Search Results (12)

Background: Long QT syndrome (LQTS) is characterized by delayed myocardial repolarization, predisposing to malignant arrhythmias such as torsades de pointes, ventricular fibrillation, and cardiac arrest. Recent reports suggest that acquired LQTS (aLQTS) may represent an early manifestation of hypopituitarism, potentially contributing to its increased cardiovascular mortality, although evidence remains limited to 16 published case reports. Objective: The objective was to investigate the relationship between hypopituitarism and corrected QT (QTc) interval. Methods: We retrospectively analyzed data from 185 patients (121 males) with hypothalamic–pituitary disorders who underwent a 12-lead electrocardiogram between April 2023 and September 2024. Clinical characteristics, hormone replacement therapy, and same-day laboratory parameters (electrolytes, fT3, fT4, IGF-I, testosterone) were recorded. QTc was calculated using Bazett’s formula. Multivariate logistic regression identified predictors of QTc prolongation. Results: Age (OR 1.07–1.09, p = 0.02) was a significant predictor in 5 of 8 models. The presence of expansive lesions other than pituitary adenomas, craniopharyngiomas, and Rathke’s cleft cysts was also associated with QTc prolongation (OR 8.35–17.73, p < 0.05 and p = 0.03). Potassium (OR 0.14–0.17, p = 0.09) and albumin-corrected calcium levels (OR 0.0003, p = 0.06) showed consistent, though borderline, associations. Conclusions: Age and the presence of expansive lesions other than pituitary adenomas, craniopharyngiomas, and Rathke’s cleft cysts are the main predictors of QTc duration in patients with hypothalamic–pituitary disease. Electrolyte imbalances—particularly low potassium and albumin-corrected calcium—may further contribute. The influence of specific pituitary deficiencies remains uncertain, likely due to adequate replacement therapy in most patients. Full article

Background/Objectives: Pituitary tumors account for over 90% of all sellar region masses. However, a spectrum of rare neoplastic, inflammatory, infectious, and vascular lesions—benign and malignant—can arise in the intra- and parasellar compartments and clinically and radiologically mimic PitNETs. We report a cohort of 47 such rare and cystic midline intracranial lesions, emphasizing their distinctive morphological, clinical, and imaging features and the personalized treatment strategies applied. Methods: In this retrospective single-center study, we reviewed all patients treated for suspected PitNETs via transsphenoidal approach between 2015 and 2024. Of 529 surgical cases, we excluded confirmed PitNETs, meningiomas, and classical intradural craniopharyngiomas. Collected data encompassed patient demographics, tumor characteristics, presenting symptoms, extent of resection or medical therapy, endocrine outcomes, and follow-up information. Results: Among all 529 patients who underwent surgical treatment for sellar lesions from 2015 to 2024, 47 cases (8.9%) were identified as rare or cystic masses. Forty-six underwent transsphenoidal resection; one patient with hypophysitis received corticosteroid therapy alone. Presenting symptoms included headache (n = 16), dizziness (n = 5), oculomotor disturbances (n = 2), and visual impairment (n = 17). Endocrine dysfunction was found in 30 patients, 27 of whom required hydrocortisone replacement. Histopathological diagnoses were led by colloid cysts (n = 14) and Rathke’s cleft cysts (n = 11). The remaining 22 cases comprised plasmacytoma, germinoma, lymphoma, pituicytoma, inverted papilloma, metastatic carcinoma, chordoma, nasopharyngeal carcinoma, chloroma, and other rare entities. Preoperative imaging diagnosis proved incorrect in 38% (18/47) of cases, with several lesions initially misidentified as PitNETs. Conclusions: Nearly 9% of presumed PitNETs were rare, often benign or inflammatory lesions requiring distinct management. Most could be safely resected and demonstrated excellent long-term outcomes. Yet, despite advanced imaging techniques, accurate preoperative differentiation remains challenging, with over one-third misdiagnosed. Clinical red flags—such as early hormone deficits, rapid progression or atypical imaging findings—should prompt early interdisciplinary evaluation and, when indicated, image-guided biopsy to avoid unnecessary surgery and ensure tailored therapy. Full article

