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Surgical Neurology International 2024Non-functioning pituitary adenomas (NFPAs) are well-differentiated benign tumors originating from the adenohypophyseal cells of the pituitary gland. They present with... (Review)
Review
Comparing redo surgery and stereotactic radiosurgery for recurrent, residual, and/or tumors showing progression in nonfunctioning pituitary adenomas: A systematic review and meta-analysis.
BACKGROUND
Non-functioning pituitary adenomas (NFPAs) are well-differentiated benign tumors originating from the adenohypophyseal cells of the pituitary gland. They present with headaches, visual disorders, or cranial nerve deficits. NFPAs can recur, progress, or present as residual tumors. We, therefore, conducted this review to compare the effects of both revision surgery and stereotactic surgery on tumor size, visual status, endocrine status, and complications.
METHODS
A systematic review of published literature on recurrent, residual, or progressing NFPAs that underwent redo surgery or stereotactic radiosurgery from the inception till June 2020 was conducted as per Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines. Thirteen records (1209 patients) were included, and risk ratio (RR) and 95% confidence intervals (CIs) estimated from each study were pooled using a random-effects meta-analysis model.
RESULTS
Redo surgery was the preferred intervention in patients presenting with larger tumor sizes and was more effective in reducing the tumor size as compared to stereotactic radiosurgery (SRS) (risk ratio [RR] 56.14; 95% CI, 16.45-191.58). There was more visual loss with revision surgery as compared to SRS (risk ratio [RR] 0.08; 95% CI, 0.03-0.20). However, SRS was associated with fewer complications, such as new diabetes insipidus, as compared to the redo surgery (risk ratio [RR] 0.01; 95% CI 0.01-0.03).
CONCLUSION
Redo surgery is the superior choice in the treatment of recurrent/residual or progressing NFPAs if the tumor size is large and an immediate reduction in tumor burden through debulking is warranted. However, redo surgery is associated with a higher risk of visual loss, new endocrinopathies, and other complications, in contrast to SRS.
PubMed: 38468659
DOI: 10.25259/SNI_907_2023 -
European Archives of... Apr 2024Clinical presentations encompass respiratory, feeding issues, nasopharyngeal mass, meningitis, CSF leakage, craniofacial anomalies, and endocrine problems. Surgery is... (Meta-Analysis)
Meta-Analysis Review
PURPOSE
Clinical presentations encompass respiratory, feeding issues, nasopharyngeal mass, meningitis, CSF leakage, craniofacial anomalies, and endocrine problems. Surgery is the primary treatment, transitioning from frontal craniotomy to endoscopic methods, offering improved outcomes. Yet, more studies are needed. A comprehensive review on trans-sellar trans-sphenoidal encephalocele (TSTSE) is missing. Our study aims to fill this gap, offering a comprehensive perspective for physicians.
METHODS
This review adhered to the PRISMA guideline. Eligible studies focused on human subjects, specifically trans-sellar encephaloceles, and provided comprehensive treatment details. English language articles published up to April 11th, 2023, were considered. Two trained researchers conducted article screening using consistent criteria. Data extraction covered various aspects, including clinical presentation, surgical methods, and outcomes, with results presented descriptively in two tables. Due to the rarity of this congenital anomaly, meta-analysis and publication bias assessment were not feasible. Data extraction was independently conducted by two reviewers, with subsequent cross-verification.
RESULTS
A total of 36 patients were identified from 14 studies, the most frequently observed clinical presentation was dyspnea (41.67%) and the most frequently observed accompanying anomaly was cleft lip/palate (55.56%). CT and MRI were adopted in nearly all the cases, and trans-nasal approach was the most often used surgical approach (57.14%) with the 'soft material combination' the most commonly used method for cranial base repairment (35.71%). A total of two deaths occurred and diabetes insipidus was the most common perioperative complication which occurred in six surgery patients (21.43%).
