Purpose or Case Report: Patients with Von Hippel Lindau are predisposed to develop neuroendocrine tumors (NET) such as pheochromocytoma. These tumors are known to be somatostatin receptor positive and therefore can be imaged with specific radiotracers that target these receptors like ¹¹¹In-octreotide and ⁶⁸Ga-Dotatate. In addition to NET’s, 80% of these patients will also develop hemangioblastomas in the brain and spinal cord which have been shown to harbor somatostatin receptor subtypes including SSTR-2a, one of the target receptors for DOTATATE. We present a case of a teenager with suspected NET in the adrenal gland who underwent ⁶⁸Ga-DOTATATE PET/CT. The PET confirmed uptake in the adrenal lesion, but also highlighted a focus of uptake in the spine. Correlative MRI of the spine demonstrates classic imaging features of hemangioblastoma. This finding has not previously been described in the published literature in children.
This case report will discuss the mechanism and utility of ⁶⁸Ga-Dotatate PET/CT and emphasize the manifestations of Von Hippel Lindau disease. We will review the normal pattern of Dotatate uptake and the significance of somatostatin receptors in neoplasms in the VHL patient population.
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