more both likely for subtotally resected SPAs than subtotally resected FPAs, but this pattern was not observed in SPAs and FPAs after GTR. Among SPAs, recurrence was associated with STR (odds ratio [OR] 9.3; 95%CI 1.4-64.0) and younger age (OR 0.92 per year; 95%CI 0.88-0.98) in multivariable analysis. Of SPAs that recurred, 12 of 19 (63.2%) were retreated with repeat surgery (n = 11) or radiosurgery (n = 1), while the remainder were observed (n = 7).There were similar rates of recurrence across different SPA subtypes., Conclusion: Patients undergoing resection of SPAs should be closely monitored for disease recurrence through more frequent clinical follow-up and diagnostic imaging than other adenomas, particularly among patients with STR and younger patients. Several patients can be observed after radiographic recurrence, and the decision to retreat should be individualized. Longitudinal clinical follow-up of SPAs, including an assessment of symptoms, endocrine function, and imaging remains critical., (© 2024. The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature.)

Background and Objectives: The objective of this study was to study the association among various morphological parameters and surgical outcomes in pituitary macroadenomas with suprasellar extension., Methods: MRI studies of 160 patients undergoing endoscopic transsphenoidal resection of pituitary macroadenomas with suprasellar extension were reviewed. In the coronal plane, tumors were classified into Type 1 (dome-shaped, no constriction at the level of diaphragma sellae) and Type 2 (dumbbell-shaped, with constriction at the level of diaphragma sellae). Based on the dome-to-neck ratio (D/Nr), Type 2 tumors were further classified as Type 2A (wide neck; D/Nr >1 and <1.3) and Type 2B (narrow neck; D/Nr ≥1.3). Surgical outcomes and complications were analyzed using a logistic regression model. Overall extent of resection (EOR) and presence of residual sellar-suprasellar tumor was separately assessed in all patients with available postoperative MRI (n = 149)., Results: There were 108 Type 1 tumors and 26 patients each in the Type 2A and Type 2B subgroups. Tumor subtype was significantly associated with tumor size (P < .001), intraoperative cerebrospinal fluid leak (P < .001), EOR (P < .001), postoperative suprasellar residual tumor (P < .001), and postoperative complications, including diabetes insipidus (P = .005) and visual worsening (P = .003). On multivariate analysis, after adjusting for confounders, Type 2B tumors were negatively associated with EOR (odds ratio [OR] 0.22; 95% CI 0.07-0.68; P = .008) and associated with the presence of postoperative suprasellar residual tumor (OR 18.08; 95% CI 5.20-62.89; P < .001), intraoperative cerebrospinal fluid leak (OR 5.33; 95% CI 1.89-14.99; P = .002), and postoperative diabetes insipidus (OR 4.89; 95% CI 1.67-14.35; P < .001)., Conclusion: Preoperative tumor classification based on D/Nr is clinically and surgically relevant, and Type 2B macroadenomas are significantly associated with lower rates of gross total resection and higher rates of postoperative complications after endoscopic transsphenoidal resection., (Copyright © Congress of Neurological Surgeons 2023. All rights reserved.)

Context: In Cushing disease, the association between the rate of serum cortisol decline and recurrent disease after corticotroph adenoma removal has not been adequately characterized., Objective: To analyze postoperative serum cortisol and recurrence rates in Cushing disease., Methods: Patients with Cushing disease and pathology-confirmed corticotroph adenoma were retrospectively studied. Cortisol halving time was estimated using exponential decay modeling. Halving time, first postoperative cortisol, and nadir cortisol values were collected using immediate postoperative inpatient laboratory data. Recurrence and time-to-recurrence were estimated and compared among cortisol variables., Results: A total of 320 patients met inclusion/exclusion criteria for final analysis, and 26 of those patients developed recurrent disease. Median follow-up time was 25 months (95% CI, 19-28 months), and 62 patients had ≥ 5 years follow-up time. Higher first postoperative cortisol and higher nadir were associated with increased risk of recurrence. Patients who had a first postoperative cortisol ≥ 50 µg/dL were 4.1 times more likely to recur than those with a first postoperative cortisol < 50 µg/dL (HR 4.1, 1.8-9.2; P = .0003). Halving time was not associated with recurrence (HR 1.7, 0.8-3.8, P = .18). Patients with a nadir cortisol ≥2 µg/dL were 6.6 times more likely to recur than those with a nadir cortisol of < 2 µg/dL (HR 6.6, 2.6-16.6, P < .0001)., Conclusion: Postoperative nadir serum cortisol is the most important cortisol variable associated with recurrence and time-to-recurrence. Compared to first postoperative cortisol and cortisol halving time, a nadir < 2 µg/dL showed the strongest association with long-term remission and typically occurs within the first 24 to 48 hours after surgery., (© The Author(s) 2023. Published by Oxford University Press on behalf of the Endocrine Society. All rights reserved. For permissions, please e-mail: journals.permissions@oup.com.)

In the 2022 fifth edition of the WHO Classification of Endocrine Tumours and of Central Nervous System Tumours, pituitary adenomas are reclassified as neuroendocrine tumours (NETs). This change confers an oncology label to neoplasms that are overwhelmingly benign. A comprehensive clinical classification schema is required to guide prognosis, therapy and outcomes for all patients with pituitary adenomas. Pituitary adenomas and NETs exhibit some morphological and ultrastructural similarities. However, unlike NETs, pituitary adenomas are highly prevalent, yet indolent and rarely become malignant. This Perspective presents the outcomes of an interdisciplinary international workshop that addressed the merit and clinical implications of the classification change of pituitary adenoma to NET. Many non-histological factors provide mechanistic insight and influence the prognosis and treatment of pituitary adenoma. We recommend the development of a comprehensive classification that integrates clinical, genetic, biochemical, radiological, pathological and molecular information for all anterior pituitary neoplasms., (© 2023. Springer Nature Limited.)

