| Summary: | Pheochromocytomas are rare neuroendocrine tumors. Although predominantly occurring in the adrenal glands, these tumors can present anywhere along the sympathetic chain. Indeed, classical teaching states that 10% of pheochromocytomas are extra-adrenal and 10% are malignant. We report a case of a 61-year-old female who underwent presumptive cytoreductive nephrectomy and adrenalectomy for renal carcinoma but was instead found to have malignant pheochromocytoma. Proper identification, surgical extirpation, and follow-up are imperative for treatment. We review the classic and current literature regarding management of this uncommon tumor.
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