Metanephrine negative pheochromocytoma: a rare case report of dopamine-secreting tumor in an adolescent neurofibromatosis type 1 patient

Mi Seon Lee, Rosie Lee, Sook Hyun Park, Soon Hak Kwon, Jin Young Park, Sang Woo Lee, So Mi Lee, Jung Eun Moon

Research output: Contribution to journalArticlepeer-review

1 Scopus citations

Abstract

Pheochromocytoma (PCC) occurs in 4% of pediatric neurofibromatosis type 1 (NF1) patients and is characterized by epinephrine and norepinephrine secretion. Herein, we report the first case of dopamine-secreting PCC in a 13-year-old patient with NF1. A left adrenal mass was incidentally found on abdominal computed tomography (CT) during hypertension workup. Fractionated 24-hour urine metanephrine excretion was normal, but urine dopamine level was elevated. Focal123 I-metaiodobenzylguanidine uptake was observed on single-photon emission tomography/CT (SPECT/CT). Surgery was delayed due to small tumor size, vague symptoms, and increased dopamine level. After 6 months, focal significant uptake of the lesion on 6‐[18F]fluoro‐L‐3,4‐dihydroxyphenylalanine (18F-FDOPA) PET/CT increased and tumor size increased on abdominal CT. Laparoscopic resection was performed, and the mass was histologically confirmed as PCC. Currently, the vital signs of the patient are stable, urine dopamine level is normal, and there is no abnormal uptake of18F-FDOPA PET/CT. This study reports a rare case of dopamine-secreting PCC. A multidisciplinary approach and focused examination are needed in metanephrine-negative, high-risk PCC patients.

Original languageEnglish
Pages (from-to)302-307
Number of pages6
JournalAnnals of Pediatric Endocrinology and Metabolism
Volume28
Issue number4
DOIs
StatePublished - Dec 2023

Keywords

  • Dopamine
  • Neurofibromatosis type 1
  • Pediatrics
  • Pheochromocytoma

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