Purpose: Pituitary carcinomas are a rare entity that respond poorly to multimodal therapy. Patients follow a variable disease course that remains ill-defined., Methods: We present an institutional case series of patients treated for pituitary carcinomas over a 30-year period from 1992 to 2022. A systematic review was conducted to identify prior case series of patients with pituitary carcinomas., Results: Fourteen patients with a mean age at pituitary carcinoma diagnosis of 52.5 years (standard deviation [SD] 19.4) met inclusion criteria. All 14 patients had tumor subtypes confirmed by immunohistochemistry and hormone testing, with the most common being ACTH-producing pituitary adenomas (n = 12). Patients had a median progression-free survival (PFS) of 1.4 years (range 0.7-10.0) and a median overall survival (OS) of 8.4 years (range 2.3-24.0) from pituitary adenoma diagnosis. Median PFS and OS were 0.6 years (range 0.0-2.2) and 1.5 years (range 0.1-9.6) respectively upon development of metastases. Most patients (n = 12) had locally invasive disease to the cavernous sinus, dorsum sellae dura, or sphenoid sinus prior to metastasis. Common sites of metastasis included the central nervous system, liver, lung, and bone. In a pooled analysis including additional cases from the literature, treatment of metastases with chemotherapy or a combination of radiation therapy and chemotherapy significantly prolonged PFS (p = 0.02), while failing to significantly improve OS (p = 0.14)., Conclusion: Pituitary carcinomas are highly recurrent, heterogenous tumors with variable responses to treatment. Multidisciplinary management with an experienced neuro-endocrine and neuro-oncology team is needed given the unrelenting nature of this disease., (© 2023. The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature.)

Objective: To evaluate the historical descriptive origins of the extracranial transnasal transsphenoidal route to the sphenoid sinus and sella turcica focusing on the works of two otolaryngologists: Markus Hajek (1861-1941) and Oskar Hirsch (1877-1965)., Data Sources: A collection of primary references of author publications, and contemporary references and textbooks., Review Methods: Primary references were reviewed with specific focus on surgical routes to the sphenoid sinus and sella turcica. Chronology was cross-referenced with contemporary publications by contemporaries. Translations from German were conducted by author AM when necessary., Results: Markus Hajek elegantly described the surgical approach to the posterior ethmoids and sphenoid sinus in 1904 using a transnasal route. Building on this foundation, Oskar Hirsch described the fully extracranial endonasal transethmoid transsphenoidal approach in 1909. He was first to describe surgical entrance to the sella using this exclusively unilateral endonasal route, which he demonstrated on a cadaver. He reports performing this procedure on a live patient in April, 1910, under local anesthesia in stages over 5 weeks. For better exposure, Hirsch consolidated his method with Killian's submucosal window resection of the posterior nasal septum allowing for bilateral access to the sphenoid sinus and sella, and completed a single stage procedure on a patient in June 1910., Conclusion: Oskar Hirsch was the first to describe and perform a stepwise surgical approach to the sella using an exclusively extracranial, endonasal, transethmoid, and transsphenoidal approach. He built upon his mentor Markus Hajek's approaches to the posterior ethmoid cells and sphenoid sinus., Level of Evidence: NA: Background information, synthesis from multiple sources emphasizing factual information Laryngoscope, 133:807-813, 2023., (© 2022 The Authors. The Laryngoscope published by Wiley Periodicals LLC on behalf of The American Laryngological, Rhinological and Otological Society, Inc.)

Purpose: Endonasal resection is the first-line treatment for patients harboring growth hormone (GH)-secreting pituitary adenomas. The complexity of the parasellar neurovascular structures makes pre-operative diagnostic imaging essential to understanding the anatomy of this region. We aimed to describe vascular anomalies in acromegalic patients and emphasize their relevance for surgery and preoperative planning., Methods: A systematic review following the PRISMA statement was performed in July 2021., Results: Thirty-three studies were evaluated. Elevated GH and insulin-like growth factor-1 (IGF-1) levels are linked to the occurrence of cardiovascular risk factors. This is attributed to endothelial dysfunction, mainly caused by changes in flow-mediated dilatation (FMD), which is probably the main cause of vascular anomalies in acromegaly. The occurrence of protrusions of the internal carotid artery (ICA) (35-53%), a narrow intercarotid distance, and an asymmetrical course was described. In 13-18% of acromegalic patients, the presence of an intracerebral aneurysm could be reported (incidence in the general population:0.8-1.3%). The selected studies were however performed with a small patient sample (range:1-257). We present a case report of a 57y/o male patient with anomalies of the ICA ("kissing carotid arteries") harboring a GH-secreting adenoma, which was resected via an endoscopic endonasal approach., Conclusions: There is an association between acromegaly and endothelial dysfunction, which increases cardiovascular risk factors and vascular anomalies. Preoperative vascular imaging, e.g., CT angiography, should be implemented as a standard to identify patients at risk and estimate surgical morbidity. However, no evidence-based recommendations exist so far, so future studies are necessary., (© 2022. The Author(s).)

