Metachronous contralateral pheochromocytoma detected 62 years after initial surgery

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.