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The Journal of Clinical Endocrinology... Jan 2021The characteristics of catecholamine-secreting pheochromocytomas have been well studied. However, less is known about the characteristics, management and outcome in... (Meta-Analysis)
Meta-Analysis
CONTEXT
The characteristics of catecholamine-secreting pheochromocytomas have been well studied. However, less is known about the characteristics, management and outcome in patients with ectopic adrenocorticotropic hormone (ACTH) and/or corticotrophin-releasing hormone (CRH)-secreting pheochromocytomas.
OBJECTIVE
To review the characteristics and outcomes of ACTH- and/or CRH-secreting pheochromocytomas.
DATA SOURCE
A systematic search of PubMed/MEDLINE and Web of Science, identifying relevant reports published up to 10 February 2020.
STUDY SELECTION
Original articles, including case reports and case series, reporting individual patient data from patients with ACTH- and/or CRH-secreting pheochromocytomas.
DATA EXTRACTION
Information on sex, age, symptoms at presentation, comorbidities, biochemistry, imaging, histopathology, and outcomes was extracted.
DATA SYNTHESIS
We identified 91 articles reporting on 99 cases of ACTH- and/or CRH-secreting pheochromocytomas (CRH-secreting n = 4). Median age at diagnosis was 49 years (interquartile range 38-59.5) with a 2:1 female to male ratio. Most patients presented with clinical Cushing syndrome (n = 79; 81%), hypertension (n = 87; 93%), and/or diabetes (n = 50; 54%). Blood pressure, glucose control, and biochemical parameters improved in the vast majority of patients postoperatively. Infections were the most common complication. Most cases (n = 70, 88%) with reported long-term outcome survived to publication (median follow-up 6 months).
CONCLUSION
Ectopic ACTH- and/or CRH-secreting pheochromocytoma should be considered in patients presenting with ACTH-dependent Cushing syndrome and adrenal mass. Despite the challenge in diagnosis, patient outcomes appear favorable.
Topics: Adrenal Gland Neoplasms; Adrenocorticotropic Hormone; Biomarkers; Corticotropin-Releasing Hormone; Humans; Pheochromocytoma; Prognosis
PubMed: 32710791
DOI: 10.1210/clinem/dgaa488 -
The Journal of Urology Oct 2023Incidental adrenal masses are common and require a multidisciplinary approach to evaluation and management that includes family physicians, urologists, endocrinologists,...
PURPOSE
Incidental adrenal masses are common and require a multidisciplinary approach to evaluation and management that includes family physicians, urologists, endocrinologists, and radiologists. The purpose of this guideline is to provide an updated approach to the diagnosis, management, and follow-up of adrenal incidentalomas, with a special focus on the areas of discrepancy/controversy existing among the published guidelines from other associations.
MATERIALS AND METHODS
This guideline was developed by the Canadian Urological Association (CUA) through a working group comprised of urologists, endocrinologists, and radiologists and subsequently endorsed by the American Urological Association (AUA). A systematic review utilizing the GRADE approach served as the basis for evidence-based recommendations with consensus statements provided in the absence of evidence. For each guideline statement, the strength of recommendation was reported as weak or strong, and the quality of evidence was evaluated as low, medium, or high.
RESULTS
The CUA working group provided evidence- and consensus-based recommendations based on an updated systematic review and subject matter expertise. Important updates on evidence-based radiological evaluation and hormonal testing are included in the recommendations. This guideline clarifies which patients may benefit from surgery and highlights where short term surveillance is appropriate.
CONCLUSION
Incidentally detected adrenal masses require a comprehensive assessment of hormonal function and oncologic risk. This guideline provides a contemporary approach to the appropriate clinical, radiographic, and endocrine assessments required for the evaluation, management, and follow-up of patients with such lesions.
Topics: Humans; Adrenal Gland Neoplasms; Follow-Up Studies; Canada; Incidental Findings
PubMed: 37556768
DOI: 10.1097/JU.0000000000003644 -
The Journal of Clinical Endocrinology... Jun 2023Adrenal hyperfunction is associated with an increased risk of cardiometabolic complications in subjects with adrenal incidentaloma (AI). Reliable prevalence estimates of... (Meta-Analysis)
Meta-Analysis
CONTEXT
Adrenal hyperfunction is associated with an increased risk of cardiometabolic complications in subjects with adrenal incidentaloma (AI). Reliable prevalence estimates of functioning AIs are important to direct resources allocations.
OBJECTIVE
To assess the prevalence of autonomous/possible autonomous cortisol secretion (ACS), primary aldosteronism (PA), pheochromocytoma (PHEO), and Cushing syndrome (CS) in patients with AI.
