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The Lancet. Diabetes & Endocrinology Aug 2023Cyclic Cushing's syndrome is a subentity of Cushing's syndrome in which phases of biochemical hypercortisolism (peaks) are followed by spontaneous periods of... (Review)
Review
Cyclic Cushing's syndrome is a subentity of Cushing's syndrome in which phases of biochemical hypercortisolism (peaks) are followed by spontaneous periods of physiological or even hypocortisolaemic cortisol secretion (troughs). To identify common features of cyclic Cushing's syndrome, we systematically reviewed single case reports and case series in MEDLINE from database inception to Oct 10, 2022, and identified 707 articles, of which 149 articles were assessed for eligibility and 118 articles (covering 212 cases) were included in the analysis. Pituitary tumours accounted for 67% of cases of cyclic Cushing's syndrome (n=143), ectopic tumours for 17% (n=36), and adrenal tumours for 11% (n=23). Occult tumours accounted for 2% of cases (n=4), and 3% of cases were unclassified (n=6). We compared the clinical symptoms and comorbidities of patients with cyclic Cushing's syndrome with those of patients with non-cyclic Cushing's syndrome and observed no major difference. In adrenocorticotropic hormone (ACTH)-dependent cyclic Cushing's syndrome, bilateral inferior petrosal sinus sampling had a positive (ie, true pituitary) and negative (ie, true ectopic) predictive value of 100% when performed during periods of hypercortisolism, versus a positive predictive value of 73% and a negative predictive value of 86% when performed, irrespective of cortisolaemic status. Overall, 6% of patients (n=12) with cyclic Cushing's syndrome had unnecessary surgery due to misclassification. Remission rates were significantly lower and the time to remission significantly longer in patients with cyclic Cushing's syndrome compared with patients with non-cyclic Cushing's syndrome (p<0·001). Variations in biochemical test results due to unpredictable cycle duration and frequency might cause diagnostic challenges resulting in misdiagnoses and missed diagnoses.
Topics: Humans; Cushing Syndrome; Adrenocorticotropic Hormone; Petrosal Sinus Sampling; Pituitary Neoplasms; Predictive Value of Tests; Diagnosis, Differential
PubMed: 37429301
DOI: 10.1016/S2213-8587(23)00150-X -
Neurosurgery Jun 2023Craniopharyngiomas account for 1.2% to 4.6% of all intracranial tumors. Although age at presentation is distributed bimodally, with a pediatric peak occurring between 5... (Meta-Analysis)
Meta-Analysis
BACKGROUND
Craniopharyngiomas account for 1.2% to 4.6% of all intracranial tumors. Although age at presentation is distributed bimodally, with a pediatric peak occurring between 5 and 15 years and an adult peak between 50 and 70 years, presentation, treatment, and outcome differences between these two craniopharyngioma populations have not been thoroughly characterized.
OBJECTIVE
To compare treatments and outcomes between adult and pediatric craniopharyngiomas.
METHODS
This is a systematic review and meta-analysis. Web of Science, MEDLINE, and Scopus databases were searched for primary studies reporting postoperative complications, functional outcomes, recurrence, and overall survival in patients with craniopharyngioma undergoing surgery.
RESULTS
The search yielded 1,202 unique articles, of which 106 (n=4,202 patients) met criteria for qualitative synthesis and 23 (n=735 patients) met criteria for meta-analysis. Compared with adult, pediatric craniopharyngiomas were less likely to present with visual defects (odds ratio [OR] 0.54, 95% CI 0.36-0.80) or cognitive impairment (OR 0.29, 95% CI 0.12-0.71) and more likely with headaches (OR 2.08, 95% CI 1.16-3.73). Children presented with significantly larger tumors compared with adults (standardized mean difference 0.68, 95% CI 0.38-0.97). Comparing functional outcomes, pediatric patients sustained higher rates of permanent diabetes insipidus (OR 1.70, 95% CI 1.13-2.56), obesity (OR 3.15, 95% CI 1.19-8.31), and cranial nerve and/or neurological defects (OR 4.87, 95% CI 1.78-13.31) than adults. No significant differences were found in rates of postoperative cerebrospinal fluid leak, overall or progression-free survival, or recurrence.