CONCLUSION
TSTSE predominantly affects males and presents with dyspnea, visual deficits, pituitary insufficiency, and cranial base-related symptoms. Early diagnosis is critical, with advanced imaging playing a key role. Endocrine assessment is vital for hormone management. Surgery offers symptom relief but entails risks, including reported fatalities and complications. The choice between surgery and conservative management requires careful deliberation. The trans-nasal approach is favored for its reduced trauma, yet further research is necessary to validate this preference.
Topics: Male; Humans; Encephalocele; Cleft Lip; Cleft Palate; Prognosis; Dyspnea
PubMed: 38189965
DOI: 10.1007/s00405-023-08355-5 -
Acta Endocrinologica (Bucharest,... 2023Pituitary adenomas are benign tumors, usually found in men in their 3 and 5 decades of life, representing 10-15% of all intracranial tumors. The clinical manifestations...
CONTEXT
Pituitary adenomas are benign tumors, usually found in men in their 3 and 5 decades of life, representing 10-15% of all intracranial tumors. The clinical manifestations include important endocrinological disturbances and visual impairment.
OBJECTIVE
This study aimed to determine the most suitable neurosurgical approach regarding the dimensions, extensions and invasiveness of tumor extensions.
DESIGN
This was a systematic review of the literature from 2002-2022, focused on clinical outcome, especially endocrinological state according to the surgical approach.
SUBJECTS AND METHODS
We performed an advanced search on Web of Science and PubMed databases on October 10, 2022. The literature showed 300 studies in the last 20 years, and after we applied the inclusion and exclusion criteria's, 19 studies were fully read and analyzed.
RESULTS
Postoperative complications were reviewed in each surgical approach group, including visual impairment, new endocrinological disturbances, diabetes insipidus and cerebrospinal fluid leakage. Analyze of the endocrinological findings did not determined differences in transcranial groups from transsphenoidal groups. Overall complications were identified in the transcranial cohorts, while cerebrospinal fluid leakage still represent the main problem in transsphenoidal groups. The majority of studies found included extended endoscopic transsphenoidal approach, which shows results of great potential.
CONCLUSIONS
For the surgical treatment of pituitary adenoma, transsphenoidal procedure with or without extended approaches is preferred, but they're cases when a craniotomy is mandatory for a feasible gross tumor resection. Combined "above and below" simultaneous procedure or a two-staged intervention is recommended for giant pituitary adenoma, to maximize tumor resection and lower the risk of cerebrospinal fluid leakage.
PubMed: 37908878
DOI: 10.4183/aeb.2023.228 -
Acta Neurochirurgica Sep 2023Although there is an increasing body of evidence showing gender differences in various medical domains as well as presentation and biology of pituitary adenoma (PA),... (Meta-Analysis)
Meta-Analysis Review
BACKGROUND
Although there is an increasing body of evidence showing gender differences in various medical domains as well as presentation and biology of pituitary adenoma (PA), gender differences regarding outcome of patients who underwent transsphenoidal resection of PA are poorly understood. The aim of this study was to identify gender differences in PA surgery.
METHODS
The PubMed/MEDLINE database was searched up to April 2023 to identify eligible articles. Quality appraisal and extraction were performed in duplicate.
RESULTS
A total of 40 studies including 4989 patients were included in this systematic review and meta-analysis. Our analysis showed odds ratio of postoperative biochemical remission in males vs. females of 0.83 (95% CI 0.59-1.15, P = 0.26), odds ratio of gross total resection in male vs. female patients of 0.68 (95% CI 0.34-1.39, P = 0.30), odds ratio of postoperative diabetes insipidus in male vs. female patients of 0.40 (95% CI 0.26-0.64, P < 0.0001), and a mean difference of preoperative level of prolactin in male vs. female patients of 11.62 (95% CI - 119.04-142.27, P = 0.86).
