Hormone Deficits Research Articles

Early diagnosis and postoperative follow-up of 50 Chinese children with craniopharyngioma.

Craniopharyngioma is a relatively common congenital intracranial tumor for children. But only few available studies focused on the endocrine evaluation before diagnosis and postoperative endocrine evaluations of children with craniopharyngioma. The aim of this study was to aid in the early diagnosis of craniopharyngioma (CP) and follow-up postoperative children suffered from craniopharyngioma. Craniopharyngioma patients, as the CP group (N.=50), and healthy children, as the control group (N.=30), the symptoms and pituitary hormone levels were reviewed and investigated. The preoperative levels of peak of GH, IGF-1, FT4, ACTH, COR and PRL of CP patients were significantly lower than those of the control group (all the P≤0.001). Levels of pituitary-hormones after surgery were significantly lower than both those before surgery and those of the control group (all the P≤0.001). HGH treatment could significantly improve the growth velocity of postoperative children (3.8±1.5 cm/year vs 13.0±3.4 cm/year for males, P≤0.001; 4.0±1.3 cm/year vs 12.7±1.8 cm/year for females, P≤0.001). Children presenting with endocrine disturbance symptoms combined with pituitary hormone deficits should be assessed by MRI to exclude craniopharyngioma earlier. Also, long-term follow-up study was very essential to craniopharyngioma survivors.

A Therapeutic Proposal for Mini-Puberty in Male Infants with Hypogonadotropic Hypogonadism: A Retrospective Case Series.

Background: Male patients with congenital hypogonadotropic hypogonadism (CHH) have impaired postnatal activation of the hypothalamic-pituitary-gonadal axis that occurs during mini-puberty. The aim of this study was to report our experience using gonadotropin replacement therapy for mini-puberty in male infants with CHH and to establish treatment recommendations. Methods: The patients included in this retrospective case series (n = 9) were diagnosed in the postnatal period due to micropenis, with two being accompanied by cryptorchidism and four with other associated hormonal deficits. All patients started treatment with gonadotropins early after diagnosis, between 2 weeks and 5 months of age, with a schedule of discontinuous injections with subcutaneous human chorionic gonadotropin (62.5-500 IU) two times per week and recombinant follicle-stimulating hormone-alpha (37.5-75 IU) three times per week. Results: The data from our study show an early response, ranging from almost undetectable levels of testosterone at diagnosis to elevated levels after starting treatment, as well as a positive clinical response with increases in testicular volume and penis size in all cases without requiring complementary treatment with testosterone esters and without adverse effects. Conclusions: Our results show that gonadotropin replacement therapy is a well-tolerated and effective treatment for testicular and penile problems in male patients with CHH.

PDOC-08 THE CLINICAL CHARACTERISTICS OF CHILDREN WITH CRANIOPHARYNGIOMA AND THEIR MANAGEMENT IN UGANDA: A RETROSPECTIVE REVIEW

Abstract BACKGROUND Craniopharyngiomas are benign brain tumors that mostly arise in the sellar/suprasellar region. Histologically though benign, these tumors can locally infiltrate with involvement of surrounding structures. They have a 5-year survival rate of above 80% in High Income Countries (HIC). Craniopharyngioma management includes surgery, radiotherapy and intracystic treatments, which requires a multi-disciplinary team. The morbidity associated with treatment must be weighed against tumor recurrence and quality of life after treatment. Endocrine deficiencies, especially growth hormone deficiency and visual deficits are common presenting features. OBJECTIVE To describe the clinical characteristics and management of children with craniopharyngioma managed at Mulago National Referral Hospital from March 2019 to December 2023. METHODOLOGY: A retrospective descriptive study was conducted among patients< 18 years of age, admitted to the Mulago National Referral Hospital (MNRH) from March 2019 to December 2023 with a radiological diagnosis of a craniopharyngioma. RESULTS A total of 151 children with a diagnosis of a brain tumor during this period were identified and of these 40 (26%) were diagnosed with craniopharyngioma. The median age was 8.4 years and 22 (55%) were males. Only 2 children had maximal safe resection (sub-total resection), one of whom later received adjuvant external beam radiotherapy. Thirty-eight patients had insertion of Ommaya reservoirs as their tumors were predominantly cystic. Two patients received intracystic bleomycin and 11 received radical external beam radiotherapy. Twenty-seven patients had no additional therapy after Ommaya reservoir placement. Fifteen (39%) of the patients had endocrine deficits with hypothyroidism, hypocortisolism and growth hormone deficiency being the most common. The median follow-up time was 1.45 years. The overall survival was 67.5%, 95%CI (42.5-83.5). CONCLUSION Management of patients with craniopharyngioma in Uganda is mostly conservative with the aim of intervention to preserve quality of life. Limited access to radiation therapy contributed to the poorer survival compared to HIC.

