Ann Pediatr Endocrinol Metab.  2023 Dec;28(4):302-307. 10.6065/apem.2244016.008.

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

• Lee MS
• Lee R
• Park SH
• Kwon SH
• Park JY
• Lee SW
• Lee SM
• Moon JE

• KMID: 2549324
• DOI: http://doi.org/10.6065/apem.2244016.008

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. Focal ¹²³I-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 of ¹⁸F-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.

Keyword

Pheochromocytoma; Pediatrics; Dopamine; Neurofibromatosis type 1

Figure

• 

  Fig. 1. (A) Abdominal computed tomography (CT) revealed a 1.2×1.4×1.7-cm-sized mass (white arrow) in the left adrenal gland on transverse image. (B) The (B1) fusion and (B2) 24-hour delayed image on 131I-MIBG SPECT/CT showed clear MIBG uptake (white arrow) in the nodule on the left adrenal gland. MIBG, metaiodobenzylguanidine; SPECT, single-photon emission tomography.

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  Fig. 2. (A) Abdominal computed tomography (CT) performed as follow-up showing size increase of the well-defined homogeneous nodular 1.8×1.9×2.0-cm-sized lesion in the left adrenal gland (white arrow) compared to that in the previous image. (B1, B2) 18F-FDOPA PET/CT showing focal significant FDOPA uptake in the left adrenal gland (white and black arrow). 18F-FDOPA, 18F(fluoro-L-3,4-dihydroxyphenylalanine): PET, positron emission tomography.

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  Fig. 3. Gross image of the dopamine-secreting pheochromocytoma. A well demarcated mass 2.3×2.0 cm (white arrow) in size located within the left adrenal gland.

Reference

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