METHODS
We performed a comprehensive search of multiple databases (PubMed, Ovid MEDLINE, Web of Science) for potentially relevant studies without language restriction, up to February 2022. Of the 1661 publications evaluated at title and abstract levels, 161 were examined as full text and 36 were included. Study level clinical data were extracted by 3 independent reviewers.
RESULTS
The overall prevalence of functioning AIs was 27.5% (95% CI 23.0, 32.5). ACS/possible ACS, with a prevalence of 11.7% (95% CI 8.6, 15.7), was the most frequent hormonal alteration, while PA occurred in 4.4% of the patients (95% CI 3.1, 6.2). Subgroup analysis showed that PA was more prevalent in patients from Asia than in patients from Europe/America; in contrast, ACS/possible ACS had a lower prevalence in Asian countries. At meta-regression analysis, the prevalence of ACS/possible ACS was influenced by the proportion of female patients, while the prevalence of PA was positively associated with the proportion of patients with hypertension and the publication year. Finally, PHEO and CS prevalence were 3.8% (95% CI 2.8, 5.0) and 3.1% (95% CI 2.3, 4.3) respectively.
CONCLUSION
This meta-analysis provides extensive data on the prevalence of functioning AIs and the factors affecting heterogeneity in prevalence estimates.
Topics: Humans; Female; Adrenal Gland Neoplasms; Prevalence; Cushing Syndrome; Hypertension; Pheochromocytoma; Hydrocortisone
PubMed: 36718682
DOI: 10.1210/clinem/dgad044 -
Journal of Clinical Medicine Sep 2021To evaluate the risk factors of perioperative hemodynamic instability in pheochromocytoma, we conducted a systematic search of the literature using the Preferred... (Review)
Review
OBJECTIVE
To evaluate the risk factors of perioperative hemodynamic instability in pheochromocytoma, we conducted a systematic search of the literature using the Preferred Reporting Items for Systematic Reviews and Meta-analysis.
METHODS
In April 2021, we systematically searched PubMed, the Cochrane library, and Scopus for relevant studies on the risk factors of perioperative hemodynamic instability of adrenalectomy in patients with pheochromocytoma, and we subjected the findings from those studies to formal meta-analysis.
RESULTS
Our systematic review identified 14 studies involving 1725 patients, of which nine studies with 967 patients were eligible for meta-analysis. The results of meta-analysis showed that tumor size (odds ratio (OR): 1.14 for each increased cm, 95% confidence interval (CI) 1.03-1.26, z = 2.57) and urinary norepinephrine (OR, 1.51: 95% CI 1.26-1.81; z = 4.50) were most closely associated with the occurrence of perioperative hemodynamic instability.
CONCLUSION
These findings suggest that tumor size and urinary norepinephrine are important predictors and risk factors for perioperative hemodynamic instability in adrenalectomy for pheochromocytoma. Such findings may be of value to surgeons and anesthesiologists when considering or preparing for this procedure.
PubMed: 34640549
DOI: 10.3390/jcm10194531 -
Frontiers in Endocrinology 2023In patients with bilateral pheochromocytoma, partial adrenalectomy offers the chance to preserve adrenal function and avoid the need for lifelong steroid... (Meta-Analysis)
Meta-Analysis
BACKGROUND
In patients with bilateral pheochromocytoma, partial adrenalectomy offers the chance to preserve adrenal function and avoid the need for lifelong steroid supplementation. However, the risk of tumour recurrence raises questions about this procedure. The aim of our study was to compare partial and total adrenalectomy in bilateral pheochromocytoma through a systematic review with meta-analysis.
METHODS
A systematic search was carried out using databases (MEDLINE, EMBASE, Scopus, Web of Science, CENTRAL) and registers of clinical trials (ClinicalTrials.gov, European Trials Register, WHO International Trials Registry Platform). This meta-analysis included studies up to July 2022 without language restrictions. A random effects model meta-analysis was performed to assess the risk of tumor recurrence, steroid dependence and morbidity in these patients.
RESULTS
Twenty-five studies were included in the analysis involving 1444 patients. The relative risk (RR) of loss of adrenal hormone function during follow-up and the need for steroid therapy was 0.32 in patients after partial adrenalectomy: RR 0.32, 95% Confidence Interval (CI): 0.26-0.38, P < 0.00001, I2 = 21%. Patients undergoing partial adrenalectomy had a lower odds ratio (OR) for developing acute adrenal crisis: OR 0.3, 95% CI: 0.1-0.91, P=0.03, I2 = 0%. Partial adrenalectomy was associated with a higher risk of recurrence than total adrenalectomy: OR 3.72, 95% CI: 1.54-8.96, P=0.003, I2 = 28%.
