OBJECTIVE: Cushing syndrome (CS) is the result of chronic exposure to glucocorticoid excess. CS in children is most often caused by the administration of exogenous steroids. Endogenous CS is rare in the paediatric population and is caused mainly by tumours of the pituitary and adrenal glands, with ectopic sources being extraordinarily rare before the age of 18 years. In addition, children and young adults with CS present with different epidemiology, management issues, prognosis and outcomes than older adult patients. This complex disorder needs early diagnosis and management to avoid the significant morbidity and even mortality that can result from chronic untreated CS.
METHODS: In this review, we present the complex case of a 7-year-old boy with CS that highlights the diagnostic and management challenges of paediatric CS patients, including the considerations for genetic predisposition and life-long consequences of CS in children and young adults.
RESULTS: The diagnostic protocols for the evaluation of CS have been devised for adults and tested predominantly on adults. In this review, we discuss necessary modifications so that the testing can be adjusted for use in children. Additionally, pituitary adenomas in children are generally smaller and thus more difficult to recognize on pituitary imaging.
CONCLUSIONS: The management of the case and its complexities underline the need for children with CS to be managed in a centre with experienced paediatric endocrinologists and skilled neurosurgeons both for their initial diagnosis and treatment as well as for their long-term follow-up and management.

This study reviews recent progress in the surgical treatment of Rathke\'s cleft cysts (RCCs) and Sellar region meningiomas, based on findings from three key studies. RCCs are benign, fluid-filled remnants from pituitary gland development that are usually asymptomatic and found by chance. However, surgical intervention is needed when they become symptomatic or increase in size. Research by Stefan Linsler et al. and others examines various surgical methods, including transcranial keyhole and transsphenoidal techniques for RCCs, and endoscopic endonasal and supraorbital keyhole approaches for Sellar meningiomas. The results show that both transcranial keyhole and transsphenoidal surgeries for RCCs have high success rates with no recurrences over 5.7 years, although the keyhole approach has fewer complications. For Sellar meningiomas, the choice between endoscopic endonasal and supraorbital keyhole techniques should be based on tumor characteristics, highlighting the importance of surgeon proficiency in both methods. These studies emphasize the need for personalized treatment strategies tailored to patient and tumor characteristics and highlight the importance of ongoing surgical skill development and further research to refine minimally invasive techniques. This study highlights the crucial role of personalized surgical approaches in improving outcomes for patients with RCCs and Sellar region meningiomas.

Pituitary apoplexy (PA) is a clinical syndrome caused by acute hemorrhage and/or infarction of the pituitary gland, most commonly in the setting of a pituitary macroadenoma. PA generally presents with severe headache, nausea, vomiting, visual disturbance, and, in more severe cases, altered mental status. Many factors have been attributed to the risk of developing PA, including most recently, numerous reports showcasing an association with COVID-19 infection or vaccination. Initial management of PA includes evaluation and correction of deficient hormones and electrolytes and an assessment if surgical decompression to relieve pressure on optic nerves and other brain structures is needed. While prompt recognition and treatment are crucial to avoid morbidity and mortality, in the modern era, PA is less commonly considered a true neurosurgical emergency requiring immediate (< 24 h) surgical decompression. Traditionally, surgical decompression has been the standard of care for significant mass effects. However, several studies have shown similar outcomes in visual and hormonal recovery with either surgical decompression or conservative medical management. Unfortunately, most evidence on optimal management strategies is limited to retrospective case series, small prospective studies, and one multi-center observational study. This review aims to provide the most up-to-date evidence on the role of COVID-19 in PA and best management strategies.

BACKGROUND: Prolactinoma account to the most common pituitary adenomas and current therapy regime constitutes of dopamine agonist therapy (DA) and surgery in selected cases [17]. Due to tumor fibrosis induced by previous DA therapy, surgical removal can be challenging though. Therefore, this study investigates how preoperative DA usage influences perioperative treatment and surgical outcome in prolactinoma and aims to ascertain whether a specific subgroup of prolactinoma patients could derive greater benefit from exclusive surgical intervention.
METHODS: We retrospectively analyzed n = 159 surgically treated and histologically confirmed prolactinomas in the sella region from 2013-2022 in our institution. Clinical, radiological and surgical features were analyzed. Univariate and multivariate analyses were performed.
RESULTS: Out of total of 159 prolactinoma patients, 83.6% received previous treatment with DA followed by surgery, while only 16.4% received exclusive surgery. Both groups presented similar initial tumor volumes (1.9cm3 vs. 1.5cm3, p = 0.59) and equal preoperative prolactin levels (PRL) (199.7 µg/l vs. 191.0 µg/l, p = 0.44). Surgical procedures took significantly longer when patients received prior DA treatment (79 min. vs. 70 min., p = 0.0479). Six months after surgery, pretreated patients revealed significantly higher PRL compared to non-treated (107 g/l vs. 8.64 µg/, p = 0.0009). Additionally, untreated microprolactinoma presented a remission of 100%, whereas pretreated exhibited a remission rate of 88.75%.
CONCLUSIONS: The current study demonstrates that prior DA treatment is associated with significantly longer surgeries, higher recurrence rates and lower rates of normalization of PRL levels after surgery, particularly in microprolactinomas and support the latest recommendations of the Pituitary Society\'s Consensus Statement 2023, which favors the option of surgery alone as first-line therapy for microprolactinomas.

