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Pheochromocytoma and paraganglioma (PPGL) are neuroendocrine tumors with malignant potential. Although surgery is often curative, local recurrence, metastatic disease, or new tumors can occur even decades later; therefore, long-term follow-up for at least 10 years is generally recommended. We present a case of metachronous contralateral pheochromocytoma (PCC) diagnosed 62 years after the initial surgery-the longest postoperative interval reported through our literature search. We identified an old Japanese case report of the same patient that contained valuable clinical data and treatment details, providing a unique opportunity for longitudinal comparison of the initial and later clinical courses across 6 decades. Interestingly, the second tumor showed a shift from a mixed epinephrine-norepinephrine secretory profile to a predominantly norepinephrine profile, suggesting a potential change in the tumor characteristics over time. Although the patient declined surgery and genetic testing, a genetic etiology of PCC was suspected. This case highlights the importance of lifelong follow-up for all patients with PPGL, regardless of apparent cure.
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Kawabe et al. (2026) studied this question.
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