Von Hippel-Lindau (vHL) with left adrenal pheochromocytoma

Case contributed by Dr Justin G Peacock

Presentation

Patient referred to Nuclear Medicine with congenital syndrome, abdominal pain, and prior cross-sectional imaging.

Patient Data

Age: 5 years-old
Gender: Male

Anterior and posterior whole body planar images from the I-123 MIBG scan demonstrate an oval focus of intense uptake overlying the left kidney (best seen on posterior views). Otherwise normal MIBG uptake pattern.

In pediatric patients with abdominal pain, the first line of imaging is US, which was performed in this case (images to follow), followed by MRI or possibly CT (images to follow). In this case, a suprarenal mass with typical cross-sectional features for a pheochromocytoma can be followed by MIBG to confirm the likely diagnosis and to assess for additional sites of disease.

Ultrasound of the left adrenal gland demonstrates a heterogeneously hyperechoic mass superior to the left kidney.

Coronal T1 and T2 images with and without contrast and fat saturation demonstrate a kidney bean-shaped, avidly enhancing mass superior to the left kidney, with mild mass effect on the upper pole. The enhancement pattern is mildly heterogeneous. The lesion is T2 hyperintense. The mass appears to originate from the lateral limb of the left adrenal gland.

Coronal CT images demonstrate an avidly enhancing mass superior to the left kidney, with small areas of heterogeneous enhancement. The mass appears to arise from the lateral limb of the left adrenal gland. There is mild mass effect on the upper pole of the left kidney.

Case Discussion

Key Learning Points:

  • I-123 MIBG is a norepinephrine analog that localizes to the pre-synaptic adrenergic neuron granules
  • I-123 MIBG is used in neuroblastoma, pheochromocytoma, and paraganglioma
  • von Hippel-Lindau (vHL) is a congenital syndrome characterized by mutation of the VHL tumor suppressor gene on chromosome 3
  • pheochromocytoma is a tumor of the adrenal gland that can occur in vHL and is characterized by
    • avid enhancement
    • T2 hyperintensity
    • variable Nuclear Medicine radiopharmaceutical avidity
      • I-123 MIBG
      • F-18 DOPA
      • Ga-68 DOTATATE
      • In-111 Octreotide 

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