CONCLUSION
Adult and pediatric craniopharyngiomas seem to have fundamental differences in clinical presentation and functional outcomes. These patients frequently require multimodality treatment and are best managed with a multidisciplinary team and an individualized approach.
Topics: Adolescent; Adult; Child; Child, Preschool; Humans; Combined Modality Therapy; Craniopharyngioma; Diabetes Insipidus; Neurosurgical Procedures; Pituitary Neoplasms; Postoperative Complications; Treatment Outcome
PubMed: 36639856
DOI: 10.1227/neu.0000000000002346 -
Pituitary Oct 2023Giant prolactinomas are a rare entity, representing approximately 5% of all prolactinomas. A systematic review of 196 adult cases was performed. A comparison of the... (Review)
Review
PURPOSE
Giant prolactinomas are a rare entity, representing approximately 5% of all prolactinomas. A systematic review of 196 adult cases was performed. A comparison of the clinical, biochemical and radiological characteristics, management and therapeutic outcomes in men versus women is made.
METHODS
A structured search was conducted using the term 'giant prolactinoma'. Following inclusion criteria were used: diameter ≥ 40 mm, prolactin levels > 1000 ng/ml and no concomitant GH/ ACTH secretion.
RESULTS
196 cases were included [age: 38 (28-50) years, F/M ratio: 1/3.6]. Median tumor diameter was 53 (43-69) mm. Pituitary deficiency was present in 91% of cases, with hypogonadotropic hypogonadism being the most frequent. Most common presenting symptoms were visual impairment (73%) and headache (50%) in men and amenorrhea (58%) in women. 82% of cases were treated with a dopamine agonist (DA) as first-line treatment which led to normoprolactinemia, tumor shrinkage and visual improvement in 51%, 88% and 85% of cases, respectively. Surgery was performed in 29% of cases and all showed tumor remnant and persistent hyperprolactinemia. Women had a lower prolactin level and a smaller tumor diameter at diagnosis but pituitary deficiencies were more frequent and outcome was worse.
CONCLUSION
Giant prolactinomas are rare and have a male predominance. Visual impairment is the most frequent presenting symptom in men and amenorrhea in women. The gender-related difference in tumor size and level of prolactin was confirmed in this analysis where men had a larger diameter and a higher baseline prolactin level. DAs are the treatment of choice, irrespective of tumor size and presence of visual impairment. As only half of the cases achieved normoprolactinemia we do not, in contrast to previous literature, state giant prolactinomas to be exquisitely sensitive to DAs. Patient characteristics associated with persistent hyperprolactinemia after treatment with a DA were female gender, higher baseline prolactin and larger tumor size . This analysis did show TSH- and ACTH-deficiency to be more frequent after surgery which was not seen for LH/FSH deficiency.
Topics: Female; Adult; Male; Humans; Prolactinoma; Pituitary Neoplasms; Hyperprolactinemia; Prolactin; Amenorrhea; Dopamine Agonists; Hypopituitarism; Vision Disorders; Adrenocorticotropic Hormone
PubMed: 37544978
DOI: 10.1007/s11102-023-01337-0 -
World Neurosurgery May 2023Supratentorial hemangioblastomas are benign and highly vascularized neoplasms that appear most often in the spine and posterior cranial fossa. They can also be located... (Review)
Review
BACKGROUND
Supratentorial hemangioblastomas are benign and highly vascularized neoplasms that appear most often in the spine and posterior cranial fossa. They can also be located in the supratentorial compartment of the brain. We conducted a systematic review of the literature to better understand the clinical insights of supratentorial hemangioblastoma in adults.
METHODS
Using Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines, the authors reviewed the English-language literature in the PubMed/MEDLINE database on supratentorial hemangioblastoma in adults, published in the past 40 years. We analyzed the differences between sporadic hemangioblastomas and hemangioblastomas associated with von Hippel-Lindau disease. In addition, we compared the characteristics of supratentorial hemangioblastomas with infratentorial and spinal cord locations.
RESULTS
We reviewed 92 articles, describing 157 supratentorial hemangioblastomas in a total cohort of 148 adult patients. Most articles reporting supratentorial hemangioblastomas were published in the United States. Supratentorial hemangioblastomas occur more frequently in women than men. The median age at the time of diagnosis was 44.48 years. The sellar/parasellar region was the most commonly tumor location, while the most common morphological type was the solid type. Almost 80% of hemangioblastomas were completely resected; the outcome was favorable in more than 3 fourth of patients.