CONCLUSIONS
There was a significantly higher rate of postoperative DI in female patients after endoscopic or microscopic transsphenoidal PA surgery, and although there was some data in isolated studies suggesting influence of gender on postoperative biochemical remission, rate of GTR, and preoperative prolactin levels, these findings could not be confirmed in this meta-analysis and demonstrated no statistically significant effect. Further research is needed and future studies concerning PA surgery should report their data by gender or sexual hormones and ideally further assess their impact on PA surgery.
Topics: Humans; Male; Female; Treatment Outcome; Prolactin; Retrospective Studies; Pituitary Neoplasms; Adenoma; Hormones; Postoperative Complications
PubMed: 37555999
DOI: 10.1007/s00701-023-05726-z -
Orphanet Journal of Rare Diseases Jun 2023Pituitary abscess (PA) is a rare condition and not well understood. We aimed to describe a case and perform a comprehensive systematic review to explore presenting...
BACKGROUND
Pituitary abscess (PA) is a rare condition and not well understood. We aimed to describe a case and perform a comprehensive systematic review to explore presenting symptoms, radiological findings, endocrine abnormalities and mortality.
AIM
To identify presenting symptoms, radiological findings, endocrinological abnormalities and predictors of mortality for PA.
METHODS
We systematically reviewed the literature to identify all case reports of PA. Data regarding presentation, mortality, radiological findings, endocrinological abnormalities and treatment was extracted.
RESULTS
We identified 488 patients from 218 articles meeting the inclusion criteria. Mortality was 5.1%, with days to presentation (OR 1.0005, 95% CI 1.0001-1.0008, p < 0.01) being the only identified independent predictor of mortality. Mortality rates have decreased over time, with cases published prior to 2000 having higher mortality rates (OR 6.92, 95% CI 2.80-17.90, p < 0.001). The most common symptom was headache (76.2%), followed by visual field defects (47.3%). Classical signs of infection were only present in 43%. The most common imaging feature on magnetic resonance imaging (MRI) was high T2 and low T1 signal of the pituitary gland with peripheral contrast enhancement. Over half (54.8%) were culture negative, with the most common bacterial organism being staphylococcus aureus (7.8%) and fungal organism being aspergillus (8.8%). The most common endocrine abnormality was hypopituitarism (41.1%), followed by diabetes insipidus (24.8%). Whilst symptoms resolved in most patients, persistent endocrine abnormalities were present in over half of patients (61.0%).
CONCLUSION
PA is associated with significant mortality, with delayed presentation increasing risk of mortality. Ongoing endocrinological abnormalities are common. Given the non-specific clinical presentation, the appearance of high T2, low T1 and peripheral contrast enhancement of the pituitary on MRI should prompt consideration of this rare disease.
Topics: Humans; Abscess; Pituitary Diseases; Hypopituitarism; Pituitary Neoplasms; Magnetic Resonance Imaging
PubMed: 37365629
DOI: 10.1186/s13023-023-02788-1 -
Nigerian Journal of Clinical Practice May 2023The most effective treatment method for meningiomas is surgical treatment. However, complete resection of cavernous sinus meningiomas is quite difficult. The biggest...
BACKGROUND
The most effective treatment method for meningiomas is surgical treatment. However, complete resection of cavernous sinus meningiomas is quite difficult. The biggest reasons for this are; narrow surgical corridor, the optic chiasm is the close relationship between the cranial nerves and anterior cerebral and internal carotid arteries. Gamma knife radiosurgery (GKRS) may be a good option in these lesions with high mortality and morbidity.
AIM
This study aimed to evaluate and compare the early effects at a mean of 6 months and 3 years and outcomes between surgery followed by gamma knife radiosurgery (GKRS) and GKRS alone for cavernous sinus meningiomas.
METHODS
We included 20 patients with cavernous sinus meningioma treated via single fraction Leksell Gamma Knife Perfexion (Elekta Instruments; Stockholm, Sweden) between 2015 and 2018. The mean age of the patients was 54.95 (range: 32-77) years. Nine patients underwent primary surgery (for the resection of extracavernous components of the tumor) followed by GKRS (for the resection of intracavernous components of the tumor) (group A). Meanwhile, 11 patients were managed with GKRS alone (group B). The tumor volume ranged from 2.8 to 32.8 (mean: 14.76) cm3 and the isodose to the tumor margin from 10 to 13.5 (mean: 11.65) Gy.
