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Ann Pediatr Endocrinol Metab > Accepted Articles
DOI: https://doi.org/10.6065/apem.2244016.008    [Accepted] Published online June 28, 2022.
Metanephrine Negative Pheochromocytoma: A rare case report of dopamine-secreting tumor in an adolescent patient with NF1
Mi-Seon Lee1, Rosie Lee1, Sook-Hyun Park1, SoonHak Kwon1, Jin-Young Park2, Sang-Woo Lee3, So-Mi Lee4, Jung-Eun Moon1 
1Department of Pediatrics, School of Medicine, Kyungpook National University, Kyungpook National University Hospital, Daegu, Korea
2Department of Surgery, School of Medicine, Kyungpook National University, Kyungpook National University Hospital, Daegu, Korea
3Department of Nuclear Medicine, School of Medicine, Kyungpook National University, Kyungpook National University Hospital, Daegu, Korea
4Department of Radiology, School of Medicine, Kyungpook National University, Kyungpook National University Hospital, Daegu, Korea
Address for correspondence:  Jung-Eun Moon
Email: subuya@daum.net
Received: January 18, 2022   Revised: March 31, 2022   Accepted: April 8, 2022
Abstract
Pheochromocytoma (PCC) occurs in 4% of pediatric neurofibromatosis type 1 (NF1) patients and is mostly characterized by epinephrine and norepinephrine secretion. Herein, we report the first case of a dopamine-secreting PCC in a 13-year-old patient with NF1, in whom a left adrenal mass was incidentally found on abdominal computed tomography (CT) during hypertension workup. Fractionated 24-h urine metanephrine excretion was normal but urine dopamine level was elevated. On 123I-metaiodobenzylguanidine (MIBG) single photon emission tomography/CT (SPECT/CT), focal MIBG uptake was observed. Our multidisciplinary team determined that surgery would be difficult to perform because the tumor was small and the symptoms were vague, with only increased dopamine level. After six months, the tumor increased in size on abdominal CT, with focal significant uptake of the lesion on 6‐[18F]fluoro‐L‐3,4‐dihydroxyphenylalanine (18F-FDOPA) PET/CT. Laparoscopic resection was performed, and the mass was histologically confirmed as PCC. Currently, the vital signs of the patient are stable, urine dopamine levels are normal, and there is no abnormal uptake of 18F-FDOPA PET/CT. This study reports a case of a rare dopamine-secreting PCC. When metanephrine is negative in patients at high risk of PCC, focused examination and multidisciplinary approach are needed.
Keywords: Pheochromocytoma, pediatrics, dopamine, neurofibromatosis type 1
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