CONCLUSIONS
Given their rarity, the literature on supratentorial hemangioblastomas is limited and based mostly on case reports. Complete surgical resection is the first choice of treatment and is associated with a good treatment outcome.
Topics: Male; Adult; Humans; Female; Hemangioblastoma; Spinal Cord; von Hippel-Lindau Disease; Treatment Outcome; Brain; Spinal Cord Neoplasms; Magnetic Resonance Imaging
PubMed: 36822402
DOI: 10.1016/j.wneu.2023.02.071 -
International Journal of Molecular... Oct 2023Recently, advances in molecular biology and bioinformatics have allowed a more thorough understanding of tumorigenesis in aggressive PitNETs (pituitary neuroendocrine... (Review)
Review
Recently, advances in molecular biology and bioinformatics have allowed a more thorough understanding of tumorigenesis in aggressive PitNETs (pituitary neuroendocrine tumors) through the identification of specific essential genes, crucial molecular pathways, regulators, and effects of the tumoral microenvironment. Target therapies have been developed to cure oncology patients refractory to traditional treatments, introducing the concept of precision medicine. Preliminary data on PitNETs are derived from preclinical studies conducted on cell cultures, animal models, and a few case reports or small case series. This study comprehensively reviews the principal pathways involved in aggressive PitNETs, describing the potential target therapies. A search was conducted on Pubmed, Scopus, and Web of Science for English papers published between 1 January 2004, and 15 June 2023. 254 were selected, and the topics related to aggressive PitNETs were recorded and discussed in detail: epigenetic aspects, membrane proteins and receptors, metalloprotease, molecular pathways, PPRK, and the immune microenvironment. A comprehensive comprehension of the molecular mechanisms linked to PitNETs' aggressiveness and invasiveness is crucial. Despite promising preliminary findings, additional research and clinical trials are necessary to confirm the indications and effectiveness of target therapies for PitNETs.
Topics: Animals; Humans; Pituitary Neoplasms; Pituitary Gland; Neuroendocrine Tumors; Aggression; Tumor Microenvironment
PubMed: 37958702
DOI: 10.3390/ijms242115719 -
Expert Review of Endocrinology &... Nov 2022Hyperprolactinemia has been proven to induce hypogonadism and metabolic derangements in both genders, while the consequences of prolactin (PRL) deficiency have been... (Meta-Analysis)
Meta-Analysis
INTRODUCTION
Hyperprolactinemia has been proven to induce hypogonadism and metabolic derangements in both genders, while the consequences of prolactin (PRL) deficiency have been poorly investigated.
AREAS COVERED
To systematically review and analyze data from clinical studies focusing on the metabolic consequences of abnormally high prolactin levels (HPRL) and low prolactin levels (LPRL). In addition, data from preclinical studies about underlying pathophysiological mechanisms were summarized and discussed.
EXPERT OPINION
PRL contributes to providing the correct amount of energy to support the mother and the fetus/offspring during pregnancy and lactation, but it also has a homeostatic role. Pathological PRL elevation beyond these physiological conditions, but also its reduction, impairs metabolism and body composition in both genders, increasing the risk of diabetes and cardiovascular events. Hence, hypoprolactinemia should be avoided as much as possible during treatment with dopamine agonists for prolactinomas. Patients with hypoprolactinemia, because of endogenous or iatrogenic conditions, deserve, as those with hyperprolactinemia, careful metabolic assessment.
Topics: Male; Pregnancy; Humans; Female; Prolactin; Hyperprolactinemia; Prolactinoma; Pituitary Neoplasms
PubMed: 36447418
DOI: 10.1080/17446651.2022.2144829 -
Frontiers in Endocrinology 2022Pasireotide (PAS) is a novel somatostatin receptor ligands (SRL), used in controlling hormonal hypersecretion in both acromegaly and Cushing's Disease (CD). In previous... (Meta-Analysis)
Meta-Analysis
INTRODUCTION
Pasireotide (PAS) is a novel somatostatin receptor ligands (SRL), used in controlling hormonal hypersecretion in both acromegaly and Cushing's Disease (CD). In previous studies and meta-analysis, first-generation SRLs were reported to be able to induce significant tumor shrinkage only in somatotroph adenomas. This systematic review and meta-analysis aim to summarize the effect of PAS on the shrinkage of the pituitary adenomas in patients with acromegaly or CD.