Background: Technically and ergonomically correct positioning is essential in endoscopic transsphenoidal pituitary surgery., Objective: To propose a safe, ergonomic approach to conduct endoscopic endonasal pituitary and skull base surgery in large patients without sacrificing important aspects of current standard practice., Methods: The patient's right arm is outstretched and secured in a supine position, with the primary surgeon standing in the axillary area. Considerations include maintaining comfort, immobility, anesthesia access, endotracheal tube fixation, intravenous lines, thorax elevated 25° to 30° for optimization of respiration, free and exposed abdomen for fat graft, legs positioned with no sciatic stretch or venous strain, and the patient's head in parallel with the surrounding area to sustain a strict midline., Results: Ten patients who underwent transsphenoidal pituitary tumor resections conducted using the hitchhiker position from October 2019 to June 2021 comprised our study cohort. The patients' mean height was 168.70 ± 9.29 cm, their mean weight was 114.35 ± 19.32 kg, and their mean body mass index was 40.19 ± 6.39 kg/m 2 . Twenty percent (n = 2) patients had a body mass index classified as "obesity class 1," 50% (n = 5) as "obesity class 2," and 30% (n = 3) as "extreme obesity class 3." The primary surgeon consistently reported decreased bodily strain and improved ergonomic access to the surgical site using the hitchhiker position., Conclusion: The hitchhiker position offers the opportunity to improve both patient and physician well-being in the context of endoscopic pituitary surgery., (Copyright © Congress of Neurological Surgeons 2022. All rights reserved.)

Objective: Country-by-country estimates of the macroeconomic disease burden of central nervous system (CNS) cancers are important when determining the allocation of resources related to neuro-oncology. Accordingly, in this study the authors investigated macroeconomic losses related to CNS cancer in 173 countries and identified pertinent epidemiological trends., Methods: Data for CNS cancer incidence, mortality, and disability-adjusted life years (DALYs) were collected from the Global Burden of Disease 2019 database. Gross domestic product data were combined with DALY data to estimate economic losses using a value of lost welfare approach., Results: The mortality-to-incidence ratio of CNS cancer in 2019 was 0.60 in high-income regions compared to 0.82 in Sub-Saharan Africa and 0.87 in Central Europe, Eastern Europe, and Central Asia. Welfare losses varied across both high- and low-income countries. Welfare losses attributable to CNS cancer in Japan represented 0.07% of the gross domestic product compared to 0.23% in Germany. In low- and middle-income countries, Iraq reported welfare losses of 0.20% compared to 0.04% in Angola. Globally, the DALY rate in 2019 was the same for CNS cancer as for prostate cancer at 112 per 100,000 person-years, despite a 75% lower incidence rate, equating to CNS cancer welfare losses of 182 billion US dollars., Conclusions: Macroeconomic losses vary across high- and low-income settings and appear to be region specific. These differences may be explained by differences in regional access to screening and diagnosis, population-level genetic predispositions, and environmental risk factors. Mortality-to-incidence ratios are higher in low- and middle-income countries than in high-income countries, highlighting possible gaps in treatment access. Quantification of macroeconomic losses related to CNS cancer can help to justify the spending of finite resources to improve outcomes for neuro-oncological patients globally.

Purpose: In Cushing disease, early post-operative serum cortisol fluctuations have not been adequately characterized, and their association with initial remission and recurrence is unclear., Methods: A retrospective cohort study of patients with Cushing disease was conducted at two institutions. A "riser" was defined a priori as a paradoxical increase in serum cortisol with an immediate incremental increase in serum cortisol over three consecutive cortisol draws separated by roughly 6-h (definition 1). Post hoc analyses used a definition of two consecutive increases (definition 2). Risers were compared to non-risers for initial remission and time-to-recurrence., Results: A total of 505 patients with Cushing disease were screened, and 469 had adequate data for group assignment. Analysis of post-operative cortisol showed a subgroup of "risers" with a frequency of 3.6% for definition 1 and 42.6% for definition 2. In these patients, cortisol levels were significantly higher until approximately 36 h post-operatively, and cortisol had a significantly longer mean serum half-life. In the post hoc analysis, definition 2 risers had a lower remission rate compared to non-risers (162/196, 82.7%, versus 243/264, 92.0%) with an odds ratio of 0.41 (0.23-0.73; p = 0.003). For both definitions, recurrence was similar between groups., Conclusions: We found that almost half of Cushing disease patients experienced a temporary increase in serum cortisol level during the early post-operative period. Serum cortisol half-life was longer, and the remission rates were lower, however, recurrence rates were similar to non-risers., (© 2021. The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature.)

Context: No prospective epidemiologic studies have examined associations between use of oral contraceptives (OCs) or menopausal hormone therapy (MHT) and risk of pituitary adenoma in women., Objective: Our aim was to determine the association between use of OC and MHT and risk of pituitary adenoma in two separate datasets., Methods: We evaluated the association of OC/MHT with risk of pituitary adenoma in the Nurses' Health Study and Nurses' Health Study II by computing multivariable-adjusted hazard ratios (MVHR) of pituitary adenoma by OC/MHT use using Cox proportional hazards models. Simultaneously, we carried out a matched case-control study using an institutional data repository to compute multivariable-adjusted odds ratios (MVOR) of pituitary adenoma by OC/MHT use., Results: In the cohort analysis, during 6 668 019 person-years, 331 participants reported a diagnosis of pituitary adenoma. Compared to never-users, neither past (MVHR = 1.05; 95% CI, 0.80-1.36) nor current OC use (MVHR = 0.72; 95% CI, 0.40-1.32) was associated with risk. For MHT, compared to never-users, both past (MVHR = 2.00; 95% CI, 1.50-2.68) and current use (MVHR = 1.80; 95% CI, 1.27-2.55) were associated with pituitary adenoma risk, as was longer duration (MVHR = 2.06; 95% CI, 1.42-2.99 comparing more than 5 years of use to never, P trend = .002). Results were similar in lagged analyses, when stratified by body mass index, and among those with recent health care use. In the case-control analysis, we included 5469 cases. Risk of pituitary adenoma was increased with ever use of MHT (MVOR = 1.57; 95% CI, 1.35-1.83) and OC (MVOR = 1.27; 95% CI, 1.14-1.42) compared to never., Conclusion: Compared to never use, current and past MHT use and longer duration of MHT use were positively associated with higher risk of pituitary adenoma in 2 independent data sets. OC use was not associated with risk in the prospective cohort analysis and was associated with only mildly increased risk in the case-control analysis., (© The Author(s) 2021. Published by Oxford University Press on behalf of the Endocrine Society. All rights reserved. For permissions, please e-mail: journals.permissions@oup.com.)