Endocrine sequelae after pediatric craniopharyngioma treatment: a single-center retrospective cohort study.

Craniopharyngiomas (CP) are rare brain tumors with a low mortality rate, but with significant morbidity, in part due to the various long-term endocrine sequelae related to hypothalamic/pituitary deficiencies. Our objective was to assess the prevalence of endocrine dysfunction and outcome after treatment of CP at our institution and to apply the novel diagnostic criteria for hypothalamic syndrome (HS). In addition, we give an overview of treatments already attempted for hypothalamic obesity (HO). This retrospective cohort study included children treated and followed up for CP at the pediatric oncology and endocrinology department at University Hospitals Leuven between January 2000 and December 2023. Clinical and endocrine characteristics were collected during a five-year period following diagnosis of CP. The Müller radiological criteria and the novel diagnostic criteria for HS were applied. A brief literature review regarding treatments already attempted for HO was conducted. Fifteen patients with pediatric CP were included in thestudy, all of whom developed endocrine sequelae over time. Seven patients (47 %) presented with at least one hormonal deficit, and eight patients (53 %) developed panhypopituitarism over time. HO was clinically confirmed in nine patients (60 %). 10 patients (67 %) met the diagnostic criteria for HS. Currently, no overall effective treatment strategies are available for HO. Long-term endocrine sequelae and HO are highly prevalent in pediatric CP. Continuing multidisciplinary care to improve the quality of life of these patients is necessary. International cooperation and further long-term prospective trials for the treatment of HO are needed.

Delayed onset arginine vasopressin deficiency after traumatic brain injury.

Delayed arginine vasopressin deficiency (AVP-D) can present in patients following traumatic brain injury (TBI) and may occur years after the trauma, presenting with nonspecific symptoms. The objective of this case is to highlight the importance of considering the delayed onset AVP-D in patients with a history of TBI. We report a case of a patient who had sustained severe traumatic brain injury 8 years before and who presented with polydipsia, behavioural disorder and frequent falls during the last 3 months. The diagnosis of AVP-D was confirmed by water restriction with a positive response to desmopressin, and pituitary MRI showed an absent spontaneous posterior hyperintensity on T1WI. Follow-up confirmed permanent diabetes insipidus as well as a suspected anterior pituitary deficiency. Pituitary dysfunction occurs following TBI and is correlated with severity. As in our case, symptoms are generally non-specific and are difficult to explore given the patient's neurologic sequelae. MRI 8 years post trauma showed changes in pituitary morphology. Some authors have proposed the need for active screening of post-TBI patients. This case highlights the need for clinicians to be aware that AVP-D can occur years after traumatic brain injury. Delayed onset post-traumatic arginine vasopressin deficiency may occur in patients with TBI and is correlated with the severity. The clinical picture is usually non-specific and diagnosis of AVP-D is challenging in non-verbal patients. An active screening for pituitary dysfunction is warranted in TBI patients and should be extended should one hormone deficit be identified.

Ophthalmologic symptoms as the first manifestation of primary complete empty sella syndrome- case report

Background: Empty sella syndrome (ESS) is a rare condition associated with loss of pituitary gland volume. ESS has a heterogeneous clinical manifestation including endocrine, neurological, and visual deficits. The most common symptoms include chronic headaches and hormonal disturbances associated with hypopituitarism.Aim of the study: To emphasize the role of ophthalmologists in the initial management of intracranial hypertension and the significance of a multidisciplinary approach in the management of ESS.Case report: Primary complete ESS was reported in a 32-year-old male presenting nonspecific ophthalmologic symptoms including ophthalmalgia and subjective worsening of the visual acuity. The ophthalmological exam revealed bilateral optic disk elevation. The subsequent MRI identified a suprasellar cistern invaginating within sella turcica with resulting pituitary gland flattening. A complete empty sella syndrome was confirmed. In addition, idiopathic intracranial hypertension was suspected. The patient was proposed hospitalization in the neurology department for additional testing however, he refused due to personal reasons.Conclusions: To date, only a few papers reported ESS with preliminary ophthalmological symptoms. All patients with nonspecific eye symptoms should have a complex ophthalmological examination. Bilateral optic disk elevation requires urgent imaging of the brain and orbits, and the golden-standard approach remains MRI. During the diagnostic process, the secondary causes of intracranial hypertension including tumor, bleeding, abscess, hydrocephalus, or venous sinus thrombosis must be excluded. Due to the heterogeneous clinical course of ESS, patients require multidisciplinary management and follow-up by endocrinologists, neurologists, neurosurgeons, and ophthalmologists.