RESULTS
In total, 20 patients were followed up at a mean time of 18.95 (range: 6-36) months. The mean Karnofsky Performance Score of the patients was 95.2%. The mean follow up times of groups A and B were 23.6 and 15 months, respectively. Meanwhile, only patients with a follow up time of at least 6 months were included in the study. The mean follow up time after GKRS in group A was 17.6 months. There was no change in the tumor volume in 15% of patients. In the remaining cases, the tumor volume decreased. The mean tumor regression rates were 82.2% in Group A and 17.7% in Group B. The tumor volume did not decrease in three patients (n = 1, group A and n = 2, group B). Cranial nerve deficits improved, worsened, and remained stable in 46.6%, 26.6%, and 26.6% of cases, respectively. The temporary morbidity rate was 10%. In group A, transient postoperative diabetes insipidus was observed in one patient and atelectasis in another. None of these complications affected the final status of patients. The mortality rate after treatment was 0%.
CONCLUSION
Volume staged GKRS is safe and effective for cavernous sinus meningioma. GKRS is effective for long term tumor growth control and has a low complication rate. Hence, it is the preferred management strategy for tumors with a suitable volume (average tumor diameter: 3 cm or volume: 10 cm3 ). In tumors with a volume of more than 10 mL and/or without a 3 mm safety margin with the optical system, it is recommended to prepare for radiosurgery by cytoreducing the tumor volume surgically. Based on our opinion, the best results were obtained by GKRS to the intracavernous.
Topics: Humans; Adult; Middle Aged; Aged; Meningioma; Meningeal Neoplasms; Radiosurgery; Cavernous Sinus; Treatment Outcome; Follow-Up Studies; Retrospective Studies
PubMed: 37357468
DOI: 10.4103/njcp.njcp_2033_21 -
World Journal of Oncology Jun 2023Pituitary metastasis (PM) from differentiated thyroid cancer (DTC) is extremely rare and may adversely affect outcomes. We aimed to assess the characteristics and... (Review)
Review
BACKGROUND
Pituitary metastasis (PM) from differentiated thyroid cancer (DTC) is extremely rare and may adversely affect outcomes. We aimed to assess the characteristics and outcomes of patients with PM from DTC.
METHODS
We systematically reviewed the literature on publications on PM and the different DTC histologic types (papillary, follicular, and Hurthle cell cancers). Three databases (PubMed, Embase, and Scopus) were searched for articles published from 1967 to 2022. Survival time was estimated as the period from the first treatment of PM to the time of death or last follow-up.
RESULTS
Twenty-five articles comprising 27 cases that met the eligibility criteria were identified using the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA). The median age of the patients was 60 years (23 - 86). A preponderance of females (66.7%) with PM most commonly reported papillary thyroid cancer (55.6%). This was followed by follicular thyroid cancer (37.0%) and Hurthle cell cancer (7.4%). The most common presentations were headache, nausea, and vomiting, with visual symptoms in 44.4%. Diabetes insipidus was an infrequent finding (7.4%). The median time from diagnosis or first treatment of DTC to the diagnosis of PM was 3 years (0 - 25). The most common endocrine abnormality was hyperprolactinemia (63.2%), while the most frequently deficient hormone was luteinizing hormone (50%). The most common treatment modality for PM was a combination of radiotherapy and surgery with or without radio-iodine. At the end of the follow-up, 30% of the patients died. Only 33.3% of the patients achieved complete resolution of symptoms. The overall median survival time was 12 months (3 - 108). There was a moderate inverse correlation between the age of patients and survival, which was, however, not statistically significant (rs = -0.45, P = 0.103).
