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Abstract Context Pretreatment with α-adrenergic receptor blockers is recommended to prevent hemodynamic instability during resection of a pheochromocytoma or sympathetic paraganglioma (PPGL). Objective To determine which type of α-adrenergic receptor blocker provides the best efficacy. Design Randomized controlled open-label trial (PRESCRIPT; ClinicalTrials.gov NCT01379898) Setting Multicenter study including 9 centers in The Netherlands. Patients 134 patients with nonmetastatic PPGL. Intervention Phenoxybenzamine or doxazosin starting 2 to 3 weeks before surgery using a blood pressure targeted titration schedule. Intraoperative hemodynamic management was standardized. Main Outcome Measures Primary efficacy endpoint was the cumulative intraoperative time outside the blood pressure target range (ie, SBP >160 mmHg or MAP <60 mmHg) expressed as a percentage of total surgical procedure time. Secondary efficacy endpoint was the value on a hemodynamic instability score. Results Median cumulative time outside blood pressure targets was 11.1% (interquartile range [IQR]: 4.3–20.6] in the phenoxybenzamine group compared to 12.2% (5.3–20.2)] in the doxazosin group (P = .75, r = 0.03). The hemodynamic instability score was 38.0 (28.8–58.0) and 50.0 (35.3–63.8) in the phenoxybenzamine and doxazosin group, respectively (P = .02, r = 0.20). The 30-day cardiovascular complication rate was 8.8% and 6.9% in the phenoxybenzamine and doxazosin group, respectively (P = .68). There was no mortality after 30 days. Conclusions The duration of blood pressure outside the target range during resection of a PPGL was not different after preoperative treatment with either phenoxybenzamine or doxazosin. Phenoxybenzamine was more effective in preventing intraoperative hemodynamic instability, but it could not be established whether this was associated with a better clinical outcome.

Background The survival of dogs with pheochromocytoma (PCC) treated with adrenoreceptor antagonists has not been described or compared to surgically managed cases. Hypothesis/Objectives The objective of this study is to evaluate the survival of medically and surgically managed dogs with PCC and investigate factors associated with survival. Animals Two hundred fifty‐five dogs with PCC, treated with alpha‐adrenoreceptor antagonists (AA) without adrenalectomy (Group 1, n = 75), adrenalectomy +/– AA (Group 2, n = 128), or neither treatment (Group 3, n = 52). Methods Retrospective, multicenter review of medical records. Median overall survival time (OST) for Groups 1 and 2 combined was calculated using Kaplan–Meier estimates, and then compared between Group 1 and Group 2 using Log‐Rank testing. Cox proportional hazard analysis identified factors associated with survival in Groups 1 and 2 individually and combined. Results Median OST for all cases was 854 (95% CI: 572–1136) days. Median OST was lower in Group 1 (247 days, 95% CI: 76–418 days) than in Group 2 (927 days, 95% CI: 587–1267 days; p < 0.001). In Group 2, 88/92 dogs (97.8%) that received presurgical AA treatment survived to discharge compared to 23/27 (85.2%) that did not receive AA pretreatment (p = 0.03). Lack of clinical signs at presentation was associated with increased survival in both groups combined (HR 0.5; 95% CI 0.3–0.9; p = 0.02) and in Group 2 alone (HR 0.3; 95% CI 0.1–0.7; p = 0.01). Conclusions and Clinical Importance Dogs with PCC treated with adrenalectomy have longer survival compared to those managed with AA without adrenalectomy.

Laparoscopic adrenalectomy (LA) is normally used to treat small-sized (<6 cm) pheochromocytoma (PCC). This study evaluated the effectiveness and safety of LA for treating large (≥6 cm) PCC. Fifty-one patients with resectable, large-sized (≥6 cm) PCC were prospectively enrolled for elective LA (n = 23) or open adrenalectomy (n = 28). LA was converted into open adrenalectomy in 2 patients (2/23, 8.7%); LA was associated with relatively longer operative time (P = .033) but less intraoperative bleeding (P < .001), faster resumption of ambulatory status (P < .001), and shorter duration of postoperative hospitalization (P < .001). Frequency of PCC recurrence was similar between the 2 groups (P = 1.000). LA is a feasible, effective, and safe treatment modality for large-sized (≥6 cm) PCC. LA is associated with minimal invasiveness and faster postoperative recovery. •LA had a longer operative time for treating large-sized pheochromocytoma.•LA was superior in minimal invasiveness and faster postoperative recovery.•LA had a risk of tumor recurrence similar to open procedure.

