Case report reveals biochemical activity in silent pheochromocytoma, indicating need for careful management.
Background Asymptomatic pheochromocytomas are increasingly detected as incidentalomas. However, large tumors presenting with marked biochemical elevation but complete clinical silence (“biochemical-clinical dissociation”) are rare and prone to catastrophic mismanagement if mistaken for non-functional masses. Case presentation We report a case of a 43-year-old normotensive female presenting with a large (57×53 mm) incidental left adrenal mass. Abdominal imaging revealed a cystic-solid tumor with intratumoral hemorrhage. Despite the complete absence of sympathetic symptoms, biochemical evaluation showed markedly elevated plasma normetanephrine levels (1168.0 pg/ml), indicating significant secretory activity. The patient was successfully managed with preoperative alpha-blockade (doxazosin) and volume expansion, followed by an uneventful transperitoneal laparoscopic adrenalectomy. Histopathology confirmed a pheochromocytoma with loss of SDHB expression. Conclusion This suggests a multifactorial mechanism involving cystic sequestration and biochemical alterations associated with SDHB deficiency. Clinicians must recognize that biochemistry, not symptoms, dictates management. Mandatory preoperative α-blockade remains the cornerstone for preventing lethal intraoperative hemodynamic crises in these “silent” yet biochemically active tumors.
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Zhang et al. (2026) studied this question.
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