CONCLUSION
PM from DTC is extremely rare, and Hurtle cell cancer appears to be the least associated with PM. Diabetes insipidus is a rare initial manifestation of PM from DTC. Complete resolution of symptoms is less likely to be achieved in PM from DTC. Older age may confer an increased survival tendency, probably due to more intracranial space volume in older people compared to the younger population. Larger studies are needed to examine the relationship between age and survival in PM from DTC. Also, more observational data are required to determine the predictors of survival and compare the efficacy of the different treatment modalities in patients with PM from DTC.
PubMed: 37350806
DOI: 10.14740/wjon1593 -
BMC Medical Genomics Apr 2023Wolfram syndrome type 1 gene (WFS1), which encodes a transmembrane structural protein (wolframin), is essential for several biological processes, including proper inner...
BACKGROUND
Wolfram syndrome type 1 gene (WFS1), which encodes a transmembrane structural protein (wolframin), is essential for several biological processes, including proper inner ear function. Unlike the recessively inherited Wolfram syndrome, WFS1 heterozygous variants cause DFNA6/14/38 and wolfram-like syndrome, characterized by autosomal dominant nonsyndromic hearing loss, optic atrophy, and diabetes mellitus. Here, we identified two WFS1 heterozygous variants in three DFNA6/14/38 families using exome sequencing. We reveal the pathogenicity of the WFS1 variants based on three-dimensional (3D) modeling and structural analysis. Furthermore, we present cochlear implantation (CI) outcomes in WFS1-associated DFNA6/14/38 and suggest a genotype-phenotype correlation based on our results and a systematic review.
METHODS
We performed molecular genetic test and evaluated clinical phenotypes of three WFS1-associated DFNA6/14/38 families. A putative WFS1-NCS1 interaction model was generated, and the impacts of WFS1 variants on stability were predicted by comparing intramolecular interactions. A total of 62 WFS1 variants associated with DFNA6/14/38 were included in a systematic review.
RESULTS
One variant is a known mutational hotspot variant in the endoplasmic reticulum (ER)-luminal domain WFS1(NM_006005.3) (c.2051 C > T:p.Ala684Val), and the other is a novel frameshift variant in transmembrane domain 6 (c.1544_1545insA:p.Phe515LeufsTer28). The two variants were pathogenic, based on the ACMG/AMP guidelines. Three-dimensional modeling and structural analysis show that non-polar, hydrophobic substitution of Ala684 (p.Ala684Val) destabilizes the alpha helix and contributes to the loss of WFS1-NCS1 interaction. Also, the p.Phe515LeufsTer28 variant truncates transmembrane domain 7-9 and the ER-luminal domain, possibly impairing membrane localization and C-terminal signal transduction. The systematic review demonstrates favorable outcomes of CI. Remarkably, p.Ala684Val in WFS1 is associated with early-onset severe-to-profound deafness, revealing a strong candidate variant for CI.
CONCLUSIONS
We expanded the genotypic spectrum of WFS1 heterozygous variants underlying DFNA6/14/38 and revealed the pathogenicity of mutant WFS1, providing a theoretical basis for WFS1-NCS1 interactions. We presented a range of phenotypic traits for WFS1 heterozygous variants and demonstrated favorable functional CI outcomes, proposing p.Ala684Val a strong potential marker for CI candidates.