Deciphering the Presentation and Etiologies of Hypophysitis Highlights the Need for Repeated Systematical Investigation

Abstract Context Hypophysitis is defined as an inflammation of the pituitary gland and/or infundibulum. Objective Our aim was to characterize the initial course and evolution of patients with hypophysitis according to the different etiologies. Methods Retrospective observational study conducted in a university referral hospital center. Patients over 15 years of age were included if they had a diagnosis of hypophysitis between January 2014 and October 2023, with the exclusion of hypophysitis secondary to immune checkpoint inhibitors. Results Sixty-one patients (64% women; median age, 34 years) were included. Polyuria–polydipsia, headache, and asthenia were present in 64%, 48%, and 44% of cases respectively. At diagnosis, at least 1 anterior pituitary deficiency was present in 91.5% of cases and vasopressin deficiency in 56%. Magnetic resonance imaging was abnormal in 97% of cases. Secondary hypophysitis was found in 46% of cases (n = 28), including sarcoidosis in 28% (n = 17) and L (Langerhans) group histiocytoses in 13.1% (n = 8). Among patients with secondary hypophysitis, pituitary deficiency preceded systemic manifestations in 23% and occurred concomitantly in 23% of cases. Patients were treated in 36% of cases (glucocorticoids, surgery), without improvement of pituitary hormone deficits. Conclusion A systemic etiology of hypophysitis was found in almost half of the patients. Pituitary disorders preceded the systemic disease in a quarter of the cases. This emphasizes the importance of a systematic repeated workup looking for a secondary etiology of hypophysitis in these patients.

Pituitary Apoplexy: Comorbidities, Management and Outcomes. A Spanish Observational Multicenter Study

Abstract Background Pituitary apoplexy (PA) is the paradigm of endocrine and neurosurgical emergency. Objective To evaluate the comorbidities, risk factors, clinical presentation, pituitary apoplexy score (PAS) and the outcomes of surgical vs. conservative management of PA in Spain. Methods Spanish multicenter, observational study of 301 patients with acute PA. Statistical analyses compared risk factors, clinical presentation and outcomes between the surgical and conservative treatment groups, adjusting for potential confounders. The prevalence of cardiovascular risk factors in patients with pituitary apoplexy was compared with the Spanish population and with patients with non-functioning pituitary adenomas. Results Median age was 59.3 years, 201 (66.8%) were men and non-functioning adenomas (77.9%) were the most common tumor type. The prevalence of diabetes (20.3% vs 13.9%, p<0.01), hypertension (48.8% vs 33.4%, p<0.01) and dyslipidemia (44.2% vs 23.3%, p<0.01), exceeded the Spanish age-adjusted population prevalence. Overall, 209 (69.4%) underwent surgery and 92 (30.6%) received conservative treatment. Surgical patients had larger tumors (26.2 vs 21.0 mm, p<0.01), chiasmal compression more frequently (77.2% vs 53.4%, p<0.01) and higher values of PAS. In the follow-up, while there were no statistically significant differences in anterior pituitary hormonal deficits between treatments, permanent vasopressin deficiency was more frequent after surgery (14.8% vs 3.3%, p<0.01). Conclusion There is a high burden of cardiovascular risk factors among patients with PA suggesting that metabolic factors may play a potential role in the development of PA. This underscores the need for comprehensive management of these conditions in addition to treating the apoplexy itself in this population. Surgical management has a relevant place in PA approach mainly in patients with higher PAS. However, it leads permanent vasopressin deficit more frequently than conservative approach.

Endocrine Disorders in Children with Primary Mitochondrial Diseases: Single-Center Experience.