Background: Differentiating pituitary adenoma (PA) from Rathke’s cleft cyst (RCC) on magnetic resonance imaging (MRI) remains challenging due to overlapping imaging features such as the cystic appearance, and location within the sellar region. A magnetic resonance image compilation (MAGiC) sequence can simultaneously acquire R1 and R2 relaxation rates. This study evaluated the diagnostic performance of MAGiC-driven imaging parameters for distinguishing PA from RCC. Methods: In total, 108 patients (54 each with PA or RCC) who underwent MRI using the MAGiC sequence were included in this study. The R1 and R2 relaxation rates were measured from the regions of interest identified in the MAGiC images. The relaxation rates between the PA and RCC groups were compared and diagnostic performance was assessed. Results: The relaxation rates of PA and RCC differed significantly. PA exhibited lower R1 (0.71 vs. 1.31, p < 0.001) and R2 (13.62 vs. 11.38, p = 0.029) relaxation rates than RCC. The R1 relaxation rate demonstrated superior diagnostic performance, with an area under the curve (AUC) of 0.74 (95% confidence interval [CI]: 0.65–0.82), surpassing the R2 rate (AUC 0.62; 95% CI: 0.52–0.71). The optimal threshold for R1 was determined to be 0.82, which provided a sensitivity of 79.6% and specificity of 74.1% (p < 0.001), whereas the optimal threshold for R2 was 14.89 (p = 0.030). Conclusions: MAGiC-driven parameters, particularly the R1 relaxation rate, show promise for improving the differentiation between PA and RCC. These findings suggest the potential for the broader application of MAGiC imaging in clinical practice to improve diagnostic accuracy. Full article

Background: Rathke’s cleft cysts (RCCs) are non-neoplastic and rare sellar lesions derived from remnants of Rathke’s pouch. While asymptomatic RCCs often require only conservative management, symptomatic cases may necessitate surgical intervention. The aim of our study is to investigate the correlations between clinical, radiological and histological features of RCCs to propose a novel management model. Methods: We conducted a retrospective analysis from patients who underwent surgery for cystic lesions between 2013 and 2023 in the Neurosurgery Department of Treviso Hospital (Italy) using for our purpose only those confirmed by histological specimen as RCCs. Results: 20 patients for a total of 24 procedures (20 primary surgeries/4 cases for recurrence) were performed for RCCs. A gross total resection was achieved in 23 cases, resulting in improvement of headache and visual symptoms in all patients. Hyperintensity on T1-weighted MRI sequences is more strongly correlated with inflammation and with intralesional metaplasia (p = 0.009). Both characteristics are involved in the development of hypopituitarism (p = 0.057), headache, and visual impairment (p = 0.082) compared to cysts with CSF-like content, even when the latter are smaller in size (p = 0.078). Discussion and Conclusions: RCCs are rare lesions whose management is challenging due to a lack of established guidelines. Intraoperative cystic content and MRI cystic characteristics seem to correlate with clinical presentation and long-term outcome in these patients. The transsphenoidal endoscopic approach is a safe and effective treatment, especially in cysts with inflammatory aspect in histopathological specimens and in dedicated MRI sequences that could take advantage of an early surgical resection. A decision-making model based on clinical, radiological and histopathological features of cysts could be useful to guide RCCs’ treatment, underlining the role of inflammation that seems to be involved in the onset of visual and hormonal impairment and in recurrence risk. Full article

Cystic lesions are common lesions of the sellar region with various pathological types, including pituitary apoplexy, Rathke’s cleft cyst, cystic craniopharyngioma, etc. Suggested surgical approaches are not unique when dealing with different cystic lesions. However, cystic lesions with different pathological types were hard to differentiate on MRI with the naked eye by doctors. This study aimed to distinguish different pathological types of cystic lesions in the sellar region using preoperative magnetic resonance imaging (MRI). Radiomics and deep learning approaches were used to extract features from gadolinium-enhanced MRIs of 399 patients enrolled at Peking Union Medical College Hospital over the past 15 years. Paired imaging differentiations were performed on four subtypes, including pituitary apoplexy, cystic pituitary adenoma (cysticA), Rathke’s cleft cyst, and cystic craniopharyngioma. Results showed that the model achieved an average AUC value of 0.7685. The model based on a support vector machine could distinguish cystic craniopharyngioma from Rathke’s cleft cyst with the highest AUC value of 0.8584. However, distinguishing cystic apoplexy from pituitary apoplexy was difficult and almost unclassifiable with any algorithms on any feature set, with the AUC value being only 0.6641. Finally, the proposed methods achieved an average Accuracy of 0.7532, which outperformed the traditional clinical knowledge-based method by about 8%. Therefore, in this study, we first fill the gap in the existing literature and provide a non-invasive method for accurately differentiating between these lesions, which could improve preoperative diagnosis accuracy and help to make surgery plans in clinical work. Full article

