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Langenbeck's Archives of Surgery Mar 2022Composite phaeochromocytoma is a tumour containing a separate tumour of neuronal origin in addition to a chromaffin cell tumour. This study reports on two cases from a... (Review)
Review
INTRODUCTION
Composite phaeochromocytoma is a tumour containing a separate tumour of neuronal origin in addition to a chromaffin cell tumour. This study reports on two cases from a single centre's records and presents a systematic literature review of composite phaeochromocytomas.
METHODS
In addition to describing 2 case reports, a systematic search of the Medline database from inception up to April 2020 was done for human case reports on composite phaeochromocytomas. Relevant titles and/or abstracts were screened, and full texts were reviewed to identify appropriate studies. Data was extracted and a descriptive analysis of presentation, clinical features, management strategies and outcomes was performed. The quality of included studies was assessed using a critical appraisal checklist.
RESULTS
There were 62 studies included, with a total of 94 patients. Of 91 patients where data was available, the median (range) age of patients was 48 (4-86) years. Of 90 patients where information was provided, 57% were female. In at least 28% of patients, a genetic cause was identified. Common presenting features include abdominal pain, palpable mass, cardiovascular and gastrointestinal symptoms. The most common tumour component with phaeochromocytoma is ganglioneuroma; other components include ganglioneuroblastoma, neuroblastoma and malignant peripheral nerve sheath tumours. In patients with follow-up data (n=48), 85% of patients were alive and well at a median (range) follow-up time of 18 (0.5-168) months.
CONCLUSION
Composite phaeochromocytoma is a rare tumour, with a significant genetic predisposition. This review summarises available epidemiological data, which will be useful for clinicians managing this rare condition.
Topics: Adrenal Gland Neoplasms; Aged; Aged, 80 and over; Brain Neoplasms; Female; Humans; Middle Aged; Pheochromocytoma
PubMed: 33651160
DOI: 10.1007/s00423-021-02129-5 -
International Journal of Surgery... Apr 2015Partial adrenalectomy is typically performed for the treatment of hereditary and sporadic bilateral tumours, to reduce the risk of adrenal failure, particularly in... (Meta-Analysis)
Meta-Analysis Review
BACKGROUND
Partial adrenalectomy is typically performed for the treatment of hereditary and sporadic bilateral tumours, to reduce the risk of adrenal failure, particularly in younger patients. Partial adrenalectomy proposes a postoperative steroid-free course nevertheless, is associated with the risk of local recurrence. In this study we evaluate the recurrence and functional outcomes of partial adrenalectomy.
METHODS
A systematic search was conducted using MEDLINE, PubMed, EMBASE, Current Contents Connect, Cochrane library, Google Scholar, Science Direct, and Web of Science. The search identified 60 relevant articles reporting on patients who underwent partial adrenalectomy. Data was extracted from each study and used to calculate a pooled event rate and 95% confidence interval (95% CI).
RESULTS
The overall recurrence rate was 8% (95% CI: 0.05-0.12) and the 85% (95% CI: 0.78-0.9) of the patients were steroid free. The recurrence rates were the least in the retroperitoneoscopic group 1% (95% CI: 0-0.04) and Conn's syndrome group 2% (95% CI: 0.01-0.05) and highest in open group 15% (95% CI: 0.07-0.28) and Pheochromocytoma group 10% (95% CI: 0.07-0.16). Steroid independence rates were best in the Conn's syndrome group 97% (95% CI: 0.85-0.99) and laparoscopic group 88% (95% CI: 0.75-0.95).
CONCLUSIONS
Partial adrenalectomy can obviate the need for steroid replacement in the majority of patients and local recurrence rates appear to be infrequent. For patients with hereditary and bilateral adrenal tumours, partial adrenalectomy should be recommended as a primary surgical approach whenever possible.
Topics: Adrenal Gland Neoplasms; Adrenalectomy; Global Health; Humans; Incidence; Laparoscopy; Neoplasm Recurrence, Local; Pheochromocytoma; Postoperative Period
PubMed: 25681039
DOI: 10.1016/j.ijsu.2015.01.015 -
International Journal of Surgery... Sep 2022To compare the safety and effectiveness of robot-assisted adrenalectomy (RA) and laparoscopic adrenalectomy (LA). (Meta-Analysis)
Meta-Analysis Review
OBJECTIVE
To compare the safety and effectiveness of robot-assisted adrenalectomy (RA) and laparoscopic adrenalectomy (LA).