Pheochromocytoma and paraganglioma are related tumors that differ mainly in location; pheochromocytomas are adrenal, and paragangliomas can be anywhere else. The symptoms are protean and common, but the tumors are rare. The diagnosis may signify an underlying genetic syndrome.

Background Laparoscopic adrenalectomy for pheochromocytoma remains subject of debate, owing to the systemic consequences of pneumoperitoneum in patients with catecholamine-secreting tumors. Methods A prospective randomized study was conducted (2000–2006), evaluating cardiovascular instability during open ( n  = 9, group A) or laparoscopic ( n  = 13, group B) adrenalectomy for pheochromocytoma. Haemodynamic parameters were recorded by invasive monitoring. Results Haemodynamic instability was observed in 3/9 (group A) and 6/13 patients (group B), with a mean of 1.8 and 2.2 hypertensive peaks per patient ( p  = n.s.). Blood loss (164 ± 94 cc versus 48 ± 36 cc, p  < 0.05) and operative time (180 ± 40 versus 158 ± 45 min, p  = n.s.) favored laparoscopic procedures. Postoperative morbidity and mortality were nil. Hospital stay was shorter in group B ( p  < 0.05). Long-term follow-up was always normal. Conclusions Laparoscopic approach for pheochromocytoma can be as safe as open surgery; intraoperative haemodynamic instability, although usually controlled with success, remains a source of concern.

ContextMalignant pheochromocytoma (PHEO) and paraganglioma (PGL) are rare and knowledge of the natural history is limited.ObjectiveWe aimed to describe baseline characteristics and outcomes of patients with malignant PHEO and PGL (PPGL) and to identify predictors of shorter survival.DesignRetrospective review of patients with malignant PPGL evaluated from 1960 to 2016.SettingReferral center.PatientsThe group comprised 272 patients.Main Outcome MeasuresBaseline description, survival outcomes, and predictors of shorter survival were evaluated in patients with rapidly progressive (n = 29) and indolent disease (n = 188).ResultsMalignant PPGL was diagnosed at a median age of 39 years (range, 7 to 83 years), with synchronous metastases in 96 (35%) patients. In 176 (65%) patients, metastases developed at a median of 5.5 years (range, 0.3 to 53.4 years) from the initial diagnosis. Median follow-up was 8.2 years (range, 0.01 to 54.1 years). Median overall and disease-specific survivals were 24.6 and 33.7 years, respectively. Shorter survival correlated with male sex (P = 0.014), older age at the time of primary tumor (P = 0.0011), synchronous metastases (P < 0.0001), larger primary tumor size (P = 0.0039), elevated dopamine (P = 0.0195), and not undergoing primary tumor resection (P < 0.0001). There was no difference in the type of primary tumor or presence of SDHB mutation.ConclusionsThe clinical course of patients with malignant PPGL is remarkably variable. Rapid disease progression is associated with male sex, older age at diagnosis, synchronous metastases, larger tumor size, elevated dopamine, and not undergoing resection of primary tumor. An individualized approach to patients with metastatic PPGL is warranted.We studied patients with malignant pheochromocytoma and paraganglioma. Overall and disease-specific survivals were 24.6 and 33.7 years, respectively. Patients had a markedly variable clinical course.

Pheochromocytomas are rare tumors of chromaffin cells for which the optimal management is surgical resection. Precise diagnosis and localization may be elusive. We evaluated whether positron emission tomography (PET) scanning with the combination of [18F]fluorodeoxyglucose (FDG) and the norepinephrine analogue [11C]metahydroxyephedrine (mHED) would allow more exact diagnosis and localization. Fourteen patients with suspected pheochromocytoma were evaluated by anatomical imaging (computed tomography or magnetic resonance imaging) and [131I]metaiodobenzylguanidine (MIBG) planar imaging. PET imaging was performed by using mHED with dynamic adrenal imaging, followed by a torso survey and FDG with a torso survey. Images were evaluated qualitatively by an experienced observer. Eight patients had pathology-confirmed pheochromocytoma. Of the other six, two patients had normal adrenal tissue at adrenalectomy, and the other four had subsequent clinical courses inconsistent with a diagnosis of pheochromocytoma. In four of eight patients with pheochromocytoma, MIBG failed to detect one or more sites of pathology-confirmed disease. The mHED-PET detected all sites of confirmed disease, whereas FDG-PET detected all sites of adrenal and abdominal disease, but not bone metastases, in one patient. MIBG and FDG-PET results were all negative in the six patients without pheochromocytoma. One patient with adrenal medullary hyperplasia had a positive mHED-PET scan. PET scanning aided the decision not to operate in three of six patients. The resolution of PET functional imaging was superior to that of MIBG. PET scanning for pheochromocytoma offers improved quality and resolution over current diagnostic approaches. PET may significantly influence the clinical management of patients with a suspicion of these tumors and warrants further investigation.

