ObjectiveTo characterize large adrenal tumors (≥4 cm in diameter) and to identify features associated with malignancy.Patients and MethodsWe investigated the clinical, biochemical, and imaging characteristics in a large retrospective single-center cohort of patients with adrenal tumors of 4 cm or more in diameter during the period of January 1, 2000, through December 31, 2014.ResultsOf 4085 patients with adrenal tumors, 705 (17%) had adrenal masses measuring 4 cm or more in diameter; of these, 373 (53%) were women, with a median age of 59 years (range, 18-91 years) and median tumor size of 5.2 cm (range, 4.0-24.4 cm). Underlying diagnoses were adrenocortical adenomas (n=216 [31%]), pheochromocytomas (n=158 [22%]), other benign adrenal tumors (n=116 [16%]), adrenocortical carcinomas (n=88 [13%]), and other malignant tumors (n=127 [18%]). Compared with benign tumors, malignant tumors were less frequently diagnosed incidentally (45.5% vs 86.7%), were larger (7 cm [range, 4-24.4 cm] vs 5 cm [range, 4-20 cm]), and had higher unenhanced computed tomographic (CT) attenuation (34.5 Hounsfield units [HU] [range, 14.1-75.5 HU] vs 11.5 HU [range, −110 to 71.3 HU]; P<.001). On multivariate analysis, older age at diagnosis, male sex, nonincidental mode of discovery, larger tumor size, and higher unenhanced CT attenuation were all found to be statistically significant predictors of malignancy.ConclusionThe prevalence of malignancy in patients with adrenal tumors of 4 cm or more in diameter was 31%. Older age, male sex, nonincidental mode of discovery, larger tumor size, and higher unenhanced CT attenuation were associated with an increased risk for malignancy. Clinical context should guide management in patients with adrenal tumors of 4 cm or more in diameter.
Metastatic pheochromocytoma and paraganglioma (PPGL) are incurable neuroendocrine tumors. The goals of treatment include palliating symptoms and reducing tumor burden. Little is known about the use of radiofrequency ablation (RFA), cryoablation (CRYO), and percutaneous ethanol injection (PEI) to treat metastatic PPGL. We performed a retrospective study of patients age 17 years and older with metastatic PPGL who were treated with ablative therapy at Mayo Clinic, USA, between June 14, 1999 and November 14, 2017. Our outcomes measures were radiographic response, procedure-related complications, and symptomatic improvement. Thirty-one patients with metastatic PPGL had 123 lesions treated during 42 RFA, 23 CRYO, and 4 PEI procedures. The median duration of follow-up was 60 months (range, 0–163 months) for non-deceased patients. Radiographic local control was achieved in 69/80 (86%) lesions. Improvement in metastasis-related pain or symptoms of catecholamine excess was achieved in 12/13 (92%) procedures. Thirty-three (67%) procedures had no known complications. Clavien-Dindo Grade I, II, IV, and V complications occurred after 7 (14%), 7 (14%), 1 (2%), and 1 (2%) of the procedures, respectively. In patients with metastatic PPGL, ablative therapy can effectively achieve local control and palliate symptoms.
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.
BackgroundSurgical manipulation of pheochromocytomas and paragangliomas (PPGLs) may induce large hemodynamic oscillations due to catecholamine release. Little is known regarding hemodynamic instability during percutaneous ablation of PPGLs. We examined intraprocedural hemodynamic variability and postoperative complications related to percutaneous ablation of extra-adrenal metastases of PPGL.MethodsFrom institutional PPGL registry we identified patients undergoing ablation of extra-adrenal PPGL metastases from January 1, 2000, through December 31, 2016. We reviewed medical records for clinical characteristics and hospital outcomes. Tumors were categorized as functional or nonfunctional based on preprocedural fractionated catecholamine and metanephrine profiles.ResultsTwenty-one patients (14 female [67%]) underwent 38 ablations. Twenty-four ablations were performed in patients with functional metastatic lesions, and 14 were in nonfunctional lesions. Intraprocedural use of potent vasodilators for hypertension was higher for patients with functional tumors (P = 0.02); use of vasopressors for hypotension was similar for functional and nonfunctional tumors (P = 0.74). Mean (±SD) intraprocedural blood pressure range (maximum–minimum blood pressure) during 38 procedures was greater for functional than nonfunctional tumors [systolic: 106 (±48) vs 64 (±30) mm Hg, P = 0.005; diastolic: 58 (±22) vs 35 (±14) mm Hg, P = 0.002; mean arterial: 84 (±43) vs 47 (±29) mm Hg, P = 0.007]. Complications included 5 unplanned intensive care unit admissions (3 for precautionary monitoring, 1 for recalcitrant hypotension, and 1 for hypertensive crisis), 1 case of postoperative bleeding, and 1 death.ConclusionsSubstantial hemodynamic instability may develop during ablation of functional and nonfunctional PPGL metastases. When anesthesia is provided for ablation of metastatic PPGLs in radiology suites, preparation for hemodynamic management should match standards used for surgical resection.
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