Endocrine abnormalities may represent the only clinical manifestation of primary mitochondrial disorders. This study aimed to evaluate the endocrinological characteristics of mitochondrial disease in our cohort. A total of twenty-six pediatric patients diagnosed with mitochondrial disease were categorized on the basis of their specific genetic abnormalities. The auxologic data, pubertal development, and, based on their clinical symptoms, hormonal profiles were obtained. Twelve of the cohort of 26 patients (46%) were female. In 15 of the patients (57.6%), their mitochondrial disease (MD) was caused by nuclear DNA mutations (nDNA group). Four patients had Leigh syndrome, 2 patients had LHON syndrome, 2 patients had MELAS, and 1 patient had KSS clinical phenotype. The median age at diagnosis was 2.91 (0.59-16.8) years, and the median age at first endocrinologic evaluation was 4.62 (1.26-18) years. The mean height SDS was -1.34 ± 2.12, and the mean BMI SDS was -0.82 ± 1.96 for all patients. Of the 26 patients, 6 (23%) had a range of hormonal deficits. Ovarian insufficiency, central adrenal insufficiency, central hypothyroidism, diabetes mellitus, and critical illness-related adrenal insufficiency were all observed. Three of the patients were initially monitored in the endocrine clinic for hormone deficiencies but it was later determined that the hormonal abnormalities were caused by underlying mitochondrial disease. Individuals diagnosed with mitochondrial disease, particularly those with specific genetic abnormalities, are considered a high-risk group for developing hormonal deficits. Endocrine diseases could be one of the primary mitochondrial disorders' early warning symptoms.

Video-Based Performance Analysis in Pituitary Surgery - Part 2: Artificial Intelligence Assisted Surgical Coaching

Superior surgical skill improves surgical outcomes in endoscopic pituitary adenoma surgery. Video-based coaching programs, pioneered in professional sports, have shown promise in surgical training. In this study, we developed and assessed a video-based coaching program using artificial intelligence (AI) assistance. An AI-assisted video-based surgical coaching was implemented over 6months with the pituitary surgery team. The program consisted of 1) monthly random video analysis and review; and 2) quarterly 2-hour educational meetings discussing these videos and learning points. Each video was annotated for surgical phases and steps using AI, which improved video interactivity and allowed the calculation of quantitative metrics. Primary outcomes were program feasibility, acceptability, and appropriateness. Surgical performance (via modified Objective Structured Assessment of Technical Skills) and early surgical outcomes were recorded for every case during the 6-month coaching period, and a preceding 6-month control period. Beta and logistic regression were used to assess the change in modified Objective Structured Assessment of Technical Skills scores and surgical outcomes after the coaching program implementation. All participants highly rated the program's feasibility, acceptability, and appropriateness. During the coaching program, 63 endoscopic pituitary adenoma cases were included, with 41 in the control group. Surgical performance across all operative phases improved during the coaching period (P < 0.001), with a reduction in new postoperative anterior pituitary hormone deficit (P= 0.01). We have developed a novel AI-assisted video surgical coaching program for endoscopic pituitary adenoma surgery - demonstrating its viability and impact on surgical performance. Early results also suggest improvement in patient outcomes. Future studies should be multicenter and longer term.

Defective thyroid hormone transport to the brain leads to astroglial alterations

Allan-Herndon-Dudley syndrome (AHDS) is a rare X-linked disorder that causes severe neurological damage, for which there is no effective treatment. AHDS is due to inactivating mutations in the thyroid hormone transporter MCT8 that impair the entry of thyroid hormones into the brain, resulting in cerebral hypothyroidism. However, the pathophysiology of AHDS is still not fully understood and this is essential to develop therapeutic strategies. Based on evidence suggesting that thyroid hormone deficit leads to alterations in astroglial cells, including gliosis, in this work, we have evaluated astroglial impairments in MCT8 deficiency by means of magnetic resonance imaging, histological, ultrastructural, and immunohistochemical techniques, and by mining available RNA sequencing outputs. Apparent diffusion coefficient (ADC) imaging values obtained from magnetic resonance imaging showed changes indicative of alterations in brain cytoarchitecture in MCT8-deficient patients (n = 11) compared to control subjects (n = 11). Astroglial alterations were confirmed by immunohistochemistry against astroglial markers in autopsy brain samples of an 11-year-old and a 30th gestational week MCT8-deficient subjects in comparison to brain samples from control subjects at similar ages. These findings were validated and further explored in a mouse model of AHDS. Our findings confirm changes in all the astroglial populations of the cerebral cortex in MCT8 deficiency that impact astrocytic metabolic and mitochondrial cellular respiration functions. These impairments arise early in brain development and persist at adult stages, revealing an abnormal distribution, density, morphology of cortical astrocytes, along with altered transcriptome, compatible with an astrogliosis-like phenotype at adult stages. We conclude that astrocytes are potential novel therapeutic targets in AHDS, and we propose ADC imaging as a tool to monitor the progression of neurological impairments and potential effects of treatments in MCT8 deficiency.

