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- Title
Pheochromocytoma Characteristics and Behavior Differ Depending on Method of Discovery.
- Authors
Gruber, Lucinda M; Hartman, Robert P; Thompson, Geoffrey B; McKenzie, Travis J; Lyden, Melanie L; Dy, Benzon M; Young, William F Jr; Bancos, Irina; Young, William F
- Abstract
<bold>Context: </bold>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.<bold>Objective: </bold>To describe the characteristics and behavior of PHEOs discovered incidentally on imaging or through mutation-based genetic case detection testing.<bold>Design: </bold>Retrospective study.<bold>Setting: </bold>Referral center.<bold>Patients: </bold>Consecutive patients with pathology-confirmed PHEOs, treated from 2005 to 2016.<bold>Main Outcome Measure(s): </bold>Tumor size, plasma/urine fractionated metanephrines and catecholamines, and preoperative management.<bold>Results: </bold>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).<bold>Conclusions: </bold>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.
- Subjects
ADRENAL tumors; DIAGNOSIS; LONGITUDINAL method; GENETIC mutation; PHEOCHROMOCYTOMA; PROGNOSIS; GENETIC testing; CROSS-sectional method; RETROSPECTIVE studies; SEVERITY of illness index
- Publication
Journal of Clinical Endocrinology & Metabolism, 2018, pN.PAG
- ISSN
0021-972X
- Publication type
journal article
- DOI
10.1210/jc.2018-01707