METHODS
We performed a systematic review and cumulative meta-analysis of the primary outcomes of interest according to the PRISMA (Preferred Reporting Items for Systematic Reviews and Meta-Analyses) and AMSTAR (Assessing the Methodological Quality of Systematic Reviews) Guidelines. Five databases, including Medline, PubMed, Cochrane Library, Scopus, and Web of Science, were systematically searched. The search timeframe was set from the creation of the database to December 2021.
RESULTS
There were 26 studies including 2985 patients. Our study found that the robotic technique was superior to conventional laparoscopy for estimated blood loss (WMD = -18.25, 95% CI [-27.85, -8.65], P < 0.01), length of stay (WMD = -0.45, 95% CI [-0.57, -0.33], P < 0.01), and conversion to open (OR = 0.31, 95% CI [0.12, 0.78], P = 0.01), while complications and readmissions were comparable. Interestingly, there was no difference in operative time between the two surgical modalities, but subgroup analysis found that the retroperitoneal route robotic technique took longer (WMD = 14.64, 95% CI [0.04, 29.24], P < 0.05), whereas the study of the mixed surgical modality (RA versus LA with mixed transabdominal and retroperitoneal surgical routes) found that the robot required less time (WMD = -12.29, 95% CI [-22.86, -1.72], P < 0.05). For pheochromocytoma, RA was superior to LA in terms of length of stay (WMD = -0.49, 95% CI [-0.83, -0.15], P < 0.01), with no difference in other indicators.
CONCLUSION
robotic-assisted adrenalectomy is a superior technique to conventional laparoscopy in managing adrenal tumors, even in the case of a specific adrenal tumor - pheochromocytoma.
Topics: Adrenal Gland Neoplasms; Adrenalectomy; Humans; Laparoscopy; Length of Stay; Operative Time; Pheochromocytoma; Robotic Surgical Procedures; Treatment Outcome
PubMed: 36075556
DOI: 10.1016/j.ijsu.2022.106853 -
European Journal of Clinical... Feb 2021Current studies investigating the association between inhaled corticosteroid (ICS) use and risk of lung cancer have yielded inconsistent findings. The aim of this... (Meta-Analysis)
Meta-Analysis
INTRODUCTION
Current studies investigating the association between inhaled corticosteroid (ICS) use and risk of lung cancer have yielded inconsistent findings. The aim of this systematic review and meta-analysis was to pool all currently available data to estimate this association.
METHODS
We systematically searched MEDLINE (1946 to July 2020), EMBASE (1974 to July 2020) and the Cochrane Library (June 2020) via Ovid to identify relevant articles investigating the association between the ICS use and the risk of lung cancer. Random-effects analysis was used to calculate pooled relative risks (RRs) with 95% confidence intervals (CIs).
RESULTS
Ten articles including 234 920 patients were analysed. ICS use was identified to have a decreased risk of lung cancer in chronic obstructive pulmonary disease (8 studies, 1806 patients; RR = 0.73, 95% CI: 0.61-0.87, P < .01; I = 60.0 %), asthma (1 study, 41 438 patients; RR = 0.44, 95% CI: 0.34-0.57, P < .01) and mixed (1 study, 46 225 patients; RR = 0.79, 95% CI: 0.69-0.90, P < .01) patients. The findings of reduced risk of lung cancer were consistent in all subgroup analyses except for the short-term follow-up (≤5 years) (RR = 0.94, 95% CI: 0.81-1.07, P = .34) and free of immortal time bias (RR = 0.94, 95% CI: 0.82-1.08, P = .38) subgroups.
CONCLUSIONS
The present study suggested that ICS use was associated with decreased risk of lung cancer. However, our findings should be interpreted with caution because most original studies were judged to be at high risk of immortal time bias.