From Hypothalamic Obesity to Metabolic Dysfunction-Associated Steatotic Liver Disease: Physiology Meets the Clinics via Metabolomics.

Metabolic health is tightly regulated by neuro-hormonal control, and systemic metabolic dysfunction may arise from altered function of the hypothalamic-anterior pituitary axis (HAPA). Ancient experimental observations of hypothalamic obesity (HO) and liver cirrhosis occurring among animals subjected to hypothalamic injury can now be explained using the more recent concepts of lipotoxicity and metabolic dysfunction-associated steatotic liver disease (MASLD). Lipotoxicity, the range of abnormalities resulting from the harmful effects of fatty acids accumulated in organs outside of adipose tissue, is the common pathogenic factor underlying closely related conditions like hypothalamic syndrome, HO, and MASLD. The hormonal deficits and the array of metabolic and metabolomic disturbances that occur in cases of HO are discussed, along with the cellular and molecular mechanisms that lead, within the MASLD spectrum, from uncomplicated steatotic liver disease to steatohepatitis and cirrhosis. Emphasis is placed on knowledge gaps and how they can be addressed through novel studies. Future investigations should adopt precision medicine approaches by precisely defining the hormonal imbalances and metabolic dysfunctions involved in each individual patient with HO, thus paving the way for tailored management of MASLD that develops in the context of altered HAPA.

Childhood craniopharyngioma: a retrospective study of children followed in Hôpital Universitaire de Bruxelles.

Craniopharyngiomas (CPs) are benign brain tumors accounting for 5 - 11% of intracranial tumors in children. These tumors often recur and can cause severe morbidity. Postoperative radiotherapy efficiently controls and prevents progression and recurrence. Despite advancements in neurosurgery, endocrinological, visual, and neuropsychological complications are common and significantly lower the quality of life of patients. We performed a retrospective study, including all patients younger than sixteen diagnosed with CP between July 1989 and August 2022 and followed up in Hôpital Universitaire de Bruxelles. Nineteen children with CP were included, with median age of 7 years at first symptoms and 7.5 at diagnosis. Common symptoms at diagnosis were increased intracranial pressure (63%), visual impairment (47%), growth failure (26%), polyuria/polydipsia (16%), and weight gain (10.5%). As clinical signs at diagnosis, growth failure was observed in 11/18 patients, starting with a median lag of 1 year and 4 months before diagnosis. On ophthalmological examination, 27% of patients had papillary edema and 79% had visual impairment. When visual disturbances were found, the average preoperative volume was higher (p=0.039). Only 6/19 patients had gross total surgical resection. After the first neurosurgery, 83% experienced tumor recurrence or progression at a median time of 22 months. Eleven patients (73%) underwent postsurgical radiotherapy. At diagnosis, growth hormone deficiency (GHD) was the most frequent endocrine deficit (8/17) and one year post surgery, AVP deficiency was the most frequent deficit (14/17). Obesity was present in 13% of patients at diagnosis, and in 40% six months after surgery. There was no significant change in body mass index over time (p=0.273) after the first six months post-surgery. CP is a challenging brain tumor that requires multimodal therapy and lifelong multidisciplinary follow-up including hormonal substitution therapy. Early recognition of symptoms is crucial for prompt surgical management. The management of long-term sequelae and morbidity are crucial parts of the clinical path of the patients. The results of this study highlight the fundamental importance of carrying out a complete assessment (ophthalmological, endocrinological, neurocognitive) at the time of diagnosis and during follow-up so that patients can benefit from the best possible care.