Background: The association between alcohol intake and incidence of pituitary adenoma has not been reported previously. We examined this association in three large, prospective cohort studies., Methods: Using data from the Nurses' Health Study, Nurses' Health Study II, and Health Professionals Follow-Up Study, we computed multivariable-adjusted hazard ratios (MVHR) and 95% confidence intervals (CI) for pituitary adenoma by levels of alcohol intake using Cox proportional hazards regression., Results: We identified 292 incident cases of pituitary adenoma (225 among women, 67 among men) among 235,973 participants with 6,548,732 person-years of follow-up. Compared with intake of ≤ 0.5 g/day, cumulative average alcohol intake in all categories was associated with reduced risk of pituitary adenoma (MVHR = 0.60, 95% CI 0.43-0.83 for 0.5-≤ 2 g/day, MVHR = 0.57, 95% CI 0.41-0.79 for > 2.0-≤ 8.0, MVHR = 0.70, 95% CI 0.47-1.04 for > 8.0-≤ 15.0, and MVHR = 0.51, 95% CI 0.32-0.83 for > 15.0 g/day). Significant inverse findings were present in women and were similar but non-significant in men. For specific alcoholic beverages, inverse associations were statistically significant for total wine (MVHR = 0.58, 95% CI 0.43-0.79 comparing 0.5-≤ 2 to ≤ 0.5 g/day), red wine (MVHR = 0.65, 95% CI 0.46-0.92 comparing 0.5-≤ 2 to ≤ 0.5 g/day), and white wine (MVHR = 0.72, 95% CI 0.53-0.97 comparing 0.5-≤ 2 to ≤ 0.5 g/day). Results were consistent using baseline intake, recent intake, and with an 8-year lag., Conclusion: In three prospective cohorts, compared to almost no consumption, alcohol consumption was associated with reduced risk of pituitary adenoma. Sensitivity analyses suggest that these results are unlikely to be the result of reverse causation or diagnostic bias., (© 2022. The Author(s), under exclusive licence to Springer Nature Switzerland AG.)

Purpose: To examine the contemporary epidemiology of adult pituitary tumors with a particular focus on uncommon tumor types, using the 2017 WHO Classification of pituitary tumors., Methods: Adult patients presenting with a pituitary or sellar tumor between 2004 and 2017 were identified from the U.S. National Cancer Database, with tumor type categorized according to the 2017 WHO classification. Descriptive epidemiological statistics were evaluated and reported for all pituitary tumor types and subtypes., Results: 113,349 adults with pituitary tumors were identified, 53.0% of whom were female. The majority of pituitary tumors were pituitary adenomas (94.0%), followed by craniopharyngiomas (3.8%). Among pituitary adenomas, whereas 71.6% of microadenomas presented in females, only 46.7% of macroadenomas and 41.3% of giant adenomas did (p < 0.001). For craniopharyngiomas, 71.2% were adamantinomatous and 28.8% were papillary, with adamantinomatous tumors associated with Black non-Hispanic race/ethnicity (OR adj  = 2.44 vs. White non-Hispanic, 99.9 %CI = 1.25-4.75, p < 0.001) in multivariable analysis. The remaining 0.7% (n = 676) of pathology-confirmed pituitary tumor types were composed of: 21% tumors of the posterior pituitary, 16% chordomas, 11% pituitary carcinomas (i.e. adenohypophyseal histology with metastasis; herein most frequently to bone), 10% meningiomas, 8% germ cell tumors, 7% hematolymphoid (largely DLBCLs), and 4% neuronal/paraneuronal (largely gangliogliomas). Pituitary carcinomas and posterior pituitary tumors demonstrated a male predilection (62.2% and 56.0%, respectively), whereas sellar meningiomas predominated in females (84.1%). Age, race/ethnicity, tumor size, and overall survival further varied across uncommon pituitary tumor types., Conclusions: Our findings provide a detailed contemporary dissection of the epidemiology of common and uncommon adult pituitary tumors in the context of WHO2017., (© 2021. The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature.)

Purpose: A classification system for cystic sellar lesions does not exist. We propose a novel classification scheme for these lesions based on the heterogeneity of the cyst wall/contents and the presence of a solid component on imaging., Methods: We retrospectively reviewed 205 patients' medical records (2008-2020) who underwent primary surgery for a cystic sellar lesion. Cysts were classified a priori into 1 of 4 cyst types based on the heterogeneity of the cyst wall/contents and the presence of a solid component imaging. There was high interrater reliability. Univariable and multivariable models were used to estimate the ability of cyst type to predict the two most common diagnoses: Rathke cleft cyst (RCC) and cystic pituitary adenoma., Results: The frequencies of RCC and cystic pituitary adenoma in our cohort were 45.4% and 36.4%, respectively. Non-neoplastic lesions (e.g., arachnoid cysts and RCC) were more likely to be Type 1 or 2, whereas cystic neoplasms (e.g., pituitary adenomas and craniopharyngiomas) were more likely to be Type 3 or 4 (p<0.0001). Higher cyst types, compared to Type 1, had higher odds of being cystic pituitary adenomas compared to RCCs (OR: 23.7, p=0.033, and 342.6, p <0.0001, for Types 2 and 4, respectively). Lesions with a fluid-fluid level on preoperative MRI also had higher odds of being pituitary adenomas (OR: 12.7; p=0.023). Cystic pituitary adenomas were more common in patients with obesity (OR: 5.0, p=0.003) or symptomatic hyperprolactinemia (OR: 11.5; p<0.001, respectively). The multivariable model had a positive predictive value of 82.2% and negative predictive value of 86.4%., Conclusion: When applied to the diagnosis of RCC versus cystic pituitary adenoma, higher cystic lesion types (Type 2 & 4), presence of fluid-fluid level, symptomatic hyperprolactinemia, and obesity were predictors of cystic pituitary adenoma. Further validation is needed, but this classification scheme may prove to be a useful tool for the management of patients with common sellar pathology., Competing Interests: The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest., (Copyright © 2021 Tavakol, Catalino, Cote, Boles, Laws and Bi.)

