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Perfusion May 2020Phaeochromocytoma is a catecholamine-secreting tumour associated with clinical presentation ranging from paroxysmal hypertension to intractable cardiogenic shock....
INTRODUCTION
Phaeochromocytoma is a catecholamine-secreting tumour associated with clinical presentation ranging from paroxysmal hypertension to intractable cardiogenic shock. Extracorporeal life support, in veno-arterial mode, application in refractory acute heart dysfunction is sharply increasing worldwide. However, its clinical utility in phaeochromocytoma-induced cardiogenic shock remains still unclear.
METHODS
A systematic review of published reports was performed in accordance with the Preferred Reporting Items for Systematic Reviews and Meta-Analysis Statement. Searches were accomplished on PubMed, Embase and Google Scholar to identify articles describing the use of extracorporeal life support in the setting of phaeochromocytoma-induced cardiogenic shock (PROSPERO: CRD42019125225).
RESULTS
Thirty-five reports, including 62 patients supported with extracorporeal life support because of intractable phaeochromocytoma crisis, were included for the analysis. Almost all the subjects underwent peripheral cannulation for extracorporeal life support. The median duration of the mechanical circulatory support was 5 days, and most of the patients recovered normal myocardial function (left ventricular ejection fraction ⩾50%). In-hospital survival was 87%. Phaeochromocytoma was removed surgically during extracorporeal life support in 10 patients (16%), while in the remaining after haemodynamic stabilization and weaning from the mechanical support.
CONCLUSION
Successful management of phaeochromocytoma-induced cardiogenic shock depends on prompt recognition and immediate treatment of shock. In this scenario, extracorporeal life support may play a significant role allowing cardiac and end-organ recovery and giving time for accurate diagnosis and specific treatment.
Topics: Adrenal Gland Neoplasms; Adult; Extracorporeal Membrane Oxygenation; Female; Humans; Male; Middle Aged; Pheochromocytoma; Shock, Cardiogenic
PubMed: 32397890
DOI: 10.1177/0267659120908413 -
Clinical Cardiology May 2020Innumerable physical stress factors including externally administered catecholamines, and pheochromocytomas and paragangliomas (PPGLs) have been reported to trigger... (Meta-Analysis)
Meta-Analysis
Clinical features, complications, and outcomes of exogenous and endogenous catecholamine-triggered Takotsubo syndrome: A systematic review and meta-analysis of 156 published cases.
Innumerable physical stress factors including externally administered catecholamines, and pheochromocytomas and paragangliomas (PPGLs) have been reported to trigger Takotsubo syndrome (TS). A systematic search of PubMed/MEDLINE identified 156 patients with catecholamine-induced TS up to December 2017. Data were compared within the catecholamine-induced TS cohort, but some comparisons were also done to a previously published large all-TS cohort (n = 1750). The mean age was 46.4 ± 16.4 years (72.3% women). The clinical presentation was dramatic with high complication rates in (68.2%, n = 103; multiple complications 34.6%, n = 54). The most common TS ballooning pattern was apical or mid-apical (45.2%, n = 69), followed by basal pattern (28.8%, n = 45), global pattern (16.0%, n = 25), mid-ventricular (8.3%, n = 13), focal (0.6%, n = 1), and unidentified pattern (1.9%, n = 3). There was an increase in the prevalence of apical sparing ballooning pattern compared to all-TS population (37.7% vs 18.3%, P < .00001). Higher complication rates were observed in TS with global ballooning pattern compared to apical ballooning pattern (23/25, 92% vs 38/65, 58.5%; P = .0022). Higher complication rates were observed in patients with age < 50 years than patients >50 years (73/92, 79.3% vs 29/56, 51.8%, P = 0.0009). Recurrence occurred exclusively in patients with PPGL-induced TS (18/107 patients, 16.8%). PPGL-induced TS was characterized by more global ballooning's pattern (22/104, 21.2% vs 3/49, 6.1%, P = 0.02), and lower left ventricular ejection fraction (25.54 ± 11.3 vs 31.82 ± 9.93, P = 0.0072) compared to exogenous catecholamine-induced TS. In conclusion, catecholamine-induced TS was characterized by a dramatic clinical presentation with extensive left ventricular dysfunction, and high complication rate.
Topics: Adult; Aged; Biomarkers; Catecholamines; Female; Humans; Male; Middle Aged; Norepinephrine; Phenylephrine; Stroke Volume; Takotsubo Cardiomyopathy; Ventricular Function, Left
PubMed: 32125009
DOI: 10.1002/clc.23352 -
European Journal of Surgical Oncology :... Jun 2020To evaluate the efficacy and safety of laparoscopic adrenalectomy (LA) vs open adrenalectomy (OA) for pheochromocytoma (PHEO). (Meta-Analysis)
Meta-Analysis
PURPOSE
To evaluate the efficacy and safety of laparoscopic adrenalectomy (LA) vs open adrenalectomy (OA) for pheochromocytoma (PHEO).