GCT-18. GERMINOMAS OF THE CENTRAL NERVOUS SYSTEM (CNS): 20 YEARS EXPERIENCE WITH WHOLE VENTRICULAR IRRADIATION (WVI) AT FONDAZIONE IRCCS ISTITUTO NAZIONALE DEI TUMORI, MILAN

Abstract BACKGROUND Intracranial germ cell tumors account for 1-3% of pediatric CNS tumors, with germinomas constituting up to 65%, their prognosis being favourable compared to the non-germinomatous counterpart. METHODS After an unsuccessful experience with focal irradiation, from 2001 to 2021 we treated 68 patients (51 males/17 females, median age at diagnosis 16 years) with 4 PEB cycles (cisplatin 25 mg/sqm days 1-4, etoposide125 mg/sqm days 1-4, bleomycin 18 mg/sqm day 2, cycles every 21 days) followed by whole ventricular irradiation (WVI) 30,6 Gy/36Gy if CR/PR-SD. Patients with metastatic disease received craniospinal irradiation only (CSI 24 Gy+16Gy TB-tumor bed/WVI). RESULTS Fifty-nine patients had localized disease: 23 pineal, 22 suprasellar, 13 bifocal, 1 in the basal ganglia and 9 were metastatic. Suprasellar localization was associated with longer diagnostic delay. Five, 10 and 20 years OS and EFS were 100%, 97.5%, 97.5% and 95.3%, 92.8%.92.8% respectively. There was no statistical difference according to age at diagnosis, site and metastatic/bifocal disease. During follow-up 13/59 patients (22%) revealed moderate (2)/slight (11) impairment of the pulmonary function at spirometry assessment. Audiometric tests were available for 59 patients: 42 were normal (71%), 11 had a slight deficit, 4 moderate and 2 severe. Neurocognitive homogeneous evaluations were available only for 9 patients, so no clear correlation can be done, except observing the lowest scores in the patient with basal ganglia involvement. New endocrine deficits appeared in 9 patients after the treatment. CONCLUSIONS WVI tailored on response to chemotherapy proved effective in curing children CNS germinoma. Cisplatin and bleomycin-containing regimens induce high-response rates with known but in our experience limited sides effects. New collaborative protocols will investigate the reduction of the doses of irradiation and the use of less toxic drugs in order to achieve the same cure rates with less side effects.

CP-08. THE CLINICAL CHARACTERISTICS OF CHILDREN WITH CRANIOPHARYNGIOMA AND THEIR MANAGEMENT IN UGANDA: A RETROSPECTIVE REVIEW

Abstract BACKGROUND Craniopharyngiomas are benign brain tumors that mostly arise in the sellar/suprasellar region. Histologically though benign, these tumors can locally infiltrate with involvement of surrounding structures. They have a 5-year survival rate of above 80% in High Income Countries (HIC). Craniopharyngioma management includes surgery, radiotherapy and intracystic treatments and requires a multi-disciplinary team. The morbidity associated with treatment must be weighed against tumor recurrence and quality of life after treatment. Endocrine deficiencies, especially growth hormone deficiency and visual deficits are common presenting features. OBJECTIVE To describe the clinical characteristics and management of children with craniopharyngioma managed at Mulago National Referral Hospital from March 2019 to December 2023. METHODOLOGY A retrospective descriptive study was conducted among patients&amp;lt; 18 years of age, admitted to the Mulago National Referral Hospital (MNRH) from March 2019 to December 2023 with a radiological diagnosis of a craniopharyngioma. RESULTS A total of 151 children with a diagnosis of a brain tumor during this period were identified and of these 40 (26%) were diagnosed with craniopharyngioma. The median age was 8.4 years and 22 (55%) were males. Only 2 children had maximal safe resection (sub-total resection), one of whom later received adjuvant external beam radiotherapy. Thirty-eight patients had insertion of ommaya reservoirs as their tumors were predominantly cystic. Two patients received intracystic bleomycin and 11 received radical external beam radiotherapy. Twenty-seven patients had no additional therapy after ommaya reservoir placement. Fifteen (39%) of the patients had endocrine deficits with hypothyroidism, hypocortisolism and growth hormone deficiency being the most common. The median follow-up time was 1.45 years. The overall survival was 67.5%, 95%CI(42.5-83.5). CONCLUSION Management of patients with craniopharyngioma in Uganda is mostly conservative with the aim of intervention to preserve quality of life. Limited access to radiation therapy contributed to the poorer survival compared to HIC.