Abstract Context Modern pheochromocytomas (PHEOs) are often discovered by incidental finding on cross-sectional imaging or mutation-based genetic case detection testing. Little is known about how these PHEOs behave. Objective To describe the characteristics and behavior of PHEOs discovered incidentally on imaging or through mutation-based genetic case detection testing. Design Retrospective study. Setting Referral center. Patients Consecutive patients with pathology-confirmed PHEOs, treated from 2005 to 2016. Main Outcome Measure(s) Tumor size, plasma/urine fractionated metanephrines and catecholamines, and preoperative management. Results Two hundred seventy-one patients (52% women, median age 52.0 years) presented with 296 PHEOs. Discovery method was most often incidental finding on cross-section imaging (61%) rather than PHEO-related symptoms (27%) or mutation-based case detection testing (12%). Patients with incidentally discovered PHEOs were older than symptomatic and mutation-based case detection testing patients (median age 56.6 vs 43 vs 35 years, P < 0.0001). Mutation-based case detection PHEOs were smaller than those discovered due to symptoms (median size 29.0 vs 50.5 mm, P = 0.0027). Patients with PHEOs discovered due to symptoms had the highest median concentration of 24-hour urinary metanephrines and total plasma metanephrines (P < 0.0001). These patients required a higher cumulative phenoxybenzamine dose than patients with incidental or case detection PHEO (median 450 vs 375 vs 270 mg, P = 0.029). Conclusions PHEOs are primarily discovered due to incidental finding on cross-sectional imaging rather than PHEO-related symptoms. PHEOs discovered through mutation-based genetic case detection testing were smaller and required less α-adrenergic blockade preoperatively compared with PHEOs found due to symptoms, which supports routine case detection testing for patients genetically predisposed for PHEOs. Pheochromocytomas found by mutation-based case detection testing are less biochemically active and require less preoperative α-adrenergic blockade compared with those found due to symptoms.

Pheochromocytomas and sympathetic paraganglioma (PPGL) are rare chromaffin cell tumors originating in the adrenal medulla and sympathetic paraganglia, respectively, which share the capacity to synthesize and release catecholamines. The incidence of PPGL has increased in recent years. Surgical resection is the only curative treatment for PPGL. Management of patients with PPGL is complex and should be done by a specialized multidisciplinary team in centers with broad expertise. Surgical resection of a PPGL is a high-risk procedure for which optimal pretreatment with antihypertensive drugs is required in combination with state-of-the-art surgical procedures and anesthesiological techniques. In this article we discuss the underlying evidence and the pros and cons of presurgical medical preparation. Finally, the areas of uncertainty and controversies in this field are addressed.

High levels of catecholamines in pheochromocytoma (PHEO) are associated with risk of cardiovascular complications. In this study, we looked for potential differences in markers of oxidative stress – vitamin C, superoxide dismutase (SOD) and malondialdehyde (MDA) in PHEO before and after the operation. We studied 18 subjects with PHEO who were examined before and approximately one year after the successful tumor removal (free of disease). All subjects had elevated urinary epinephrine and/or norepinephrine levels before the operation. Vitamin C levels increased significantly after the operation from 61±27 to 77±20 μmol/l (P=0.02), and MDA decreased significantly after the tumor removal from 2.6±0.4 to 2.0±0.6 μmol/l (P=0.01). However, no changes were found in SOD activity before and after the operation. In conclusion, increased catecholamine production in PHEO is accompanied by decreased levels of vitamin C and increased levels of MDA which may indicate the activation of oxidative stress in PHEO. Successful operation was associated with lowering of oxidative stress by using both biomarkers. On the contrary, no changes in SOD activity before and after the tumor removal were noted.