MATERIALS AND METHODS
We searched the Medline database for original studies in patients with acromegaly or CD receiving PAS as monotherapy, that assessed the proportion of significant tumor shrinkage in their series. After data extraction and analysis, a random-effect model was used to estimate pooled effects. Quality assessment was performed with a modified Joanna Briggs's Institute tool and the risk of publication bias was addressed through Egger's regression and the three-parameter selection model.
RESULTS
The electronic search identified 179 and 122 articles respectively for acromegaly and CD. After study selection, six studies considering patients with acromegaly and three with CD fulfilled the eligibility criteria. Overall, 37.7% (95%CI: [18.7%; 61.5%]) of acromegalic patients and 41.2% (95%CI: [22.9%; 62.3%]) of CD patients achieved significant tumor shrinkage. We identified high heterogeneity, especially in acromegaly (I of 90% for acromegaly and 47% for CD), according to the low number of studies included.
DISCUSSION
PAS treatment is effective in reducing tumor size, especially in acromegalic patients. This result strengthens the role of PAS treatment in pituitary adenomas, particularly in those with an invasive behavior, with progressive growth and/or extrasellar extension, with a low likelihood of surgical gross-total removal, or with large postoperative residual tissue.
SYSTEMATIC REVIEW REGISTRATION
https://www.crd.york.ac.uk/prospero/display_record.php?ID=CRD42022328152, identifier CRD42022328152.
Topics: ACTH-Secreting Pituitary Adenoma; Acromegaly; Adenoma; Humans; Pituitary ACTH Hypersecretion; Pituitary Neoplasms; Somatostatin
PubMed: 35846311
DOI: 10.3389/fendo.2022.935759 -
Child's Nervous System : ChNS :... Feb 2022Brachytherapy has been indicated as an alternative option for treating cystic craniopharyngiomas. Despite the difficulties regarding the complex nuclear infrastructure... (Meta-Analysis)
Meta-Analysis Review
PURPOSES
Brachytherapy has been indicated as an alternative option for treating cystic craniopharyngiomas. Despite the difficulties regarding the complex nuclear infrastructure and consequent small number of brachytherapy studies, recent discoveries relating tumour characteristics to sensitivity to brachytherapy have stimulated this extensive systematic review and meta-analysis of the recent results aiming to summarise its efficacy and complications.
METHODS
The systematic review was structured using PRISMA statements. The MEDLINE database was systematically reviewed from March 2010 to February 2021 to identify qualified trials dealing with radioisotope brachytherapy in cystic craniopharyngiomas in the paediatric population, emphasising tumour control rates and complications.
RESULTS
A total of 228 individuals were analysed, of which 66 were children. The minimum average follow-up was 5 years. Considering the paediatric trials, partial and complete responses were achieved in 89% of patients with exclusively cystic lesions, compared to 58% in non-exclusively cystic lesions. The former observed progression in 3% of patients, while in the latter, 35% was reported. Visual and endocrine improvement reached 64% and 20%, respectively, in the first group, in contrast to 48% and 7% in the second group, respectively. There were similar results considering the non-exclusive paediatric series, but with less expressive numbers.
CONCLUSION
These results reinforce the positive impact of radioisotope brachytherapy in the treatment of predominant monocystic or multicystic craniopharyngiomas, especially in the paediatric population, grounded by impressive tumour control rates, lower morbidities and single application in the majority of the cases. In the future, specific morphological tumour characteristics might be considered for a more assertive patient selection.
