Purpose or Case Report: Hereditary paraganglioma-pheochromocytoma syndromes (PGL/PCC) are marked by the presence of multiple and/or multifocal neoplasms of neuroendocrine origin, namely paragangliomas and pheochromocytomas. Though considered an overarching familial cancer syndrome, PGL/PCC can be further characterized based on cellular origin, secretory status, and predisposing genetic mutation, comprising at least 6 distinct subtypes of the disease. Because of the unique metabolic features of neuroendocrine neoplasms, distribution of disease in patients with PGL/PCC can often be precisely evaluated via targeted nuclear medicine radiotracers. However, the true extent and burden of disease involvement may be artificially suppressed owing to the variability in tumor differentiation and subsequent metabolic function. We present the case of a 15-year-old with hereditary paraganglioma-pheochromocytoma syndrome who was evaluated with several distinct nuclear medicine radiotracers – DOTATATE, FDG, and MIBG – with synergistic findings. Initial evaluation with DOTATATE PET/CT demonstrated multiple somatostatin-receptor positive lesions in the neck, chest, and abdomen. Further assessment with MIBG SPECT/CT highlighted the previously seen, dominant abdominal mass, though otherwise revealed less abnormal foci of disease than that seen on preceding DOTATATE study. Finally, whole body FDG PET/MRI was utilized, revealing intense gastric avidity corresponding to gastrointestinal stromal tumor, with milder uptake at the other sites of previously characterized malignancy. This case emphasizes the underlying heterogeneity of tumors which may arise in patients with PGL/PCC and lends credence to the value of multi-tracer imaging for true disease staging purposes.
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