Purpose: Surgical workflow analysis seeks to systematically break down operations into hierarchal components. It facilitates education, training, and understanding of surgical variations. There are known educational demands and variations in surgical practice in endoscopic transsphenoidal approaches to pituitary adenomas. Through an iterative consensus process, we generated a surgical workflow reflective of contemporary surgical practice., Methods: A mixed-methods consensus process composed of a literature review and iterative Delphi surveys was carried out within the Pituitary Society. Each round of the survey was repeated until data saturation and > 90% consensus was reached., Results: There was a 100% response rate and no attrition across both Delphi rounds. Eighteen international expert panel members participated. An extensive workflow of 4 phases (nasal, sphenoid, sellar and closure) and 40 steps, with associated technical errors and adverse events, were agreed upon by 100% of panel members across rounds. Both core and case-specific or surgeon-specific variations in operative steps were captured., Conclusions: Through an international expert panel consensus, a workflow for the performance of endoscopic transsphenoidal pituitary adenoma resection has been generated. This workflow captures a wide range of contemporary operative practice. The agreed "core" steps will serve as a foundation for education, training, assessment and technological development (e.g. models and simulators). The "optional" steps highlight areas of heterogeneity of practice that will benefit from further research (e.g. methods of skull base repair). Further adjustments could be made to increase applicability around the world., (© 2021. The Author(s).)

Aggressive pituitary neuroendocrine tumors (APT) account for 10% of pituitary tumors. Their management is a rapidly evolving field of clinical research and has led pituitary teams to shift toward a neuro-oncological-like approach. The new terminology "Pituitary neuroendocrine tumors" (PitNet) that was recently proposed to replace "pituitary adenomas" reflects this change of paradigm. In this narrative review, we aim to provide a state of the art of actual knowledge, controversies, and recommendations in the management of APT. We propose an overview of current prognostic markers, including the recent five-tiered clinicopathological classification. We further establish and discuss the following recommendations from a neurosurgical perspective: (i) surgery and multi-staged surgeries (without or with parasellar resection in symptomatic patients) should be discussed at each stage of the disease, because it may potentialize adjuvant medical therapies; (ii) temozolomide is effective in most patients, although 30% of patients are non-responders and the optimal timeline to initiate and interrupt this treatment remains questionable; (iii) some patients with selected clinicopathological profiles may benefit from an earlier local radiotherapy and/or chemotherapy; (iv) novel therapies such as VEGF-targeted therapies and anti-CTLA-4/anti-PD1 immunotherapies are promising and should be discussed as 2nd or 3rd line of treatment. Finally, whether neurosurgeons have to operate on "pituitary adenomas" or "PitNets," their role and expertise remain crucial at each stage of the disease, prompting our community to deal with evolving concepts and therapeutic resources., (© 2021. The Author(s), under exclusive licence to Springer-Verlag GmbH Austria, part of Springer Nature.)

Purpose: Rathke cleft cysts (RCC) are a target for transsphenoidal surgery when symptomatic; however, the surgical techniques and outcomes continue to be of interest. Our goal is to highlight nuances in diagnosis, surgical philosophy and techniques, and outcomes., Methods: We retrospectively reviewed a single surgeon experience in a contemporary series of RCC from April 2008 through December 2019. The clinical presentation, radiographic studies, intraoperative findings, histopathological diagnosis, postoperative complications, and outcome data were recorded and analyzed., Results: A total of 131 endoscopic endonasal operations were performed on 116 patients (30 male, 86 female), with an average age of 39 years. We identified 102 patients without prior surgery, and 14 patients with prior surgery elsewhere. Postoperative complications included transient diabetes insipidus (DI) (n = 23, 20%), permanent DI (n = 5, 4%), SIADH (n = 6, 5%), epistaxis (n = 6, 5%), postoperative infection (n = 3, 2.5%), postoperative CSF leak (n = 1, < 1%), intrasellar hematoma (n = 1, < 1%), and stroke (n = 1, < 1%). Visual improvement was observed in 74% (n = 31/42) of patients presenting with visual loss, and headache improvement in 86% (n = 80/93). Overall, the recurrence rate in our cohort was 11%, with a lower recurrence rate seen in males (p = 0.02). The median follow-up duration was 15.2 months (interquartile range 1.7-52.3) CONCLUSION: Our results reflect the philosophy of a conservative surgical approach, designed to preserve normal pituitary function, and avoid DI, while achieving the maximal feasible resection. Evacuation of the cyst contents and meticulous sampling of the cyst wall when possible for histopathological diagnosis are crucial. The postoperative relief of symptoms and signs is gratifying, as are the overall rates of recurrence and outcomes., (© 2021. This is a U.S. government work and not under copyright protection in the U.S.; foreign copyright protection may apply.)