Topics: Administration, Inhalation; Adrenal Cortex Hormones; Asthma; Humans; Lung Neoplasms; Protective Factors; Pulmonary Disease, Chronic Obstructive
PubMed: 33053199
DOI: 10.1111/eci.13434 -
The Clinical Respiratory Journal Oct 2023Montelukast is a highly selective and specific cysteinyl leukotriene receptor antagonist used in the treatment of asthma. Whether montelukast as adjuvant therapy can... (Meta-Analysis)
Meta-Analysis Review
BACKGROUND
Montelukast is a highly selective and specific cysteinyl leukotriene receptor antagonist used in the treatment of asthma. Whether montelukast as adjuvant therapy can significantly and safely treat adults with cough variant asthma (CVA) remains inconclusive.
AIMS
This meta-analysis systematically evaluated the efficacy and safety of montelukast as an adjuvant treatment for adults with CVA.
MATERIALS AND METHODS
Randomized controlled trials (RCTs) on montelukast combined with inhaled corticosteroids (ICS) and long-acting β2 agonists (LABAs) to treat CVA in adults, from inception to March 6, 2023, were retrieved from the CNKI, Wanfang, VIP, CBM, PubMed, Embase, Cochrane Library, and Web of Science databases and Clinical Trials website. Review Manager (version 5.4) and Stata (version 15.0) were used to conduct the meta-analysis.
RESULTS
A total of 15 RCTs were ultimately included in the meta-analysis. It was established that montelukast as adjuvant therapy raised the total effective rate (RR = 1.20, 95% confidence interval [CI] [1.13, 1.27], P < 0.01) and improved the FEV1% (SMD = 0.91, 95% CI [0.40, 1.41], P < 0.01), PEF% (SMD = 0.63, 95% CI [0.38, 0.88], P < 0.01), FEV1 (SMD = 1.15, 95% CI [0.53, 1.77], P < 0.01), PEF (SMD = 0.64, 95% CI [0.42, 0.86], P < 0.01), and FEV1/FVC% (SMD = 0.76, 95% CI [0.51, 1.01], P < 0.01) and reduced the recurrence rate (RR = 0.28, 95% CI [0.15, 0.53], P < 0.01). The incidence of adverse reactions was higher in the montelukast auxiliary group compared to the control group but with no statistical difference (RR = 1.32, 95% CI [0.89, 1.96], P = 0.17).
CONCLUSION
Existing evidence indicated that the use of montelukast as an adjuvant therapy had therapeutic efficacy superior to ICS + LABA alone for the treatment of adult patients with CVA. However, further research is needed, especially a combination of high-quality long-term prospective studies and carefully designed RCTs.
Topics: Adult; Humans; Anti-Asthmatic Agents; Cough; Adrenergic beta-Agonists; Drug Therapy, Combination; Asthma; Adrenal Cortex Hormones
PubMed: 37218346
DOI: 10.1111/crj.13629 -
Psychoneuroendocrinology Sep 2018To perform a qualitative synthesis (systematic review) and quantitative analysis (meta-analysis) to summarize the evidence regarding the relationship between basal... (Meta-Analysis)
Meta-Analysis Review
OBJECTIVE
To perform a qualitative synthesis (systematic review) and quantitative analysis (meta-analysis) to summarize the evidence regarding the relationship between basal cortisol levels and metabolic syndrome (MetS) in adults.
METHODS
A systematic search was performed in the PubMed, Embase, and PsycINFO databases for observational studies on the association between basal cortisol levels and MetS. The quality of individual studies was assessed by the Newcastle-Ottawa score. A random effects model was used to report pooled quantitative results and the I statistic was used to assess heterogeneity. Egger's and Begg's tests were used to evaluate publication bias.
RESULTS
Twenty-six studies (19 cross-sectional and seven case-control) met the inclusion criteria for the systematic review. The majority was classified as having a low risk of bias and used established criteria for the diagnosis of MetS. Twenty-one studies provided data on basal cortisol levels as continuous values and were included in the meta-analysis; they comprised 35 analyses and 11,808 subjects. Pooled results showed no significant difference in basal cortisol levels between subjects with and without MetS (standardized mean difference [SMD] = 0.02, 95% confidence interval [CI]=-0.11 to 0.14). There was high heterogeneity between the studies when all comparisons were considered (I = 83.1%;p < 0.001). Paradoxically, meta-analysis of studies evaluating saliva samples showed no significantly lower basal cortisol levels among subjects with MetS (SMD=-0.18, 95% CI=-0.37 to 0.01), whereas those studies that evaluated serum samples (SMD = 0.11, 95% CI=-0.02 to 0.24) and urine samples (SMD = 0.73, 95% CI=-0.40 to 1.86) showed no significantly higher basal cortisol levels among subjects with MetS. In the subgroup and meta-regression analyses, a significant difference in basal cortisol levels was observed according to study design, population base, age, gender, cortisol level assessment method, and study quality.