OBJECTIVE: Erectile dysfunction (ED) is frequently underreported in men suffering from prolactinomas and can be challenging to manage. Both dopamine agonists (DAs) and transsphenoidal surgery (TSS) correct hyperprolactinemia and restore gonadal function. However, there is scarce data regarding their effectiveness in correcting ED over the long term.
METHODS: This study is a retrospective single-center comparative cohort study analyzing men diagnosed with prolactinomas, both with and without confirmed erectile dysfunction (ED) at diagnosis. Independent risk factors for persistent ED over the long term were examined using multivariate logistic regression.
RESULTS: Among the 39 men with lactotroph adenomas, ED was one of the presenting symptoms in 22 (56%). The mean age at diagnosis was 45 ± 12 years. Surgery was the primary treatment in 6 (27%) ED patients and 8 (47%) non-ED patients. After a mean follow-up of 74 ± 48 months, remission from hyperprolactinemia was achieved in the majority (76%) of men: 71% in the non-ED cohort and 81% in the ED group (p = 0.70), regardless of the primary treatment strategy (surgical 84% versus medical 72%, p = 0.46). Long-term remission of ED was noted in 16 (73%) patients. Interestingly, high baseline BMI levels emerged as potential risk factors for persistent ED over the long term (OR 1.4, 95%CI 1.0-1.9; p = 0.04), while neither the initial adenoma size nor the primary treatment strategy (i.e., TSS vs. DAs) reached statistical significance.
CONCLUSIONS: Correcting hyperprolactinemia and its associated hypogonadism significantly improves ED in the majority of men with prolactinomas over the long term, regardless of the primary treatment strategy employed. In addition to addressing endocrine deficiencies, the early initiation of weight control programs may be considered for men with lactotroph adenomas and ED. Although our study suggests an association between BMI and the risk of persistent ED, further research is needed to establish any causal relationships.

BACKGROUND: Advances in endoscopic endonasal transsphenoidal surgery have led to improved postoperative outcomes after pituitary adenoma resection, including reduced length of stay, complications and readmission rates, without compromising safety and satisfaction.
METHODS: Our team implemented a perioperative protocol in January 2021 for patients undergoing endoscopic, transsphenoidal pituitary surgery. This study compares preoperative characteristics and postoperative outcomes in 279 patients between 2016 and 2022 (128 preprotocol and 151 postprotocol). Our protocol includes interdisciplinary preoperative evaluations, unified communication, cortisol thresholds for postoperative glucocorticoid replacement, and fluid restriction to prevent delayed hyponatremia.
RESULTS: Median age was 54 ± 17 years with 50.8% female patients. There were 229 (82.1%) macroadenomas (>1 cm) and 50 (17.9%) microadenomas/cysts (<1 cm). Mean diameter was 18 (transverse), 18 (craniocaudal), 16 (anteroposterior) mm. Tumor types included 125 (44.8%) gonadotroph, 46 (16.4%) adrenocorticotroph, 40 (14.3%) lactotroph, 26 (9.3%) Rathke cysts, 19 (6.8%) somatotroph, 13 (4.6%) nondiagnostic, 3 (1%) somatotroph-lactotroph, 3 (1%) mammosomatotroph, 2 (0.71%) null cell, and 2 (0.7%) thyrotroph adenomas. Postprotocol, 74.2% of patients were discharged on postoperative day 1 compared with 46.1% preprotocol (P < 0.0001). Transient arginine vasopressin deficiency decreased from 10.4% (preprotocol) to 4.6% postprotocol (P = 0.101). Hyponatremia occurred in 13.3% pre-protocol and 4.6% postprotocol. Emergency department visits dropped from 9.4% to 3.9%, and readmissions decreased from 7.8% to 2.6%. Persistent arginine vasopressin deficiency affected 2.3% preprotocol and 1.3% postprotocol patients. Cerebrospinal fluid leaks occurred in 8.5% preprotocol and 7.3% postprotocol.
CONCLUSIONS: Implementing an interdisciplinary, perioperative protocol for transsphenoidal endoscopic pituitary surgery improves length of stay while minimizing readmissions and surgery-related complications.