Introduction  Rene Descartes (1596-1650), the famous philosopher and scientist, identified the pineal gland as the only cerebral structure not represented bilaterally, the "seat of the soul"; and the source of rational thought. Pineal cysts (PCs) are often incidentally identified in MRI studies, with a reported prevalence of 1 to 4.3%. Rathke cleft cysts (RCCs) are pituitary lesions accounting for <1% of intracranial masses. There are scant data in the literature addressing any association between these two midline cystic lesions. Methods  We reviewed the medical records of patients presenting at our institution from April 2008 through February 2020, whose records indicated a diagnosis of RCC, and those whose records included pineal lesions. Our objective was to evaluate the association between these two midline lesions. Brain MRI studies were reviewed for the presence of PCs; only patients with PCs that measured ≥5 mm in diameter were included. Results  We identified 116 patients with RCCs, and 34 patients with PCs, treated from April 2008 through February 2020. Among the RCC group, 14/116 patients (12%) had PCs. Among the PC group, 3/34 patients (8.8%) had RCCs. Overall, 17 patients (11.3%) had concomitant RCCs and PCs. The mean maximal diameter of the PCs was 7.5 mm (range = 5-17 mm), whereas the mean maximal diameter of RCCs was 13 mm (range = 5-40 mm). Conclusion  The incidental diagnosis of cystic lesions of the pineal and pituitary gland is increasingly reported, primarily because of advances in current diagnostic modalities. Our data demonstrated no clear consensual association between pineal and pituitary cysts., Competing Interests: Conflict of interest None declared., (Thieme. All rights reserved.)

Purpose: Hypothalamic-pituitary axis dysfunction and mass effect symptoms in the pediatric population can indicate a pituitary region tumor. Herein, we evaluate the epidemiology and management of this rare entity., Methods: Pediatric patients (≤ 21yo) who presented from 2004 to 2017 with a pituitary tumor were evaluated from the U.S. National Cancer Database. The distributions and management patterns of pituitary tumors were assessed by patients' tumor type, age, sex, race/ethnicity, tumor size, and insurance status., Results: 19.7% of intracranial tumors in the pediatric population originated in the pituitary region. 7653 pediatric patients with pituitary region tumors were identified, 68.2% of whom were female, with the tumors predominantly occurring in early adolescence (46.9%) and late adolescence (34.8%). The majority of pediatric pituitary region tumors were pituitary adenomas (77.9%), followed by craniopharyngiomas (18.1%) and germ cell tumors (1.6%). Girls demonstrated higher proportions of pituitary adenomas across all ages than boys. Asian/Pacific Islander patients were independently more likely to present at younger ages (mean 13.9yrs) and with germ cell tumors than patients of other races/ethnicities. Only 5.5% of patients were uninsured (referent), but they were independently more likely to present at older ages (mean 17.9yrs) and less likely to undergo surgery than patients with private insurance (OR = 1.93, 95% CI = 1.47-2.52, p < 0.001) or Medicaid (OR = 1.51, 95% CI = 1.14-2.00, p = 0.004)., Conclusion: Pituitary region tumors comprise a significant fraction of intracranial pediatric tumors, particularly in adolescent girls. The differential diagnosis of pituitary tumor types differed significantly by patients' age, sex, and race/ethnicity. Uninsured patients were associated with delays in care and less surgical management.

Objective  Different surgical set-ups for endoscopic transsphenoidal surgery (ETS) have been described, but studies on their ergonomics are limited. The aim of this article is to describe present trends in the ergonomics of ETS. Design and Participants  A 33-question, web-based survey was sent to North American Skull Base Society members in 2018 and 116 responded to it (16% of all members). Most respondents were from North America (76%), in academic practice (87%), and neurosurgeons (65%); they had more than 5 years of experience in ETS (73%), had received specific training (66%), and performed at least 5 procedures/mo (55%). Results  Mean reported time for standard and complex procedures were 3.7 and 6.3 hours, respectively. The patient's body is usually positioned in a straight, supine position (84%); the head is in a neutral position (46%) or rotated to the side (38%). Most surgeons perform a binostril technique, work with a partner (95%), and operate standing (94%), holding suction (89%) and dissector (83%); sometimes the endoscope is held by the primary surgeon (22-24%). The second surgeon usually holds the endoscope (72%) and irrigation (42%). During tumor removal most surgeons stand on the same side (65-66%). Many respondents report strain at the dorsolumbar (50%) or cervical (26%) level. Almost one-third of surgeons incorporate a pause during surgery to stretch, and approximately half exercise to be fit for surgery; 16% had sought medical attention for ergonomic-related symptoms. Conclusion  Most respondents value ergonomics in ETS. The variability in surgical set-ups and the relatively high report of complaints underline the need for further studies to optimize ergonomics in ETS., Competing Interests: Conflict of Interest None declared., (Thieme. All rights reserved.)