CONCLUSION
This systematic review and meta-analysis does not reveal any association between basal cortisol levels and MetS based on results of observational studies. The results of a random-effect meta-analysis showed no significant difference in basal cortisol levels between subjects with and without MetS. The present findings should be considered in order to help future studies.
Topics: Adult; Aged; Case-Control Studies; Cross-Sectional Studies; Female; Humans; Hydrocortisone; Hypothalamo-Hypophyseal System; Male; Metabolic Syndrome; Middle Aged; Pituitary-Adrenal System
PubMed: 29800780
DOI: 10.1016/j.psyneuen.2018.05.023 -
Clinical Endocrinology Jul 2021To describe the presentation and outcomes of patients with adrenal ganglioneuromas (AGNs).
OBJECTIVE
To describe the presentation and outcomes of patients with adrenal ganglioneuromas (AGNs).
DESIGN
Single-centre retrospective cohort study (1 January 1995 to 31 December 2019) and systematic review of literature (1 January 1980 to 19 November 2019).
PATIENTS
Diagnosed with histologically confirmed AGN.
MEASUREMENTS
Baseline clinical, imaging and biochemical characteristics, recurrence rates and mortality. Subgroup analysis was performed on tumours with histologic elements of ganglioneuroma and pheochromocytoma (ie composite tumours).
RESULTS
The cohort study included 45 patients with AGN, 20 (44%) of which had composite tumours. Compared to pure AGN, patients with composite tumour were older (median age, 62.5 vs. 35 years, p < .001), had smaller tumours (median size, 3.9 vs. 5.7 cm, p = .016) and were discovered incidentally less frequently (65% vs. 84%, p = .009). No recurrences or ganglioneuroma-specific mortality occurred during follow-up (range, 0-266 months). The systematic review included 14 additional studies and 421 patients. The mean age of diagnosis was 39 years, and 47% were women. AGNs were discovered incidentally in 72% of patients, were predominantly unilateral (99%) and had a mean diameter of 5.8 cm and an unenhanced computed tomography (CT) attenuation of -118 to 49 Hounsfield units (HU). On imaging, 69% of AGNs were homogenous, 41% demonstrated calcifications, and 40% were lobulated.
CONCLUSIONS
AGNs are rare benign tumours that present with variable imaging features including large size, unenhanced CT attenuation >20 HU, calcifications and lobulated shape. Imaging characteristics can assist in establishing a diagnosis and avoiding an unnecessary adrenalectomy. The association of pheochromocytomas with AGNs is frequent. Diagnosis should include biochemical testing.
Topics: Adrenal Gland Neoplasms; Adult; Cohort Studies; Female; Ganglioneuroma; Humans; Middle Aged; Neoplasm Recurrence, Local; Retrospective Studies
PubMed: 33721367
DOI: 10.1111/cen.14460 -
Contemporary Clinical Trials Sep 2016Adrenal tumors are quite rare in infancy and childhood with the exception of neuroblastoma. In fact, adrenocortical tumors (ACT) account for only 0.2% of all malignant... (Review)
Review
Adrenal tumors are quite rare in infancy and childhood with the exception of neuroblastoma. In fact, adrenocortical tumors (ACT) account for only 0.2% of all malignant cancers in children and adolescents. According to a multicenter registry investigation, the median interval between first endocrine symptoms and the diagnosis of ACT is 5months, and death is seen in 38% of patients, who suffer from tumor progression following the diagnosis in about 2½years. The prognosis of pediatric ACC is poor with a 5-year event-free survival of 54%. To face this dreadful scenario, a few decades ago the International Pediatric Adrenocortical Tumor Registry (IPACTR) was established. Moreover, Children's Oncology Group (COG) and National Cancer Institute (NCI) have approved several clinical trials designed to investigate new treatment options in pediatric ACT. In this systematic review, we summarize the diagnostic histopathologic criteria, bio-markers, and clinical trials of this challenging diagnosis. Eleven pediatric ACT trials were reviewed in our investigation. Two out of 11 studies were conducted in Brazil showing apparently an increased rate of germline mutation-related pediatric ACT. A heterogeneous methodology was evident with four non-randomized clinical trials, three prospective cohort studies, and four retrospective case-control studies limiting higher statistical approach. Tumor histology remains the backbone to diagnose ACT creating a common investigative platform and potentially supporting studies aiming to increase international collaborative research, which is crucial for this challenging disease.
