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Craniopharyngiomas are tumors that arise from the remnants of Rathkes pouch along the nasopharynx to the diencephalon. Current standard of care includes maximal surgical resection versus adjuvant radiation if a maximal resection is unfeasible. Pharmacological therapy with MAPK targeted agents is an emerging therapeutic option for tumors with BRAF V600E mutations. We report a 45-year-old male with a strictly third ventricle papillary craniopharyngioma with a BRAF V600E mutation. After initial surgery with subtotal resection, the patient demonstrated durable response to targeted BRAF and MEK inhibitor therapy with vemurafenib and cobimetinib. Our report suggests that targeted therapy may reduce the need for radiation and impact surgical interventions in select cases.

Background: Pituitary adenomas represent the most common pituitary disorder, with an estimated prevalence as high as 20%, and they can manifest with hormone hypersecretion or deficiency, neurological symptoms from mass effect, or incidental findings on imaging. Transsphenoidal surgery, performed either microscopically or endoscopically, allows for a better extent of resection while minimising the associated risk in comparison to the transcranial approach. Endoscopy allows for better visualisation and improvement in tumour resection with an improved working angle and less nasal morbidity, making it likely to become the preferred surgical treatment for pituitary neoplasms. The learning curve can be aided by telementoring. Methods: We retrospectively analysed the clinical records of 94 patients who underwent an endoscopic endonasal resection of a pituitary neoplasm between the years 2011 and 2023 at Maribor University Medical Centre in Slovenia. Remote surgical telementoring over 3 years assisted with the learning curve. Results: The proportion of complication-free patients significantly increased over the observed period (60% vs. 79%). A gradual but insignificant increase in the percentage of patients with improved endocrine function was observed. Patients' vision improved significantly over the observed period. By gaining experience, the extent of gross total tumour resection increased insignificantly (67% vs. 79%). Conclusions: Telementoring for the endoscopic endonasal approach to pituitary neoplasms enables low-volume centres to achieve efficiency, decreasing rates of postoperative complications and increasing the extent of tumour resection. [ABSTRACT FROM AUTHOR]

PURPOSE: To evaluate the association between birth characteristics, including parental sociodemographic characteristics, and early-onset pituitary adenoma (PA) and craniopharyngioma. METHODS: Leveraging the population-based California Linkage Study of Early-onset Cancers, we identified the birth characteristics of incident cases with PA (n = 1,749) or craniopharyngioma (n = 227) who were born from 1978 to 2015 and diagnosed 1988-2015, as well as controls in a 50:1 ratio matched on birth year. Adjusted odds ratios (OR) and 95% confidence interval (CI) estimates were computed using unconditional multivariable logistic regression. RESULTS: Males had a lower risk of PA than females (OR = 0.37, 95%CI: 0.34-0.41), and Black (OR = 1.55, 95%CI: 1.30-1.84) or Hispanic (OR = 1.53, 95%CI: 1.34-1.74) individuals had a higher risk compared to non-Hispanic Whites. Older maternal age was positively associated with PA (OR = 1.09, 95%CI: 1.04-1.15 per 5 years, p

A recent Pituitary Society consensus statement provided clinical recommendations for diagnosing and managing prolactin-secreting pituitary adenomas, known as prolactinomas. Prolactinomas are rare, usually benign tumours of the anterior pituitary gland that result in excessive prolactin production, or hyperprolactinemia. Despite being non-life- -threatening, patients often prompt medical attention due to symptoms stemming from elevated prolactin levels, such as reproductive and sexual dysfunction, e.g. menstrual irregularities, galactorrhoea, infertility, or from mass effects like headaches, cranial nerve palsies, and vision problems. Laboratory testing showing hyperprolactinemia and low sex hormone levels is used for diagnosis. Unlike other pituitary tumours, the primary treatment for prolactinomas is typically pharmacological therapy with dopamine agonists. However, for patients with smaller tumours (microprolactinomas) or well-circumscribed larger tumours (macroprolactinomas), surgery may be considered the first-line option. It is a surprising shift in the therapeutic strategy. For patients resistant to dopamine agonists, alternative strategies include switching medications, increasing dosages, or pursuing neurosurgery or radiotherapy. The key treatment goals are to normalize prolactin levels, restore reproductive and sexual function, and reduce tumour size. This review aimed to provide clinicians with an up-to-date overview of diagnostics and treatment options for patients with prolactin- -secreting pituitary adenomas, as well as highlight the potential diagnostic challenges and complexity of treatment. [ABSTRACT FROM AUTHOR]

