A 46-year-old female presented to the emergency department with neck pain and multiple lytic lesions in the cervical spine include complete loss of height of C3 on CT imaging. Presentation was on the background of hypertension, constipation, vomiting and weight loss. Obstructive kidney injury was noted at presentation related to a large para-aortic nodal mass with creatinine 259umol/L and eGFR 19ml/min/1.73m². Further imaging revealed innumerable bony lesions in addition to chest wall and pulmonary disease.

Operative management for C3 spine lesion and ureteric obstruction was complicated by severe hypertension requiring numerous antihypertensive agents and intensive care admission. Plasma metanephrines were markedly elevated with Normetadrenaline 108 206pmol/L, metadrenaline 251pmol/L and 3-methoxytyramine 1178pmol/L. FDG PET revealed intensely avid skeletal metastasis and nodal disease above and below the diaphragm. Histology was consistent with malignant paraganglioma and subsequent genetic panel confirmed SDHB mutation. Alpha followed by beta blockade was commenced and clinical course was complicated by recurrent intestinal pseudo-obstruction (Ogilvie syndrome).

This case presentation will focus on the diagnosis and management of Ogilvie syndrome in metastatic paraganglioma (PGL) caused by effects of excess catecholamines on intestinal motility. In addition, molecular imaging modalities for SDHB-related PGL and how they guide treatment with peptide receptor radionuclide therapy (PRRT) will be discussed. At the time of diagnosis 34% of paragangliomas are metastatic with five-year overall survival ranging from 40-77%. PRRT is emerging as a promising treatment option in the setting of non-operative metastatic disease. Finally, surveillance of affected family members will be discussed to avoid a similar outcome in other mutation carriers.