Context: No studies have examined the association between body habitus and incidence of pituitary adenoma., Objective: To determine if body mass index (BMI), waist circumference, body somatotype, or height are associated with risk of pituitary adenoma., Design: Pooled analysis of 3 prospective cohort studies., Setting: Population-based study., Participants: Participants of the Nurses' Health Study (NHS), Nurses' Health Study II (NHSII), and the Health Professionals Follow-Up Study (HPFS), totaling 284 946 American health professionals., Exposures: BMI, waist circumference, body somatotype, and height., Outcome Measures: Self-reported incident pituitary adenoma. Multivariable (MV)-adjusted hazard ratios (HRs) of pituitary adenoma were estimated using Cox proportional hazards models., Results: During 7 350 156 person-years of follow-up, 387 incident pituitary adenomas were reported. Comparing BMI of ≥30 to <25 kg/m2, higher adult BMI was associated with higher risk of pituitary adenoma (MV HR = 1.74; 95% CI, 1.33-2.28), as was higher maximum adult BMI (MV HR = 1.76; 95% CI, 1.34-2.30), higher waist circumference (MV HR = 1.06; 95% CI, 1.04-1.09 per inch), and higher BMI during early adulthood (at age 18 to 21, MV HR = 2.65; 95% CI, 1.56-4.49). Taller adult height was associated with pituitary adenoma (MV HR = 1.05; 95% CI, 1.01-1.09 per inch). Overall findings were similar in women and men, although power was limited in men (n = 62 cases). Sensitivity analyses demonstrated that the association between adult BMI and pituitary adenoma extended to at least 14 years prior to diagnosis and that the results were not affected when analyses were restricted to participants with similar healthcare utilization., Conclusion: Higher BMI and waist circumference, from early adulthood to the time of diagnosis, were associated with higher risk of pituitary adenoma., (© The Author(s) 2021. Published by Oxford University Press on behalf of the Endocrine Society. All rights reserved. For permissions, please e-mail: journals.permissions@oup.com.)

Background: Corticotroph tumor progression (CTP) leading to Nelson's syndrome (NS) is a severe and difficult-to-treat complication subsequent to bilateral adrenalectomy (BADX) for Cushing's disease. Its characteristics are not well described, and consensus recommendations for diagnosis and treatment are missing., Methods: A systematic literature search was performed focusing on clinical studies and case series (≥5 patients). Definition, cumulative incidence, treatment and long-term outcomes of CTP/NS after BADX were analyzed using descriptive statistics. The results were presented and discussed at an interdisciplinary consensus workshop attended by international pituitary experts in Munich on October 28, 2018., Results: Data covered definition and cumulative incidence (34 studies, 1275 patients), surgical outcome (12 studies, 187 patients), outcome of radiation therapy (21 studies, 273 patients), and medical therapy (15 studies, 72 patients)., Conclusions: We endorse the definition of CTP-BADX/NS as radiological progression or new detection of a pituitary tumor on thin-section MRI. We recommend surveillance by MRI after 3 months and every 12 months for the first 3 years after BADX. Subsequently, we suggest clinical evaluation every 12 months and MRI at increasing intervals every 2-4 years (depending on ACTH and clinical parameters). We recommend pituitary surgery as first-line therapy in patients with CTP-BADX/NS. Surgery should be performed before extrasellar expansion of the tumor to obtain complete and long-term remission. Conventional radiotherapy or stereotactic radiosurgery should be utilized as second-line treatment for remnant tumor tissue showing extrasellar extension.

Objectives  Initial therapy for the management of prolactinomas has long been maintained to be medical, consisting of a dopamine agonist. These therapies may have troublesome side effects, and some prolactinomas are resistant to medical therapy regarding lowering prolactin levels or shrinking the tumor. These issues have revived interest in surgery for prolactin-secreting adenomas as an early therapeutic option. We report our analysis of surgery for prolactin microadenomas in women, using the transsphenoidal endoscopic approach. Design  We reviewed a contemporary series of 33 women (mean age = 31.8 years) with microprolactinomas who underwent early surgical intervention, which was a three-dimensional transnasal transsphenoidal endoscopic operation. Setting  The study was conducted at a tertiary academic referral center for pituitary tumors. Main Outcome Measures  Preoperative and postoperative prolactin. Results  Overall, 28 patients had received preoperative dopamine agonists, 24 of these experienced a variety of drug-related side effects, and 4 had tumors that were resistant to lowering prolactin or tumor shrinkage. Preoperative prolactin levels averaged 90.3 ng/mL (range = 30.7-175.8 ng/mL). We observed a 94% normalization rate in postoperative prolactin (mean = 10.08 ng/mL, range = 0.3-63.1 ng/mL). During the follow-up (mean = 33.9 months), five patients had elevated prolactin; four required reinitiation of medical therapy, two had surgical reexploration, and none received radiation therapy. Complications included syndrome of inappropriate antidiuretic hormone secretion ( n  = 3), transient diabetes insipidus ( n  = 1), postoperative epistaxis ( n  = 1), and fat graft site infection ( n  = 1). Conclusion  This review supports the consideration of transsphenoidal surgery as an early intervention for some women with prolactin-secreting microadenoma. Indications include significant side effects of medical therapy and tumors that do not respond to standard medical management., Competing Interests: Conflict of Interest None declared., (Thieme. All rights reserved.)

Context: Recurrence of Cushing disease (CD) can occur even decades after surgery. Biomarkers to predict recurrence of CD after surgery have been studied but are inconclusive., Objective: The aim of our study was to identify specific biomarkers that can predict long-term remission after neurosurgery., Design: Identification of specific biomarkers to predict long-term remission of CD was performed by logistic regression analysis followed by Kaplan-Meier survival analysis, using recurrence as the dependent variable., Setting: A total of 260 patients with CD identified from our institutional research patient data registry search tool and from patients who presented to our longitudinal multidisciplinary clinic between May 2008 and May 2018 underwent statistical analysis., Interventions: Data on clinical features, neuro-imaging study, pathology, biochemistry, and treatments were collected by reviewing digital chart records., Main Outcome Measure: Postoperative cortisol as a biomarker to predict long-term remission after surgical treatment for CD., Results: By logistic regression analysis, postoperative day 1 (POD1) morning (5-10 am) serum cortisol, female sex, and proliferative index had significant association with CD recurrence (odds ratio [OR] = 1.025, 95% CI: 1.002-1.048, P = .032). In contrast, the postoperative nadir cortisol (OR = 1.081, 95% CI: 0.989-1.181, P = .086), urinary free cortisol (OR = 1.032, 95% CI: 0.994-1.07, P = .098), and late night salivary cortisol (OR = 1.383, 95% CI: 0.841-2.274, P = .201) had no significant correlation with recurrence. A significant association between POD1 morning serum cortisol and long-term CD remission was verified by Kaplan-Meier analysis when using POD1 morning serum cortisol <5 μg/dL as the cut-off., Conclusions: The POD1 morning serum cortisol level has a significant association with CD recurrence., (Published by Oxford University Press on behalf of the Endocrine Society 2020.)