Topics: Humans; Wolfram Syndrome; Cochlear Implants; Cochlear Implantation; Pedigree; Hearing Loss; Deafness
PubMed: 37041640
DOI: 10.1186/s12920-023-01506-x -
Survey of Ophthalmology 2023Wolfram-like syndrome (WFLS) is a recently described autosomal dominant disorder with phenotypic similarities to autosomal recessive Wolfram syndrome (WS), including... (Review)
Review
Wolfram-like syndrome (WFLS) is a recently described autosomal dominant disorder with phenotypic similarities to autosomal recessive Wolfram syndrome (WS), including optic atrophy, hearing impairment, and diabetes mellitus. We summarize current literature, define the clinical characteristics, and investigate potential genotype phenotype correlations. A systematic literature search was conducted in electronic databases Pubmed/MEDLINE, EMBACE, and Cochrane Library. We included studies reporting patients with a clinical picture consisting at least 2 typical clinical manifestations of WSF1 disorders and heterozygous mutations in WFS1. In total, 86 patients from 35 studies were included. The most common phenotype consisted of the combination of optic atrophy (87%) and hearing impairment (94%). Diabetes mellitus was seen in 44% of the patients. Nineteen percent developed cataract. Patients with missense mutations in WFS1 had a lower number of clinical manifestations, less chance of developing diabetes insipidus, but a younger age at onset of hearing impairment compared to patients with nonsense mutations or deletions causing frameshift. There were no studies reporting decreased life expectancy. This review shows that, within the spectrum of WFS1-associated disorders or "wolframinopathies," autosomal dominantly inherited WFLS has a relatively mild phenotype compared to autosomal recessive WS. The clinical manifestations and their age at onset are associated with the specific underlying mutations in the WFS1 gene.
Topics: Humans; Hearing Loss; Mutation; Optic Atrophy; Tungsten; Wolfram Syndrome
PubMed: 36764396
DOI: 10.1016/j.survophthal.2023.01.012 -
Frontiers in Oncology 2022The transcranial approach (TCA) has historically been used to remove craniopharyngiomas. Although the extended endoscopic endonasal approach (EEA) to these tumors has...
INTRODUCTION
The transcranial approach (TCA) has historically been used to remove craniopharyngiomas. Although the extended endoscopic endonasal approach (EEA) to these tumors has been more commonly accepted in the recent two decades, there is debate over whether this approach leads to better outcomes. The goal of this systematic review and meta-analysis was to more comprehensively understand the benefits and limitations of these two approaches in craniopharyngioma resection based on comparative studies.
METHODS
We conducted a systematic literature search in accordance with the Preferred Reporting Items for Systematic reviews and Meta-Analyses recommendations using MEDLINE, EMBASE, and the Cochrane Library. A total of 448 articles were screened. Data were extracted and analyzed using proportional meta-analysis. Eight comparative studies satisfied the inclusion criteria. The extent of resection, visual outcomes, and postoperative complications such as endocrine dysfunction and cerebrospinal fluid (CSF) leakage were compared.
RESULTS AND DISCUSSION
Eight studies, involving 376 patients, were included. Resection by EEA led to a greater rate of gross total resection (GTR) (odds ratio [OR], 2.42; p = 0.02; seven studies) with an incidence of 61.3% vs. 50.5% and a higher likelihood of visual improvement (OR, 3.22; p < 0.0001; six studies). However, TCA resulted in a higher likelihood of visual deterioration (OR, 3.68; p = 0.002; seven studies), and was related, though not significantly, to panhypopituitarism (OR, 1.39; p = 0.34; eight studies) and diabetes insipidus (OR, 1.14; p = 0.58; seven studies). Although TCA showed significantly lower likelihoods of CSF leakage (OR, 0.26; 95% confidence interval [CI], 0.10-0.71; p = 0.008; eight studies) compared to EEA, there was no significant difference in meningitis (OR, 0.92; 95% CI, 0.20-4.25; p = 0.91; six studies) between the two approaches. When both approaches can completely resect the tumor, EEA outperforms TCA in terms of GTR rate and visual outcomes, with favorable results in complications other than CSF leakage, such as panhypopituitarism and diabetes insipidus. Although knowledge of and competence in traditional microsurgery and endoscopic surgery are essential in surgical decision-making for craniopharyngioma treatment, when both approaches are feasible, EEA is associated with favorable surgical outcomes.
SYSTEMATIC REVIEW REGISTRATION
http://www.crd.york.ac.uk/PROSPERO/, identifier CRD42021234801.
PubMed: 36530998
DOI: 10.3389/fonc.2022.1058329