Background: Pituitary abscess (PA), a rare complication following transsphenoidal (TS) surgery for pituitary adenoma with an incidence of 0.2%, poses a significant risk; carrying potential morbidity, recurrence, and the necessity for reoperation. Timely suspicion, diagnosis, and treatment are imperative. Patients and Methods: We present two cases and provide a literature review on the symptoms, risk factors, diagnosis, treatment, and outcomes associated with secondary PAs following TS surgery for adenoma. Results: We identified 12 articles reporting a total of 45 cases, in addition to our 2 cases. The primary symptoms were headache and visual impairment, with no fever or specific infectious parameters observed. Predominant risk factors identified included cerebrospinal fluid (CSF) leakage and prior radiotherapy (RT). Our first patient, a 45-year-old male, presented 10 weeks after TS surgery with sudden-onset symptoms, whereas our second patient, a 64-year-old female, presented 22 years postoperatively. In the first case, intraoperative CSF leakage, with the patient\'s history of allergic rhinitis and frequent nasal irrigation possibly contributed to the development of abscess. In the second case, RT was considered a potential risk factor. Severe headache and subclinical signs of infection associated with a cystic lesion of the pituitary gland were common findings. Both patients underwent endoscopic TS drainage and received appropriate antibiotic therapy, resulting in complete recovery without recurrence. Conclusions: When faced with severe headaches in a patient with a history of TS surgery for a pituitary adenoma, coupled with radiological evidence showing a cystic appearance with peripheral enhancement, taking a proactive approach to promptly identify and intervene in secondary PAs is essential for mitigating potential complications and optimizing patient outcomes.

BACKGROUND: Sellar masses are common intracranial neoplasms. Their clinical manifestations vary widely and include headache. We aimed to determine whether the prevalence and characteristics of headache in patients with sellar tumours differ from the general population and to investigate the effect of tumour resection on this complaint.
METHODS: We performed a prospective, controlled study in a single tertiary centre and included 57 patients that underwent transsphenoidal resection for a sellar mass (53% females, mean age 53.5 ± 16.4) and 29 of their partners (controls; 45% females, mean age 54.8 ± 14.9). Outcome measures were prevalence, characteristics and impact of headache 1 month preoperatively and at neurosurgical follow-up 3 months postoperatively.
RESULTS: Preoperatively, the prevalence of regular headache (≥1 time per month) was higher in patients than in controls (54% vs. 17%, p < 0.001), and patients scored higher on headache impact questionnaires (all p ≤ 0.01). At postoperative follow-up, headache prevalence decreased in both groups, but the decrease in regular headache frequency and impact was larger in patients than in controls, and no between-group differences remained.
CONCLUSIONS: More than half of patients with sellar tumours suffer from at least once-monthly headaches, and both regular headache occurrence and impact are higher compared with controls. The more pronounced decrease in headache complaints in patients versus controls at postoperative follow-up suggests an additional effect of tumour resection next to the factor time.

UNASSIGNED: Cerebral vasospasm is a rare postoperative complication of transsphenoidal pituitary adenoma surgery with potentially severe consequences. These vasospasms generally have a delayed presentation at a mean of 8 postoperative days. We report an unusual case of hyperacute onset of cerebral vasospasm that occurred immediately after surgery.
UNASSIGNED: A 38-year-old man underwent endoscopic transsphenoidal surgery for a nonfunctioning pituitary adenoma. The patient experienced mild subarachnoid hematoma during surgery. Three hours after surgery, he developed rightward conjugate eye deviation and complete paralysis of the left upper and lower extremities. Diagnostic imaging revealed cerebral vasospasm in both middle cerebral arteries, and symptoms improved after intra-arterial administration of fasudil hydrochloride.
UNASSIGNED: There is a need for prompt diagnosis and therapeutic intervention when typical symptoms of cerebral vasospasm, such as paralysis, occur at any time during the postoperative course.

Objective  Biochemical remission rates of endoscopic endonasal transsphenoidal surgery (EETS) and its associated predictive factors were evaluated in patients with somatotrophin pituitary adenomas. Methods  The patients who underwent EETS in Jinling Hospital were identified between 2011 and 2020. The surgeons\' experience, preoperative insulin-like growth factor 1 (IGF-1), basal growth hormone (GH) levels, nadir GH levels, and the tumor characteristics were analyzed for their relationships with endocrine outcomes. Total 98 patients were included for single factor analysis and regression analysis. They were divided into three groups according to the admission chronologic order. Results  The overall remission rate of the patients was 57% (56/98) for all the patients over 10 years. In the single factor analysis, we found that the tumor size, cavernous invasion, and sellar invasion were valuable to predict the endocrine outcome after surgery. As for the suprasellar invasion, no significant difference was found between the noninvasive group and the invasive group. The preoperative IGF-1 level ( p  = 0.166), basal GH level ( p  = 0.001), and nadir GH level ( p  = 0.004) were also different between the remission group and the nonremission group in the single factor analysis. The logistic regression analysis indicated that the preoperative nadir GH (odds ratio = 0.930, 95% confidence interval = 0.891-0.972, p  = 0.001) was a significant predictor for the endocrine outcomes after surgery. Conclusion  The surgeons\' experience is an important factor that can affect the patients\' endocrine outcomes after surgery. The macroadenomas with lateral invasion are more difficult to cure. Patients with higher preoperative nadir GH levels are less likely to achieve remission.