Endocrine practice : official journal of the American College of Endocrinology and the American Association of Clinical Endocrinologists · 2026
Composite Pheochromocytomas: More Common Than Expected and Associated With Distinct Clinical Profile.
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Zawadzka Karolina, Rzepka Ewelina, Calissendorff Jan, Ulatowska-Białas Magdalena, Juhlin C Christofer, Hubalewska-Dydejczyk Alicja, Falhammar Henrik
Аннотация
OBJECTIVE: Composite pheochromocytomas, defined by the coexistence of pheochromocytoma and neural crest-derived elements and their clinical course remain poorly defined. The aim of this study was to assess the clinical characteristics and outcomes of composite pheochromocytomas compared with typical pheochromocytomas.
METHODS: We retrospectively analyzed 190 patients with pheochromocytoma. Clinical outcomes were compared between composite and typical pheochromocytomas.
RESULTS: The study included 190 patients: 28 (14.9%) had composite tumors and 160 had typical pheochromocytoma; 2 additional patients with mixed corticomedullary tumors (MCMTs) were analyzed separately. Patients with composite tumors were older (median 67 vs 56 years, P = .015) compared with patients with typical pheochromocytoma. In multivariable analysis, the composite phenotype was associated with preoperative systolic hypertension (odds ratio (OR)-2.47, 95% confidence interval [CI] 1.00-6.06, P = .049) and postoperative elevation of blood pressure, both systolic (OR-3.24, 95%CI 1.30-8.11, P = .012) and diastolic (OR-4.66, 95%CI 1.79-12.11, P = .002). On unenhanced computed tomography, attenuation of composite pheochromocytomas was lower (median 30 vs 35 Hounsfield units, P = .01), and these were less often detected as adrenal incidentalomas. Laparoscopic adrenalectomy was performed less frequently in composite pheochromocytomas (67.9% vs 87.5%, P = .02). Composite pheochromocytomas had a higher risk of Ki-67 expression in the 3% to 5% range (OR-28.68, 95%CI 1.49-552.79, P = .026). Tumor size, recurrence and metastasis rates, and mortality were comparable to typical pheochromocytoma.
CONCLUSIONS: Composite pheochromocytomas were associated with older age, lower computed tomography attenuation, distinct perioperative features, and a higher burden of hypertension. These findings suggest that this subgroup may require closer postoperative monitoring. Although Ki-67 was higher in composite pheochromocytomas, its prognostic significance remains uncertain.
METHODS: We retrospectively analyzed 190 patients with pheochromocytoma. Clinical outcomes were compared between composite and typical pheochromocytomas.
RESULTS: The study included 190 patients: 28 (14.9%) had composite tumors and 160 had typical pheochromocytoma; 2 additional patients with mixed corticomedullary tumors (MCMTs) were analyzed separately. Patients with composite tumors were older (median 67 vs 56 years, P = .015) compared with patients with typical pheochromocytoma. In multivariable analysis, the composite phenotype was associated with preoperative systolic hypertension (odds ratio (OR)-2.47, 95% confidence interval [CI] 1.00-6.06, P = .049) and postoperative elevation of blood pressure, both systolic (OR-3.24, 95%CI 1.30-8.11, P = .012) and diastolic (OR-4.66, 95%CI 1.79-12.11, P = .002). On unenhanced computed tomography, attenuation of composite pheochromocytomas was lower (median 30 vs 35 Hounsfield units, P = .01), and these were less often detected as adrenal incidentalomas. Laparoscopic adrenalectomy was performed less frequently in composite pheochromocytomas (67.9% vs 87.5%, P = .02). Composite pheochromocytomas had a higher risk of Ki-67 expression in the 3% to 5% range (OR-28.68, 95%CI 1.49-552.79, P = .026). Tumor size, recurrence and metastasis rates, and mortality were comparable to typical pheochromocytoma.
CONCLUSIONS: Composite pheochromocytomas were associated with older age, lower computed tomography attenuation, distinct perioperative features, and a higher burden of hypertension. These findings suggest that this subgroup may require closer postoperative monitoring. Although Ki-67 was higher in composite pheochromocytomas, its prognostic significance remains uncertain.