Background and Objectives: Pituitary abscess (PA) is a rare occurrence, representing less than 1% of pituitary lesions, and is defined by the presence of an infected purulent collection within the sella turcica. Pas can be classified as either primary, when the underlying pituitary is normal prior to infection, or secondary, when there is associated a pre-existing sellar pathology (i.e., pituitary adenoma, Rathke’s cleft cysts, or craniopharyngioma), with or without a recent history of surgery. Preoperative diagnosis, owing to both non-specific symptoms and imaging features, remains challenging. Treatment options include endonasal trans-sphenoidal pus evacuation, as well as culture and tailored antibiotic therapy. Methods: A retrospective multicenter study, conducted on a prospectively built database over a 20-year period, identified a large series of 84 patients harboring primary sellar abscess. The study aimed to identify crucial clinical and imaging features in order to accelerate appropriate management. Results: The most common clinical presentation was a symptom triad consisting of various degrees of asthenia (75%), visual impairment (71%), and headache (50%). Diagnosis was achieved in 95% of cases peri- or postoperatively. Functional recovery was good for visual disturbances and headache. Pituitary function recovery remained very poor (23%), whereas the preoperative diagnosis represented a protective factor. Conclusions: In light of the high prevalence of pituitary dysfunction following the management of PAs, early diagnosis and treatment might represent a crucial issue. Currently, there are no standard investigations to establish a conclusive preoperative diagnosis; however, new, emerging imaging methods, in particular nuclear imaging modalities, represent a very promising tool, whose potential warrants further investigations. Full article

Background: Rathke’s cleft cysts (RCC) arise from the pars intermedia because of incomplete regression of the embryologic Rathke pouch. A subset of RCC becomes symptomatic causing headaches, visual and endocrinological disturbances such that surgical intervention is indicated. Several points in surgical management remain controversial including operative strategy (simple fenestration (SF) vs complete cyst wall resection (CWR)) as well as reconstructive techniques. Methods: A retrospective analysis was conducted of pathologically confirmed RCC operated on by endoscopic endonasal approach from 2006 to 2019. Pre-operative symptoms, imaging characteristics, operative strategy, symptom response, complications and recurrences were recorded. Results: Thirty-nine patients were identified. Thirty-three underwent SF and six underwent CWR. Worsening pituitary function was significantly increased with CWR (50%) compared to SF (3%) (p = 0.008). All patients underwent “closed” reconstruction with a post-operative CSF leak rate of 5% (3% SF vs 16% CWR, p = 0.287). Six (15%) recurrences necessitating surgery were reported. Recurrence rates stratified by surgical technique (18% SF vs 0% CWR, p = 0.564) were not found to be significantly different. Conclusions: The current series illustrates variability in the surgical management of RCCs. SF with closed reconstruction is a reasonable operative strategy for most symptomatic RCCs cases while CWR can be reserved for selected cases. Full article

Pituitary incidentalomas are tumors or mass lesions of the pituitary gland. These are incidentally discovered during imaging studies for symptoms that are not causally related to pituitary diseases. The most common symptom that triggers an examination is headache, and the most common type of pituitary incidentalomas are pituitary neuroendocrine tumors (PitNETs) and Rathke cleft cysts. The existing treatment strategy is controversial; however, surgical resection is recommended in cases of clinically non-functioning PitNETs with optic chiasm compression. In contrast, cystic lesions, such as Rathke cleft cysts, should be followed if the patients are asymptomatic. In this case, MRI and pituitary function tests are recommended every six months to one year; if there is no change, the follow-up period should be extended. The natural history of PitNET is partially known, and the management of pituitary incidentalomas is determined by this history. However, the pathogenesis of PitNET has significantly changed with the new World Health Organization classification, and follow-up is important based on this new classification. Therefore, a high level of evidence-based research is needed to consider treatment guidelines for pituitary incidentalomas in the future. Full article

Xanthogranuloma of the sellar region is a rare chronic inflammatory lesion resulting from secondary hemorrhage, inflammation, infarction, and necrosis of an existing Rathke’s cleft cyst, craniopharyngioma, or pituitary adenoma. Sellar xanthogranulomas are challenging to differentiate from other cystic lesions preoperatively due to the lack of characteristic imaging features. We performed a literature overview of the clinical and paraclinical features, treatment options, and long-term outcomes of patients with sellar xanthogranuloma, focusing on the preoperative radiological diagnosis. The hyperintense signal in both T1- and T2-weighted sequences, cystic or partially cystic morphology, ovoid shape, sellar epicenter, intra- and suprasellar location, intratumoral calcifications, linear rim contrast enhancement, and the absence of cavernous sinus invasion suggest xanthogranuloma in the preoperative differential diagnosis. An endoscopic endonasal gross total resection without radiotherapy is the preferred first-line treatment. Given the low rate of recurrence rate and low chance of endocrinological recovery, a mass reduction with decompression of the optic apparatus may represent an appropriate surgical goal. Identifying the xanthogranulomas’ mutational profile could complement histopathological diagnosis and give insight into their histo-pathogenesis. A better preoperative neuroimagistic diagnosis of sellar xanthogranulomas and differentiation from lesions with a poorer prognosis, such as craniopharyngioma, would result in an optimal personalized surgical approach. Full article