Purpose: Acromegaly is a rare neuroendocrine condition that can lead to significant morbidity. Despite China's vast population size, studies on acromegaly remain sparse. This study aimed to investigate the clinical characteristics and predictors of biochemical remission after surgery for acromegaly using the China Acromegaly Patient Association (CAPA) database., Methods: A retrospective nationwide study was conducted using patient-reported data from CAPA database between 1998 and 2018. The principal component analysis (PCA) and logistic regression analysis were employed to determine independent predictors of biochemical remission at 3 months in patients after surgery., Results: Of the 546 surgical cases (mean age: 36.8 years; 59.5% females), macroadenomas and invasive tumors (Knosp score 3-4) were 83.9% and 64.1%, respectively. Ninety-five percent of patients were treated with endonasal surgery and 36.8% exhibited biochemical remission at 3-months postoperatively. The following independent predictors of biochemical remission were identified: preoperative growth hormone (GH) levels between 12 and 28 μg/L [odds ratio (OR) = 0.58; 95% confidence interval (CI), 0.37-0.92; p = 0.021], preoperative GH levels > 28 μg/L (OR = 0.55; 95% CI, 0.34-0.88; p = 0.013), macroadenoma (OR = 0.56; 95% CI, 0.32-0.96; p = 0.034), giant adenomas (OR = 0.14; 95% CI, 0.05-0.38; p < 0.001), Knosp score 3-4 (OR = 0.37; 95% CI, 0.24-0.57; p < 0.001), and preoperative medication usage (OR = 2.32; 95% CI, 1.46-3.70; p < 0.001)., Conclusions: In this nationwide study spanning over two decades, we highlight that higher preoperative GH levels, large tumor size, and greater extent of tumor invasiveness are associated with a lower likelihood of biochemical remission at 3-months after surgery, while preoperative medical therapy increases the chance of remission.

Background: Diabetes insipidus (DI) is a recognized transient or permanent complication following transsphenoidal surgery (TSS) for pituitary tumors., Objective: To describe significant experience with the incidence of DI after TSS, identifying predictive characteristics and describing our diagnosis and management of postoperative DI., Methods: A retrospective analysis was performed of 700 patients who underwent endoscopic TSS for resection of pituitary adenoma (PA), Rathke cleft cyst (RCC), or craniopharyngioma. Inclusion criteria included at least 1 wk of follow-up for diagnosis of postoperative DI. Permanent DI was defined as DI symptoms and/or need for desmopressin more than 1 yr postoperatively. All patients with at least 1 yr of follow-up (n = 345) were included in analyses of permanent DI. Multivariable logistic regression models were constructed to identify predictors of transient or permanent postoperative DI., Results: The overall rate of any postoperative DI was 14.7% (103/700). Permanent DI developed in 4.6% (16/345). The median follow-up was 10.7 mo (range: 0.2-136.6). Compared to patients with PA, patients with RCC (odds ratio [OR] = 2.2, 95% CI: 1.2-3.9; P = .009) and craniopharyngioma (OR = 7.0, 95% CI: 2.9-16.9; P ≤ .001) were more likely to develop postoperative DI. Furthermore, patients with RCC (OR = 6.1, 95% CI: 1.8-20.6; P = .004) or craniopharyngioma (OR = 18.8, 95% CI: 4.9-72.6; P ≤ .001) were more likely to develop permanent DI compared to those with PA., Conclusion: Although transient DI is a relatively common complication of endoscopic and microscopic TSS, permanent DI is much less frequent. The underlying pathology is an important predictor of both occurrence and permanency of postoperative DI., (Copyright © 2020 by the Congress of Neurological Surgeons.)

Purpose: Soft tissue sarcoma (STS) of the sella is exceptionally rare. We conducted a case series, literature review, and nationwide analysis of primary and iatrogenic (radiation-associated) STS of the sella to define the clinical course of this entity., Methods: This study employed a multi-institutional retrospective case review, literature review, and nationwide analysis using the National Cancer Database (NCDB)., Results: We report five patients who were diagnosed at three institutions with malignant STS of the sella. All patients presented with symptoms related to mass effect in the sellar region. All tumors extended to the suprasellar space, with the majority displaying extension into the cavernous sinus. All patients underwent an operation via a transsphenoidal approach with a goal of maximal safe tumor resection in four patients and biopsy for 1 patient. Histopathologic evaluation demonstrated STS in all patients. Post-operative adjuvant radiotherapy and chemotherapy were given to 2 and 1 out of 4 patients with known post-operative clinical course, respectively. The 1-year and 5-year overall survival rates were 100% (5/5) and 25% (1/4). Twenty-two additional reports of primary, non-iatrogenic STS of the sella were identified in the literature. Including the three cases from our series, treatment included resection in all cases, and adjuvant radiotherapy and chemotherapy were utilized in 50% (12/24) and 17% (4/24) of cases, respectively. The national prevalence of malignant STS is estimated to be 0.01% among all pituitary and sellar tumors within the NCDB., Conclusions: We report the prevalence and survival rates of STS of the sella. Multimodal therapy, including maximal safe resection, chemotherapy, and radiotherapy are necessary to optimize outcomes for this uncommon pathology.