Chen Jennifer,  Milla Sarah,  Alazraki Adina

Final Pr. ID: Poster #: CR-031

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. Read More

Authors:  Chen Jennifer , Milla Sarah , Alazraki Adina

Keywords:  hemangioblastoma, dotatate, von hippel lindau

Mcelroy Kevin,  Czachowski Michael,  Trout Andrew,  Seghers Victor,  States Lisa,  Ahmed Safia,  Laack Nadia,  Binkovitz Larry

Final Pr. ID: Poster #: SCI-032

Though pediatric use of both ⁶⁸Gallium Dotatate and ¹⁷⁷Lutetium is off label, the advantages they offer over FDA approved agents has led to their preliminary use in a variety of pediatric clinical scenarios. The purpose of this poster is to present the range of potential diagnostic and therapeutic applications of ⁶⁸Gallium Dotatate in children. Read More

Authors:  Mcelroy Kevin , Czachowski Michael , Trout Andrew , Seghers Victor , States Lisa , Ahmed Safia , Laack Nadia , Binkovitz Larry

Keywords:  Dotatate, Neuroendocrine tumor, Lutathera

Salman Rida,  Sher Andrew

Final Pr. ID: Poster #: CR-042

14-year-old female presents with two-day history of fever and cough. She also reports two months history of fatigue, shortness of breath, palpitations and night sweat. Physical exam was only remarkable for systolic ejection murmur and hypertension. Relevant diagnostic testing: Microcytic anemia. LDH, uric acid AFP and HCG were normal. Negative HIV, Quantiferon and fungal culture. Negative double stranded DNA. Normal chromogranin A. Elevated plasma and urine normetanephrine levels.
Chest radiograph shows a well-defined right hilar mass with hilum overlay sign excluding the possibility of a middle mediastinal mass. CT chest with contrast demonstrates a necrotic right mediastinal mass. Gallium-68 Dotatate PET/CT shows avid radiotracer uptake in the right mediastinal mass compatible with biopsy-proven paraganglioma with no additional radiotracer-avid lesions elsewhere in the body. Hereditary paraganglioma/ pheochromocytoma panel analysis of a blood sample revealed a heterozygous pathogenic frameshift pathogenic variant in SDHC gene.
Paragangliomas are extra-adrenal pheochromocytomas and can be isolated or associated with hereditary diseases such as von Hippel-Lindau and succinate dehydrogenase (SDH) mutations. These tumors account for 1% of pediatric secondary hypertension.
Functional imaging, including 123I-MIBG, 111In-octreotide SPECT, 68Ga Dotatate and 18F-FDG PET, can be used to confirm the diagnosis, evaluate metastatic spread and conduct disease surveillance.
Ga-68 [DOTA-0-Tyr-3] octreotate (DOTATATE), with high affinity for somatostatin receptors (SSTR2) which are expressed by neuroendocrine tumors, is able to localize the tumor by the presence of focal increased radiotracer uptake in areas that are not typical for normal distribution such as pituitary, thyroid, adrenals, liver, spleen, uncinate process of pancreas, kidneys, and bladder. Read More

Authors:  Salman Rida , Sher Andrew

Keywords:  Ga-68 DOTATATE, Paraganglioma