The aim of this study was to assess the diagnostic value of non-contrast pituitary MRI in children with growth or puberty disorders (GPDs) and to determine the criteria indicating the necessity to perform post-contrast examination. A retrospective study included re-analysis of 567 contrast-enhanced pituitary MRIs of children treated in a tertiary reference center. Two sets of sequences were created from each MRI examination: Set 1, including common sequences without contrast administration, and Set 2, which included common pre- and post-contrast sequences (conventional MRI examination). The differences in the visibility of pituitary lesions between pairs of sets were statistically analyzed. The overall frequency of Rathke’s cleft cysts was 11.6%, ectopic posterior pituitary 3.5%, and microadenomas 0.9%. Lesions visible without contrast administration accounted for 85% of cases. Lesions not visible before and diagnosed only after contrast injection accounted for only 0.18% of all patients. Statistical analysis showed the advantage of the antero-posterior (AP) pituitary dimension over the other criteria in determining the appropriateness of using contrast in pituitary MRIs. The AP dimension was the most significant factor in logistic regression analysis: OR = 2.23, 95% CI, 1.35–3.71, p-value = 0.002, and in ROC analysis: AUC: 72.9% with a cut-off value of 7.5 mm, with sensitivity/specificity rates of 69.2%/73.5%. In most cases, the use of gadolinium-based contrast agent (GBCA) in pituitary MRI in children with GPD is unnecessary. The advantages of GBCA omission include shortening the time of MRI examination and of general anesthesia; saving time for other examinations, thus increasing the availability of MRI for waiting children; and acceleration in their further clinical management. Full article

Background: Rathke’s Cleft Cysts (RCCs) are rare epithelial cysts arising from remnants of the Rathke pouch in the pituitary gland. A subset of these lesions enlarge and produce a mass effect with consequent hypopituitarism, and may result in visual loss. Moreover, some RCCs with a high intra-cystic protein content may mimic cystic pituitary adenoma, which makes their differential diagnosis ambiguous. Currently, medical professionals have no definitive way to distinguish RCCs from pituitary adenomas. Therefore, preoperative confirmation of RCCs would be of help to medical professionals for the management and proper surgical decision making. The goal of this study is to identify molecular markers in RCCs. Methods: We characterized aqueous and chloroform extracts of surgically resected RCCs and pituitary adenomas using ex vivo ¹H NMR spectroscopy. Results: All RCCs exclusively showed the presence of mucopolysaccharides which are glycosaminoglycans (GAGs) made up of disaccharides of aminosugars and uronic sugars. Conclusion: GAGs can be used as metabolite marker for the detection of RCCs and this knowledge will lay the groundwork for the development of a non-invasive, in vivo magnetic resonance spectroscopy methodology for the differential diagnosis of RCCs and pituitary adenomas using clinical MRI scanners. Full article

Thyroid disease mainly has a thyroid origin but can occasionally have a pituitary origin. Clinicians face several challenges when these conditions occur together. We aimed to determine the prevalence of thyroid disorders in patients undergoing trans-sphenoidal adenomectomy (TSA) for pituitary disease. We reviewed the medical records of patients undergoing TSA for pituitary disease between 2008 and 2017 at Severance Hospital. Thyroid disorders were categorized using blood test results and medical histories at the time of preoperative evaluation. Among 2202 patients, 44 (2%), 218 (9.9%), and 74 (3.4%) had hyperthyroidism, hypothyroidism, and post-thyroidectomy status before TSA, respectively. Among the 44 patients with hyperthyroidism, 30 (68.2%) had central hyperthyroidism. Among the 218 patients with hypothyroidism, 165 (75.7%) had central hypothyroidism. Central hypothyroidism was more common in patients with adrenocorticotropic hormone-secreting pituitary adenomas (aOR (adjusted odds ratio) 1.85), Rathke’s cleft cysts (aOR 2.34), and craniopharyngiomas (aOR 2.58) (all p < 0.05) than in those with nonfunctioning pituitary adenomas. Contrastingly, thyroid cancer had an increased prevalence in patients with growth hormone- (aOR 3.17), prolactin- (aOR 3.66), and thyroid-stimulating hormone-secreting (aOR 6.28) pituitary adenomas (all p < 0.05). Pituitary disease sometimes accompanies thyroid disorders; their characteristics vary according to the type of pituitary disease. Full article