Enhanced Recovery After Surgery (ERAS) is a perioperative model of care aimed at optimizing postoperative rehabilitation and reducing hospital length of stay (LOS). Decreasing LOS avoids hospital-acquired complications, reduces cost of care, and improves patient satisfaction. Given the lack of ERAS protocols for endoscopic endonasal transsphenoidal surgery (EETS) resection of pituitary adenomas, a systematic review of EETS was performed to compile patient outcomes and analyze factors that may lead to increased LOS, reoperation, and readmission rates with the intention to contribute to the development of a successful ERAS protocol for EETS. The authors performed a Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines-based systematic review of the literature. Information was extracted regarding patient LOS, surgery complications, and readmission/reoperation rates. Pearson's correlations to LOS and reoperation/readmission rates were performed with variables normalized to the number of participants. Statistical significance was set at P value <0.05. Fourteen studies were included, consisting of 2083 patients. The most common complications were cerebrospinal fluid leaks (37%) and postoperative diabetes insipidus (DI) (9%). Transient DI was significantly correlated with shorter LOS. Functional pituitary adenomas were significantly correlated with lower readmission rates while nonfunctional pituitary adenomas were correlated with higher readmission rates. No other factor was found to be significantly correlated with a change in LOS or reoperation rate. EETS may be an ideal candidate for the development of ERAS cranial protocols. While our data largely supports the safe implementation of shortened LOS protocols in EETS, our findings highlight the importance of transient DI and nonfunctional pituitary adenomas management when formulating ERAS protocols. [ABSTRACT FROM AUTHOR]

BACKGROUND: Craniopharyngiomas, primary brain tumors of the pituitary-hypothalamic axis, can cause clinically significant sequelae. Treatment with the use of surgery, radiation, or both is often associated with substantial morbidity related to vision loss, neuroendocrine dysfunction, and memory loss. Genotyping has shown that more than 90% of papillary craniopharyngiomas carry BRAF V600E mutations, but data are lacking with regard to the safety and efficacy of BRAF-MEK inhibition in patients with papillary craniopharyngiomas who have not undergone previous radiation therapy. METHODS: Eligible patients who had papillary craniopharyngiomas that tested positive for BRAF mutations, had not undergone radiation therapy previously, and had measurable disease received the BRAF-MEK inhibitor combination vemurafenib-cobimetinib in 28-day cycles. The primary end point of this single-group, phase 2 study was objective response at 4 months as determined with the use of centrally determined volumetric data. RESULTS: Of the 16 patients in the study, 15 (94%; 95% confidence interval [CI], 70 to 100) had a durable objective partial response or better to therapy. The median reduction in the volume of the tumor was 91% (range, 68 to 99). The median follow-up was 22 months (95% CI, 19 to 30) and the median number of treatment cycles was 8. Progression-free survival was 87% (95% CI, 57 to 98) at 12 months and 58% (95% CI, 10 to 89) at 24 months. Three patients had disease progression during follow-up after therapy had been discontinued; none have died. The sole patient who did not have a response stopped treatment after 8 days owing to toxic effects. Grade 3 adverse events that were at least possibly related to treatment occurred in 12 patients, including rash in 6 patients. In 2 patients, grade 4 adverse events (hyperglycemia in 1 patient and increased creatine kinase levels in 1 patient) were reported; 3 patients discontinued treatment owing to adverse events. CONCLUSIONS: In this small, single-group study involving patients with papillary craniopharyngiomas, 15 of 16 patients had a partial response or better to the BRAF-MEK inhibitor combination vemurafenib-cobimetinib. (Funded by the National Cancer Institute and others; ClinicalTrials.gov number, NCT03224767.).