Comparing neuroendocrine recovery between surgical and conservative management in pituitary apoplexy patients: a propensity score-matched analysis.

Pituitary apoplexy is a rare and potentially life-threatening clinical syndrome. Patients may present with severeneuro-ophthalmologic or endocrine symptoms. Current evidence is unclear whether conservative or surgicalmanagement leads to the best neuroendocrine outcomes. This study aimed to compare neuroendocrine outcomesbetween surgical and conservative treatments in a single center. Cases of patients with pituitary apoplexy whoreceived transsphenoidal surgery or conservative management in Songklanagarind Hospital between January 1,2005 and December 31, 2022 were retrospectively reviewed. A propensity score matching method was used toadjust bias from treatment selection (surgery or conservative treatment). Differences in visual field, visual acuity,cranial nerve, and endocrine outcomes between the surgical and conservative treatment groups were analyzedusing logistic regression analysis. This study included 127 patients, with 98 and 29 patients in the surgical and theconservative treatment group, respectively. The optimal matching method was used for propensity score matching.Compared to the conservative group, the surgically treated patients had a significantly higher rate of visual fieldrecovery (odds ratio (OR): 12.89, P = 0.007). However, there were no statistical differences in the recovery rate ofpreoperative visual acuity, cranial nerve, and endocrine deficits between the groups. Transsphenoidal surgery wasassociated with a higher rate of visual field recovery when compared to the conservative treatment for pituitaryapoplexy patients. Careful selection of appropriate treatment based on the patient's presentation andneuroendocrine status will result in the best outcomes while avoiding unnecessary surgical intervention.

Management Approaches and Patient Outcomes for Giant Pituitary Neuroendocrine Tumors Classified as Knosp Grade 3 and 4.

Background Treatment of patients with a giant pituitary neuroendocrine tumor (GPitNET) is challenging. Here, we present the methods used for the clinical management of patients who underwent GPitNET resection mainly via endoscopic endonasal surgery along with multimodal supportto avoid surgical complications, which can affect the outcomes. Methodology The medical records of 25 patients with a GPitNET who underwent endonasal endoscopic surgery were retrospectively reviewed. Complications were analyzed and factors affecting the extent of resection were evaluated. Results Gross total resection was achieved in six (24%), near-total resection (>90%) in nine (36%), and partial resection in 10 (40%) patients. Multivariate analyses revealed that tumors invading the middle fossa had negative effects on the extent of resection (odds ratio = 0.092, p = 0.047). Postoperative vision improved or normalized in 16 (64%), remained stable in eight (32%), and worsened in one (4%), while a new hormonal deficit was noted in seven (28%) patients. Complications included permanent oculomotor nerve palsy in one (4%) and transient oculomotor palsy in one (4%), apoplexy of the residual tumor resulting in ischemic stroke in one (4%), postoperative cerebrospinal fluid leakage in one (4%), and permanent diabetes insipidus in six (24%) patients. Conclusions For GPitNETs that extend into the middle fossa, our study underscored the difficulties in surgical extraction and the necessity for tailored treatment approaches. To ensure the safest and most complete removal possible, the surgical strategy must be specifically adapted to each case. Additionally, employing a comprehensive support approach is essential to reduce the chance of complications in patients impacted by this condition.

Fully Endoscopic Minimally Invasive Trans-Eyebrow Supraorbital Translaminar Approach to Third Ventricle Craniopharyngiomas: Technical Nuances and Stepwise Illustrative Description

BackgroundTraditional microsurgical approaches for addressing intraventricular craniopharyngioma pose limited access to the retrochiasmatic area and tumors with significant lateral or rostrocaudal extensions. Extended endoscopic endonasal approaches can effectively overcome many of limitations, yet they require a favorable angle between the optic chiasm and pituitary gland, as well as the involvement of the third ventricle floor by the tumor. MethodsHerein, the authors describe the surgical nuances of a keyhole technique for resecting third ventricle craniopharyngiomas via a fully endoscopic minimally-invasive trans-eyebrow supraorbital translaminar approach (ESOTLA). A case description detailing the approach’s application is provided, along with a series of cadaveric photographs to highlight the relevant anatomy and step-by-step dissection process. ResultsThe patient is a 44-year-old man who presented with polyuria, low urine specific gravity, hypogonadism, and hypopituitarism. Brain MRI revealed a solid-cystic heterogeneous-enhanced retrochiasmatic mass within the third ventricle, consistent with craniopharyngioma. A one-stage ESOTLA was indicated based on the narrow corridor between the pituitary gland and optic chiasm and the high functional status of the patient. Near-total resection was achieved, and no new postoperative neurologic or endocrine deficit was observed. Targeted therapy was implemented based on the histologic result, and the most recent surveillance MRI showed no evidence of the residual tumor. ConclusionsBy combining a keyhole approach with variable-angle endoscopic visualization through a smaller bony and soft tissue exposure, ESOTLA can provide enhanced illumination within the third ventricle, potentially addressing cosmetic concerns and limited exposure area and angle of freedom associated with its conventional microsurgical counterpart.