Topics: Brachytherapy; Child; Craniopharyngioma; Humans; Pituitary Neoplasms
PubMed: 34618201
DOI: 10.1007/s00381-021-05378-3 -
Neurosurgical Review Aug 2023Although typically benign, trigeminal schwannomas (TS) may require surgical resection when large or symptomatic and can cause significant morbidity. This study aims to... (Meta-Analysis)
Meta-Analysis Review
Although typically benign, trigeminal schwannomas (TS) may require surgical resection when large or symptomatic and can cause significant morbidity. This study aims to summarize the literature and synthesize outcomes following surgical resection of TS. A systematic review was performed according to PRISMA guidelines. Data extracted included patient and tumor characteristics, surgical approaches, and postoperative outcomes. Odds ratios (OR) with corresponding 95% confidence intervals (CI) were used for outcome analysis. The initial search yielded 1838 results, of which 26 studies with 974 patients undergoing surgical resection of TS were included. The mean age was 42.9 years and 58.0% were female. The mean tumor diameter was 4.7 cm, with Samii type A, B, C, and D tumors corresponding to 33.4%, 15.8%, 37.2%, and 13.6%, respectively. Over a mean symptom duration of 29 months, patients presented with trigeminal hypesthesia (58.7%), headache (32.8%), trigeminal motor weakness (22.8%), facial pain (21.3%), ataxia (19.4%), diplopia (18.7%), and visual impairment (12.0%). Surgical approaches included supratentorial (61.4%), infratentorial (15.0%), endoscopic (8.6%), combined/staged (5.3%), and anterior (5.7%) or posterior (4.0%) petrosectomy. Postoperative improvement of facial pain (83.9%) was significantly greater than trigeminal motor weakness (33.0%) or hypesthesia (29.4%). The extent of resection (EOR) was reported as gross total (GTR), near total, and subtotal in 77.7%, 7.7%, and 14.6% of cases, respectively. Over a mean follow-up time of 62.6 months, recurrence/progression was noted in 7.4% of patients at a mean time to recurrence of 44.9 months. Patients with GTR had statistically significantly lower odds of recurrence/progression (OR: 0.07; 95% CI: 0.04-0.15) compared to patients with non-GTR. This systematic review and meta-analysis report patient outcomes following surgical resection of TS. EOR was found to be an important predictor of the risk of recurrence. Facial pain was more likely to improve postoperatively than facial hypesthesia. This work reports baseline rates of post-operative complications across studies, establishing benchmarks for neurosurgeons innovating and working to improve surgical outcomes for TS patients.
Topics: Humans; Female; Adult; Male; Hypesthesia; Neurilemmoma; Cranial Nerve Neoplasms; Postoperative Complications; Facial Pain
PubMed: 37646878
DOI: 10.1007/s10143-023-02121-1 -
World Neurosurgery Aug 2022Craniopharyngiomas are morbid tumors that significantly reduce patients' quality of life (QoL). The lifelong burden of endocrine, visual, hypothalamic, and limbic... (Review)
Review
BACKGROUND
Craniopharyngiomas are morbid tumors that significantly reduce patients' quality of life (QoL). The lifelong burden of endocrine, visual, hypothalamic, and limbic dysfunction can have disastrous consequences for the physical and psychosocial health of patients. Elucidating the factors that influence QoL could guide therapeutic interventions to improve patient well-being.
METHODS
A systematic review was performed in accordance with the PRISMA (preferred reporting items for systematic reviews and meta-analyses) statement using the PubMed and Medline databases. Studies that had reported patient QoL using validated metrics in both adult and pediatric populations were included. Bias and methodological rigor were assessed using the MINORS (methodological index for nonrandomized studies) criteria.
RESULTS
A total of 25 studies, including 2025 patients, were available for review. Most studies were small, retrospective, cohort studies with a high risk of bias. The QoL of the patients with craniopharyngioma was lower than that of the general population. Hypothalamic involvement was consistently the strongest predictor of QoL. Endocrinopathy contributed to morbidity but could be ameliorated by hormone replacement therapy. Social and emotional dysregulation and a poor memory are common complaints after surgery, and iatrogenic damage to the infundibulum, hypothalamus, limbic system, and frontal lobes might underlie these concerns. Sleep-wake cycle dysfunction and hypothalamic obesity are serious consequences of hypothalamic damage.
CONCLUSIONS
An experienced multidisciplinary team is necessary to optimally manage the complex cases of these patients. The poor QoL of patients with craniopharyngioma is multifactorial. However, the contribution of iatrogenesis is not insubstantial. Improved surgical techniques, focusing on hypothalamic preservation, and adjuvant treatment options are required to improve the well-being of these patients.
Topics: Adult; Child; Craniopharyngioma; Humans; Hypothalamus; Pituitary Neoplasms; Quality of Life; Retrospective Studies
PubMed: 35580780
DOI: 10.1016/j.wneu.2022.05.038