CONCLUSION
Partial adrenalectomy for bilateral pheochromocytoma is a treatment that offers a chance of preserving adrenal hormonal function, but is associated with a higher risk of local tumor recurrence. There was no difference for the risk of metastasis and in overall mortality among the group with bilateral pheochromocytomas undergoing total or partial adrenalectomy. This study is in line with PRISMA (Preferred Reporting Items for Systematic Reviews and Meta-Analyses) and AMSTAR (A Measurement Tool to Assess Systematic Reviews) Guidelines (10, 11).
SYSTEMATIC REVIEW REGISTRATION
https://osf.io/zx3se.
Topics: Humans; Pheochromocytoma; Neoplasm Recurrence, Local; Adrenalectomy; Adrenal Gland Neoplasms
PubMed: 36998480
DOI: 10.3389/fendo.2023.1127676 -
Clinical Endocrinology Nov 2017The outcomes of patients with metastatic phaeochromocytoma (PHEO) and paraganglioma (PGL) are unclear. We performed a systematic review and meta-analysis of baseline... (Meta-Analysis)
Meta-Analysis Review
OBJECTIVE
The outcomes of patients with metastatic phaeochromocytoma (PHEO) and paraganglioma (PGL) are unclear. We performed a systematic review and meta-analysis of baseline characteristics and mortality rates of patients with metastatic PHEO and PGL (PPGL).
DESIGN
Ovid MEDLINE In-Process & Other Non-Indexed Citations, Ovid MEDLINE, Ovid EMBASE, Ovid Cochrane Central Register of Controlled Trials, Ovid Cochrane Database of Systematic Reviews, Scopus, Web of Science, and references of key articles were searched from inception to 2016.
PATIENTS
Studies comprised ≥20 patients with metastatic PPGL and reported baseline characteristics and follow-up data.
MEASUREMENTS
Reviewers extracted standardized data and assessed risk of bias using a modified Newcastle-Ottawa tool. Random-effects meta-analysis was used to pool event rates across studies.
RESULTS
Twenty retrospective noncomparative studies reported on 1338 patients with metastatic PHEO (685/1296, 52.9%) and PGL (611/1296, 47.1%), diagnosed at a mean age of 43.9 ± 5.2 years. Mean follow-up was 6.3 ± 3.2 years. Of 532 patients with reported data, 40.4% had synchronous metastases. Five-year (7 studies, n = 738) and 10-year (2 studies, n = 55) mortality rates for patients with metastatic PPGL were 37% (95% CI, 24%-51%) and 29% (95% CI, 17%-42%), respectively. Higher mortality was associated with male sex (RR 1.50; 95% CI, 1.11-2.02) and synchronous metastases (RR 2.43; 95% CI, 1.01-5.85).
CONCLUSIONS
Available low-quality evidence from heterogeneous studies suggests low mortality rates of patients with metastatic PPGL. Male sex and synchronous metastases correlated with increased mortality. The outcomes of patients with metastatic PPGL have been inadequately assessed, indicating the need for carefully planned prospective studies.
Topics: Adrenal Gland Neoplasms; Adult; Female; Humans; Male; Middle Aged; Mortality; Neoplasm Metastasis; Paraganglioma; Pheochromocytoma; Treatment Outcome
PubMed: 28746746
DOI: 10.1111/cen.13434 -
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 -
Clinical Endocrinology Nov 2014Chemotherapy with cyclophosphamide, vincristine and dacarbazine (CVD) can be used for palliative treatment of malignant pheochromocytoma and paraganglioma. However, the... (Meta-Analysis)
Meta-Analysis Review
BACKGROUND
Chemotherapy with cyclophosphamide, vincristine and dacarbazine (CVD) can be used for palliative treatment of malignant pheochromocytoma and paraganglioma. However, the precise effect of this chemotherapeutic regimen on tumour volume is unclear. The main objective of this study was to perform a systematic review and meta-analysis assessing the effect of chemotherapy with CVD on tumour volume in patients with malignant paraganglioma/pheochromocytoma.
METHODS
A literature search was performed in October 2013 to identify potentially relevant studies. Main outcomes were the pooled percentages of complete response, partial response and stable disease after chemotherapy with CVD. A meta-analysis was performed with an exact likelihood approach using a logistic regression. Pooled percentages with 95% confidence intervals (CI) were reported.
RESULTS
Four studies concerning a total of 50 patients with malignant paraganglioma/pheochromocytoma reported on treatment with a combination of CVD chemotherapy. A meta-analysis of the effect of chemotherapy on tumour volume showed pooled percentages of complete response, partial response and stable disease of, respectively, 4% (95% CI: 1%-15%), 37%(95% CI: 25%-51%) and 14% (95% CI: 7%-27%). Only two studies concerning a total of 35 patients assessed the response on catecholamine excess; pooled percentages for complete, partial and stable hormonal response were 14% (95% CI: 6%-30%), 40% (95% CI: 25%-57%) and 20% (95% CI: 10%-36%), respectively. Duration of response was also reported in only two studies with a median duration of response of 20 months and 40 months.