The majority of corticotroph adenomas are benign but some are locally invasive, demonstrate high rates of recurrence, and exhibit a relatively poor response to often repeated surgical, medical, and radiation treatment. Herein, we summarize the currently known somatic and genetic mutations and other molecular factors that influence the pathogenesis of these tumors and discuss currently available therapies. Although recent molecular studies have advanced our understanding of the pathogenesis and behavior of these refractory corticotroph adenomas, these insights do not reliably guide treatment choices at present. Development of additional diagnostic tools and novel tumor-directed therapies that offer efficacious treatment choices for patients with refractory corticotroph adenomas are needed.

Abstract Background To evaluate the clinical usefulness of thin-slice echo-planar imaging (EPI)-based diffusion-weighted imaging (DWI) with an on-console distortion correction technique, termed reverse encoding distortion correction DWI (RDC-DWI), in patients with non-functioning pituitary neuroendocrine tumor (PitNET)/pituitary adenoma. Methods Patients with non-functioning PitNET/pituitary adenoma who underwent 3-T RDC-DWI between December 2021 and September 2022 were retrospectively enrolled. Image quality was compared among RDC-DWI, DWI with correction for distortion induced by B 0 inhomogeneity alone (B0-corrected-DWI), and original EPI-based DWI with anterior-posterior phase-encoding direction (AP-DWI). Susceptibility artifact, anatomical visualization of cranial nerves, overall tumor visualization, and visualization of cavernous sinus invasion were assessed qualitatively. Quantitative assessment of geometric distortion was performed by evaluation of anterior and posterior displacement between each DWI and the corresponding three-dimensional T2-weighted imaging. Signal-to-noise ratio (SNR), contrast-to-noise ratio (CNR), and apparent diffusion coefficient values were measured. Results Sixty-four patients (age 70.8 ± 9.9 years [mean ± standard deviation]; 33 females) with non-functioning PitNET/pituitary adenoma were evaluated. In terms of susceptibility artifacts in the frontal and temporal lobes, visualization of left trigeminal nerve, overall tumor visualization, and anterior displacement, RDC-DWI performed the best and B0-corrected-DWI performed better than AP-DWI. The right oculomotor and right trigeminal nerves were better visualized by RDC-DWI than by B0-corrected-DWI and AP-DWI. Visualization of cavernous sinus invasion and posterior displacement were better by RDC-DWI and B0-corrected-DWI than by AP-DWI. SNR and CNR were the highest for RDC-DWI. Conclusions RDC-DWI achieved excellent image quality regarding susceptibility artifact, geometric distortion, and tumor visualization in patients with non-functioning PitNET/pituitary adenoma. Relevance statement RDC-DWI facilitates excellent visualization of the pituitary region and surrounding normal structures, and its on-console distortion correction technique is convenient. RDC-DWI can clearly depict cavernous sinus invasion of PitNET/pituitary adenoma even without contrast medium. Key points • RDC-DWI is an EPI-based DWI technique with a novel on-console distortion correction technique. • RDC-DWI corrects distortion due to B 0 field inhomogeneity and eddy current. • We evaluated the usefulness of thin-slice RDC-DWI in non-functioning PitNET/pituitary adenoma. • RDC-DWI exhibited excellent visualization in the pituitary region and surrounding structures. • In addition, the on-console distortion correction of RDC-DWI is clinically convenient. Graphical Abstract

Pituitary prolactinoma is a neuroendocrine disease caused by excessive pituitary prolactin-cell adenoma synthesis and prolactin secretion. The standardized diagnosis and treatment of pituitary prolactinoma in of great significance for recovering and maintaining pituitary function and improving life quality of the patients. In January 2022, the International Chapter of Clinical Endocrinology (ICCE) and the Italian Association of Clinical Endocrinologists (AME) published a position statement on pituitary prolactinoma in the European Journal of Endocrinology: 2022 ICCE/AME Position Statement for Clinical Practice in Pituitary Prolactinoma (abbreviated as 2022 ICCE/AME position statement) . Based on the latest medical evidence, 2022 ICCE/AME position statement conducts a systematic review, analysis and recommendations on the clinical diagnosis and treatment of pituitary prolactinoma. This article provides an update on the diagnosis and treatment of pituitary prolactinoma, special populations, dopamine agonist resistance, and invasive prolactinoma in the 2022 ICCE/AME position statement, which is hoped to be helpful to the awareness of pituitary prolactinoma among general practitioners and endocrinologists, and provide a reference for the clinical practice of standardized diagnosis and treatment.