METHODS
A systematic literature research of PubMed, Ovid, Scopus, and citation lists were performed to identify eligible studies. All studies comparing LA versus OA for PHEO were included.
RESULTS
Overall, fourteen studies including 743 patients (LA 391; OA 352) were included. LA might have smaller tumor size (WMD -0.92 cm, 95% CI -1.09 to -0.76; p < 0.001) and higher body mass index (BMI) (WMD 0.31 kg/m, 95% CI 0.04 to 0.58; p = 0.02). Compared to OA, LA showed lower estimated blood loss (EBL) (WMD -207.72 ml, 95% CI -311.26, -104.19; p < 0.001), lower transfusion rate (OR 0.25, 95% CI 0.16 to 0.38; p < 0.001), lower hemodynamic instability (HI) (OR 0.61, 95% CI 0.42 to 0.88; p = 0.009), less postoperative complications (OR 0.55, 95% CI 0.34 to 0.89; p = 0.02), less Clavien Dindo score ≥3 complications (OR 0.51, 95% CI 0.27 to 0.97; p = 0.04), shorter return to diet time (WMD -0.76 days, 95% CI -1.27 to -0.25; p = 0.003), and shorter length of hospital stay (WMD -1.76 days, 95% CI -2.94 to -0.58; p < 0.001). The subgroup analysis of studies since 2008 showed consistent results.
CONCLUSION
LA shows a feasible, safe and superior treatment option for PHEO, because it provides superior perioperative and recovery outcomes without increasing complications.
Topics: Adrenal Gland Neoplasms; Adrenalectomy; Humans; Laparoscopy; Operative Time; Pheochromocytoma; Retroperitoneal Space
PubMed: 32102743
DOI: 10.1016/j.ejso.2020.02.009 -
The British Journal of Surgery Jan 2020Preoperative α-blockade in phaeochromocytoma surgery is recommended by all guidelines to prevent intraoperative cardiocirculatory events. The aim of this meta-analysis... (Comparative Study)
Comparative Study Meta-Analysis
BACKGROUND
Preoperative α-blockade in phaeochromocytoma surgery is recommended by all guidelines to prevent intraoperative cardiocirculatory events. The aim of this meta-analysis was to assess the benefit of such preoperative treatment compared with no treatment before adrenalectomy for phaeochromocytoma.
METHODS
A systematic literature search was undertaken in MEDLINE, Web of Science and CENTRAL without language restrictions. Randomized and non-randomized comparative studies investigating preoperative α-blockade in phaeochromocytoma surgery were included. Data on perioperative safety, effectiveness and outcomes were extracted. Pooled results were calculated as an odds ratio or mean difference with 95 per cent confidence interval.
RESULTS
A total of four retrospective comparative studies were included investigating 603 patients undergoing phaeochromocytoma surgery. Mortality, cardiovascular complications, mean maximal intraoperative systolic and diastolic BP, and mean maximal intraoperative heart rate did not differ between patients with or without α-blockade. The certainty of the evidence was very low owing to the inferior quality of studies.
CONCLUSION
This meta-analysis has shown a lack of evidence for preoperative α-blockade in surgery for phaeochromocytoma. RCTs are needed to evaluate whether preoperative α-blockade can be abandoned.
Topics: Adrenal Gland Neoplasms; Adrenalectomy; Adrenergic alpha-Antagonists; Cardiovascular Diseases; Humans; Pheochromocytoma; Preoperative Care
PubMed: 31903584
DOI: 10.1002/bjs.11348 -
Journal of Medical Genetics Apr 2020Pheochromocytoma and paraganglioma (PPGL) are tumours that arise from chromaffin cells. Some genetic mutations influence PPGL, among which, those in genes encoding... (Meta-Analysis)
Meta-Analysis
BACKGROUND
Pheochromocytoma and paraganglioma (PPGL) are tumours that arise from chromaffin cells. Some genetic mutations influence PPGL, among which, those in genes encoding subunits of succinate dehydrogenase (SDHA, SDHB, SDHC and SDHD) and assembly factor (SDHAF2) are the most relevant. However, the risk of metastasis posed by these mutations is not reported except for SDHB and SDHD mutations. This study aimed to update the metastatic risks, considering prevalence and incidence of each SDHx mutation, which were dealt formerly all together.
METHODS
We searched EMBASE and MEDLINE and selected 27 articles. The patients included in the studies were divided into three groups depending on the presence of PPGL. We checked the heterogeneity between studies and performed a meta-analysis using Hartung-Knapp-Sidik-Jonkman method based on a random effect model.
RESULTS
The highest PPGL prevalence was for SDHB mutation, ranging from 23% to 31%, and for SDHC mutation (23%), followed by that for SDHA mutation (16%). The lowest prevalence was for SDHD mutation, ranging from 6% to 8%. SDHAF2 mutation showed no metastatic events. The PPGL incidence showed a tendency similar to that of its prevalence with the highest risk of metastasis posed by SDHB mutation (12%-41%) and the lowest risk by SDHD mutation (~4%).
