Urology Case Reports (Jul 2017)

Multiple Recurrent Paraganglioma in a Pediatric Patient with Germline SDH-B Mutation

  • Aidan McGowan,
  • Julie Y. An,
  • Sally Tanakchi,
  • Mahir Maruf,
  • Akhil Muthigi,
  • Arvin George,
  • Daniel Su,
  • Maria J. Merino,
  • W. Marston Linehan,
  • Shawna L. Boyle,
  • Adam R. Metwalli

DOI
https://doi.org/10.1016/j.eucr.2017.03.017
Journal volume & issue
Vol. 13, no. C
pp. 107 – 109

Abstract

Read online

Magnetic Resonance Imaging (MRI) and fluoro-2-deoxy-d-glucose positron emission tomography (FDG-PET) are recognized approaches for locating paragangliomas. Recently, gallium-68 DOTA-octreotate (DOTATATE) scans have shown promise detecting neuroendocrine tumors missed by FDG-PET and MRI. 13-year-old male with SDH-B mutation presented with symptoms of paraganglioma and elevated catecholamines. MRI did not demonstrate the T2 hyper intense signal typical of paraganglioma and pheochromocytoma; FDG-PET scan did not reveal increased foci of uptake. DOTATATE scan revealed a signal consistent only with residual adrenal tissue. Resection of the right adrenal bed revealed paraganglioma. Following surgery, no further symptoms were reported and biochemical tests normalized.

Keywords