Topics: Adrenal Cortex Neoplasms; Adrenocortical Carcinoma; Biomarkers, Tumor; Child; Clinical Trials as Topic; Humans; Neoplasm Staging; Prognosis; Research Design; Survival Rate
PubMed: 27424218
DOI: 10.1016/j.cct.2016.07.011 -
Clinical Genitourinary Cancer Feb 2023Adrenocortical carcinoma (ACC) is a very rare endocrine cancer and is associated with a poor prognosis. There is a paucity of randomized clinical trials for this rare... (Review)
Review
A Systematic Review of Published Clinical Trials in the Systemic Treatment of Adrenocortical Carcinoma: An Initiative Led on Behalf of the Global Society of Rare Genitourinary Tumors.
Adrenocortical carcinoma (ACC) is a very rare endocrine cancer and is associated with a poor prognosis. There is a paucity of randomized clinical trials for this rare disease. We aimed to perform a systematic review of the literature on systemic therapy options in different stages of ACC. A systematic review was performed using Pubmed and Embase databases according to the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) statement. A total of 24 trials of systemic therapy in the treatment of ACC were identified and included in this review. Only one clinical trial in the adjuvant setting was identified, the negative phase III trial ADIUVO, which tested mitotane in low to intermediate-risk ACC patients. In the treatment of advanced ACC, cisplatin-based chemotherapy was evaluated in small and non-randomized phase II trials, and response rates ranged from 21% to 53.5%. The phase III trial FIRM-ACT compared etoposide, doxorubicin, cisplatin, and mitotane versus treatment with streptozotocin and mitotane and showed no difference in OS, but higher RR and PFS were reported with the multi-drug regimen. Six clinical trials of immunotherapy and seven studies of targeted therapy in advanced ACC were included, with modest activity and no phase 3 trials were identified. Treatment recommendations of ACC are based on retrospective and small studies with limited systemic therapy options. International and multi-center collaboration is essential to expand clinical research and improve outcomes.
Topics: Humans; Adrenocortical Carcinoma; Mitotane; Adrenal Cortex Neoplasms; Cisplatin; Retrospective Studies; Antineoplastic Combined Chemotherapy Protocols
PubMed: 36376169
DOI: 10.1016/j.clgc.2022.10.011 -
Gastroenterology Research and Practice 2017The most common pattern of esophageal cancer metastases (ECM) is to the lymph nodes, lung, liver, bones, adrenal glands, and brain. On the other hand, unexpected... (Review)
Review
The most common pattern of esophageal cancer metastases (ECM) is to the lymph nodes, lung, liver, bones, adrenal glands, and brain. On the other hand, unexpected metastasis (UM) spread to uncommon sites has increasingly reported and consequently affected the pathway of diagnosis, staging, and management. Using the PubMed database, a systematic search of the following headings "Esophageal" and "Metastasis" or "Metastases" was performed, 10049 articles were identified, and the articles were included if they demonstrated unexpected ECM. 84% of cases were men with an average age of 60.7 years. EC was located in the lower third in 65%. Two-thirds of the UM originated from the lower esophagus, and the two major histological types were adenocarcinoma 40% and squamous cell carcinoma 60%. Metastases were disseminated toward five main anatomical sites: the head and neck (42%), thoracic (17%), abdomen and pelvis (25%), extremities (9%), and multiple skin and muscle metastases (7%). The EC metastases were found to be synchronous 42% and metachronous 58%, isolated in 53.5% and multiple in 46.5%. The overall survival rate was 10.2 months. Since distant metastases are responsible for most EC-related deaths, understanding of ECM dissemination patterns needs more extensive studies. These critical data are the cornerstone of optimal cancer approach and treatment.
PubMed: 28659974
DOI: 10.1155/2017/1657310