CONCLUSION
There was no integrated evidence of how SDHx mutations are related to metastatic PPGL. However, these findings suggest that SDHA, SDHB and SDHC mutations are highly associated and should be tested as indicators of metastasis in patients with PPGL.
Topics: Adrenal Gland Neoplasms; Electron Transport Complex II; Germ-Line Mutation; Heterozygote; Humans; Membrane Proteins; Mitochondrial Proteins; Neoplasm Metastasis; Paraganglioma; Pheochromocytoma; Succinate Dehydrogenase
PubMed: 31649053
DOI: 10.1136/jmedgenet-2019-106324 -
Clinical Endocrinology Dec 2019Inoperable and metastatic pheochromocytomas and paragangliomas (PPGLs) present a therapeutic challenge with current treatment options being limited to radiolabelled... (Meta-Analysis)
Meta-Analysis
OBJECTIVE
Inoperable and metastatic pheochromocytomas and paragangliomas (PPGLs) present a therapeutic challenge with current treatment options being limited to radiolabelled meta-iodo-benzyl-guanidine (MIBG) and systemic chemotherapy. Peptide receptor radionuclide therapy (PRRT) seems to be a promising option for these patients with few studies reporting favourable response. This systematic review was conducted to evaluate the efficacy and safety of PRRT in patients with advanced PPGLs.
METHODS
This review followed the Preferred Reporting Items for Systematic Reviews and Meta-Analysis (PRISMA) guidelines. Searches in PubMed, Scopus and Embase were made using relevant keywords and articles up to May 2019 were included. Data on efficacy and toxicity were extracted from the individual articles, and pooled estimates were generated using meta-analysis.
RESULTS
Twelve articles consisting of 201 patients with advanced PPGLs were included. Overall, treatment with PRRT achieved an objective response rate of 25% (95% CI: 19%-32%) and a disease control rate of 84% (95% CI: 77%-89%). Clinical and biochemical responses were seen in 61% and 64% of the patients, respectively. Among the PRRTs, similar tumour response rates were noted for Y-yttrium- and Lu-lutetium-based agents. Treatment-related adverse effects were minimal with grade 3/4 neutropenia, thrombocytopenia, lymphopenia and nephrotoxicity observed in 3%, 9%, 11% and 4% of the patients, respectively. Treatment discontinuation was noted in five out of 102 patients.
CONCLUSIONS
Peptide receptor radionuclide therapy is a safe and efficacious treatment option for advanced PPGLs and may be considered a viable alternative to chemotherapy and I- MIBG.
Topics: Female; Heterocyclic Compounds; Humans; Male; Octreotide; Organometallic Compounds; Paraganglioma; Peptides, Cyclic; Pheochromocytoma; Radiopharmaceuticals; Receptors, Peptide
PubMed: 31569282
DOI: 10.1111/cen.14106 -
Mayo Clinic Proceedings Oct 2019To assess the diagnostic accuracy of unenhanced computed tomography (CT) attenuation values to exclude a pheochromocytoma in the diagnostic work-up of patients with an... (Meta-Analysis)
Meta-Analysis
OBJECTIVES
To assess the diagnostic accuracy of unenhanced computed tomography (CT) attenuation values to exclude a pheochromocytoma in the diagnostic work-up of patients with an adrenal incidentaloma and to model the associated difference in diagnostic costs.
METHODS
The MEDLINE and Embase databases were searched from indexing to September 27, 2018, and studies reporting the proportion of pheochromocytomas on either side of the 10-Hounsfield unit (HU) threshold on unenhanced CT were included. The pooled proportion of pheochromocytomas with an attenuation value greater than 10 HU was determined, as were the modeled financial costs of the current and alternative diagnostic approaches.
RESULTS
Of 2957 studies identified, 31 were included (N=1167 pheochromocytomas). Overall risk of bias was low. Heterogeneity was not observed between studies (Q=11.5, P=.99, I=0%). The pooled proportion of patients with attenuation values greater than 10 HU was 0.990 (95% CI, 0.984-0.995). The modeled financial costs using the new diagnostic approach were €55 (∼$63) lower per patient.
CONCLUSION
Pheochromocytomas can be reliably ruled out in the case of an adrenal lesion with an unenhanced CT attenuation value of 10 HU or less. Therefore, determination of metanephrine levels can be restricted to adrenal tumors with an unenhanced CT attenuation value greater than 10 HU. Implementing this novel diagnostic strategy is cost-saving.
Topics: Adrenal Gland Neoplasms; Costs and Cost Analysis; Diagnosis, Differential; Humans; Incidental Findings; Pheochromocytoma; Reproducibility of Results; Tomography, X-Ray Computed
PubMed: 31515105
DOI: 10.1016/j.mayocp.2019.03.030