We report a case of pheochromocytoma crisis with negative MIBG scintigraphy. A 48-year-old man was admitted for hypertension crisis. Computed tomographic scan revealed a 60 mm right adrenal mass. The MIBG scintigraphy was negative, but we diagnosed pheochromocytoma crisis because of high blood catecholamine levels. We successfully managed the patient's hemodynamics through medical treatment and the patient was able to recover from the crisis. After appropriate preparation, the tumor was removed via laparotomy. SDHB mutation, related to the negative MIBG scintigraphy, was also denied pathologically by immunostaining procedures. Histopathologically, it showed a wide range of necrotic images. So the cause of the crisis was thought to be the release of a large amount of catecholamine from necrotic tumor cells. It was thought that scintigraphy became negative due to the decreased MIBG uptake of tumor cells with extensive necrosis.
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