Real-world experience with 11C-methionine positron emission tomography in the management of acromegaly.

L-[methyl-11C]-methionine-positron emission tomography (Met-PET) is a potentially important imaging adjunct in the diagnostic workup of pituitary adenomas, including somatotroph tumors. Met-PET can identify residual or occult disease and make definitive therapies accessible to a subgroup of patients who would otherwise require lifelong medical therapy. However, existing data on its use are still limited to small case series. Here, we report the largest single-center experience (n = 61) in acromegaly. A total of 189 cases of acromegaly were referred to our national Met-PET service in the last 12 years. For this analysis, we have reviewed outcomes in those 61 patients managed exclusively by our multidisciplinary team (single center, single surgeon). Referral indications were as follows: indeterminate magnetic resonance imaging (MRI; n = 38, 62.3%), occult residual (n = 14, 23.0%), (radio-)surgical planning (n = 6, 9.8%), and occult de novo tumor (n = 3, 4.9%). A total of 33/61 patients (54.1%) underwent PET-guided surgery. Twenty-four of 33 patients (72.7%) achieved complete biochemical remission following (re-)surgery. Insulin-like growth factor 1 levels were reduced to <2 × upper limit of normal (ULN) in 6 of the remaining 9 cases, 3 of whom achieved levels of <1.1 × ULN compared with mean preoperative levels of 2.4 × ULN (SD 0.8) for n = 9. Only 3 patients developed single new hormonal deficits (gonadotropic/thyrotropic insufficiency). There were no neurovascular complications after surgery. In patients with persistent/recurrent acromegaly or occult tumors, Met-PET can facilitate further targeted intervention (surgery/radiosurgery). This led to complete remission in most cases (24/33) or significant improvement with comparatively low risk of complications. L-[methyl-11C]-methionine-positron emission tomography should therefore be considered in all patients who are potential candidates for further surgical intervention but present no clear target on MRI.

Long-term outcomes and potential predictive recurrence factors after endonasal endoscopic surgical treatment of symptomatic Rathke's cleft cysts.

Although patients with symptomatic Rathke's cleft cysts (RCCs) receive surgical treatment, recurrence sometimes occurs after surgery. However, the mechanism underlying recurrence remains unclear. We evaluated the outcomes of RCC decompression over a long-term follow-up period. We retrospectively reviewed the medical records of 35 patients with symptomatic RCC who underwent endonasal endoscopic surgery (EES) at our institution between 2008 and 2023. Patients' characteristics, intraoperative findings, and postoperative follow-up outcomes were evaluated. A univariate regression model was used to identify the predictors of recurrence. The median patient age was 48.0years, and 74.2% of the patients were female. The mean follow-up duration was 94.7 ± 47.6months. Cyst content recurrence was observed in 15 patients (42.8%). Five patients (14.2%) with symptomatic recurrence underwent reoperation. Postoperative vision improved in all 23 patients (100%); headaches improved in 20 patients (90.9%). A new hormonal deficit occurred in two patients (5.7%). Complications included intraoperative cerebrospinal fluid (CSF) leak in 10 patients (28.5%), postoperative CSF leak in two patients (5.7%), permanent diabetes insipidus in two patients (5.7%), and postoperative infection in three patients (8.5%). Univariate analyses revealed that the position of the anterior pituitary lobe (p = 0.019) and preoperative visual disturbances (p = 0.008) significantly affected recurrence after surgery. Although EES was efficient, the recurrence rate was relatively high over a long-term period. The anterior pituitary lobe position and preoperative visual disturbances were significantly associated with recurrence. The anterior-inferior position can predict a high risk of recurrence before surgery.