CONCLUSIONS
Data on the effects of a combination of CVD chemotherapy on malignant paraganglioma/pheochromocytoma suggest that a partial response concerning tumour volume can be achieved in about 37% of patients and a partial response on catecholamine excess in about 40% of patients. However, in the included studies, the protocol when to initiate treatment was not well described. Therefore, it cannot be excluded that the reported effect of chemotherapy on tumour volume reflects the natural course of the disease, at least partially.
Topics: Adrenal Gland Neoplasms; Antineoplastic Combined Chemotherapy Protocols; Catecholamines; Cyclophosphamide; Dacarbazine; Humans; Paraganglioma; Pheochromocytoma; Tumor Burden; Vincristine
PubMed: 25041164
DOI: 10.1111/cen.12542 -
Minerva Endocrinologica Mar 2017Long-term outcome of patients with adrenal incidentaloma (AI) is unknown. The aim of this study was to systematically summarize the follow-up and outcome of clinically... (Meta-Analysis)
Meta-Analysis Review
INTRODUCTION
Long-term outcome of patients with adrenal incidentaloma (AI) is unknown. The aim of this study was to systematically summarize the follow-up and outcome of clinically silent AI who do not undergo surgery.
EVIDENCE ACQUISITION
All major databases and medical literature in English-language, published from 1998 to May 2015, were systematically searched for publications on AI. Primary endpoint was hormonal hyper function; secondary endpoints were time from diagnosis to study endpoint and the outcome of adrenalectomy. Meta-analysis was performed using both qualitative and quantitative approach.
EVIDENCE SYNTHESIS
A total of 11 publications were included. Total sample size was 1298 patients. Mean follow-up duration was 44.2 months. There were 82 patients confirmed to have subclinical Cushing's syndrome at diagnosis, with 1.79% new cases at the end of follow up (95% CI, 0.002 to 0.045). Incidence of Cushing's syndrome was 0.7% (95% CI, 0.001 to 0.013) and pheochromocytoma 0.4% (95% CI, 0.001 to 0.008). The mean tumor size was 2.52cm, with mean increment of 0.03cm to 2.9cm at the end of follow up. About 3% of patients ended up with surgery (95% CI, 0.01 to 0.05) but none were due to primary adrenal malignancy. Time of greatest risk of developing Cushing's syndrome and pheochromocytoma was between months 36 and 42 (hazard rate 14%), and between months 48 and 54 (hazard rate 7%) respectively.
CONCLUSIONS
Malignant change in non-functioning AI is rare. The risk of developing overt disease over the follow-up period is low. A less stringent imaging and functional work-up interval can be considered.
Topics: Adrenal Gland Neoplasms; Disease Progression; Humans; Treatment Outcome
PubMed: 26698544
DOI: 10.23736/S0391-1977.16.02394-4 -
Cureus Apr 2023Pheochromocytoma (PCC) is a neuroendocrine tumor that may present with headaches, palpitations, and hypertension, and if left unresected, it can lead to serious... (Review)
Review
Pheochromocytoma (PCC) is a neuroendocrine tumor that may present with headaches, palpitations, and hypertension, and if left unresected, it can lead to serious complications and fatal cardiac mortality. Adequate preoperative management can decrease the risk of intraoperative complications. In this systematic review, we address and discuss what has been published in the literature about the optimization of pheochromocytoma preoperative care via various types of telemedicine (TM). We searched health research databases PubMed, Medical Literature Analysis and Retrieval System Online (MEDLINE), the Cochrane Library, and Google Scholar for literature on various types of TM employed for PCC preoperative management. We searched peer-reviewed literature in the English language published in the literature until November 5, 2022, using medical subject heading (MeSh) terms in PubMed like "telemedicine" and "pheochromocytoma." We used "telemedicine" or "telehealth" and "pheochromocytoma" in other databases. We considered all types of TM, including synchronous, asynchronous, and remote patient monitoring. Our search yielded five publications in PubMed, 59 results in Google Scholar, and none in the Cochrane Library. After excluding duplicates and evaluating the articles for relevance, five papers were selected for this review. Studies came from the United States and Italy. Findings from these studies suggested safe outcomes and reduced costs compared to what is traditionally followed in physical settings. Overall, this systematic review shows the convenience and safety of TM use for a broad spectrum of patients. Further studies are needed to consolidate these findings. Moreover, guidelines on patients' selection and procedures for safe and effective TM care for patients with PCC are required.
PubMed: 37255909
DOI: 10.7759/cureus.38290