We present a rare case of silent giant pheochromocytoma with leukemoid reaction that has not been reported before. The patient was a 61-year-old woman who complained of progressive weight loss for 3 months. Preoperatively the urine vanillylmandelic acid was 105.54 mg/24 hours. White blood cell count was 56.9 × 109/L. Abdominal ultrasound and computed tomography revealed a mass measuring 18 × 11 cm in the left adrenal area. The patient underwent adrenal gland neoplasm resection. Postoperative histopathologic evaluation confirmed the diagnosis of pheochromocytoma. The leukocyte dropped to normal gradually in 10 days after the operation. Giant pheochromocytoma with leukemoid reaction was very rare. Resection is the only curative treatment.
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