Background Delayed postoperative hyponatremia (DPH) is the most common cause of readmission after pituitary surgery. In this study, we aimed to evaluate the cutoff values of serum copeptin and determine the optimal timing for copeptin measurement for the prediction of the occurrence of DPH in patients who undergo endoscopic transsphenoidal approach (eTSA) surgery and tumor resection. Methods This was a prospective observational study of 73 patients who underwent eTSA surgery for pituitary or stalk lesions. Copeptin levels were measured before surgery, 1 hour after extubation, and on postoperative days 1, 2, 7, and 90. Results Among 73 patients, 23 patients (31.5%) developed DPH. The baseline ratio of copeptin to serum sodium level showed the highest predictive performance (area under the curve [AUROC], 0.699), and its optimal cutoff to maximize Youden’s index was 2.5×10–11, with a sensitivity of 91.3% and negative predictive value of 92.0%. No significant predictors were identified for patients with transient arginine vasopressin (AVP) deficiency. However, for patients without transient AVP deficiency, the copeptin-to-urine osmolarity ratio at baseline demonstrated the highest predictive performance (AUROC, 0.725). An optimal cutoff of 6.5×10–12 maximized Youden’s index, with a sensitivity of 92.9% and a negative predictive value of 94.1%. Conclusion The occurrence of DPH can be predicted using baseline copeptin and its ratio with serum sodium or urine osmolarity only in patients without transient AVP deficiency after pituitary surgery.

Background: To evaluate the clinical usefulness of thin-slice echo-planar imaging (EPI)-based diffusion-weighted imaging (DWI) with an on-console distortion correction technique, termed reverse encoding distortion correction DWI (RDC-DWI), in patients with non-functioning pituitary neuroendocrine tumor (PitNET)/pituitary adenoma. Methods: Patients with non-functioning PitNET/pituitary adenoma who underwent 3-T RDC-DWI between December 2021 and September 2022 were retrospectively enrolled. Image quality was compared among RDC-DWI, DWI with correction for distortion induced by B0 inhomogeneity alone (B0-corrected-DWI), and original EPI-based DWI with anterior-posterior phase-encoding direction (AP-DWI). Susceptibility artifact, anatomical visualization of cranial nerves, overall tumor visualization, and visualization of cavernous sinus invasion were assessed qualitatively. Quantitative assessment of geometric distortion was performed by evaluation of anterior and posterior displacement between each DWI and the corresponding three-dimensional T2-weighted imaging. Signal-to-noise ratio (SNR), contrast-to-noise ratio (CNR), and apparent diffusion coefficient values were measured. Results: Sixty-four patients (age 70.8 ± 9.9 years [mean ± standard deviation]; 33 females) with non-functioning PitNET/pituitary adenoma were evaluated. In terms of susceptibility artifacts in the frontal and temporal lobes, visualization of left trigeminal nerve, overall tumor visualization, and anterior displacement, RDC-DWI performed the best and B0-corrected-DWI performed better than AP-DWI. The right oculomotor and right trigeminal nerves were better visualized by RDC-DWI than by B0-corrected-DWI and AP-DWI. Visualization of cavernous sinus invasion and posterior displacement were better by RDC-DWI and B0-corrected-DWI than by AP-DWI. SNR and CNR were the highest for RDC-DWI. Conclusions: RDC-DWI achieved excellent image quality regarding susceptibility artifact, geometric distortion, and tumor visualization in patients with non-functioning PitNET/pituitary adenoma. Relevance statement: RDC-DWI facilitates excellent visualization of the pituitary region and surrounding normal structures, and its on-console distortion correction technique is convenient. RDC-DWI can clearly depict cavernous sinus invasion of PitNET/pituitary adenoma even without contrast medium. Key points: • RDC-DWI is an EPI-based DWI technique with a novel on-console distortion correction technique. • RDC-DWI corrects distortion due to B0 field inhomogeneity and eddy current. • We evaluated the usefulness of thin-slice RDC-DWI in non-functioning PitNET/pituitary adenoma. • RDC-DWI exhibited excellent visualization in the pituitary region and surrounding structures. • In addition, the on-console distortion correction of RDC-DWI is clinically convenient. [ABSTRACT FROM AUTHOR]