A 31-year-old West-African female attended our emergency department presenting with palpitations, headache, fatigue and night sweats during the last 2 weeks. Clinical examination revealed tachycardia and a painful, palpable infraumbilical mass. Ultrasound examination of the abdomen showed a smoothly rounded soft-tissue mass with a diameter of 5 cm. On contrast-enhanced CT, a prevertebral mass with intense contrast enhancement was seen, located caudal to the aortic bifurcation. On PET-CT, there were no distant 18F-FDG-avid locoregional nodes or masses. A tumourectomy was successfully performed, during which manipulation of the retroperitoneal tumour triggered a sharp rise in blood pressure. Histological analysis confirmed the diagnosis of a paraganglioma. The clinical complaints of headache, paroxysmal palpitations and night sweats disappeared postoperatively. This case is a classic presentation of a paraganglioma occurring in the organs of Zuckerkandl, a collection of paraganglia. The diagnosis should be suspected in the presence of a heterogeneous, hypervascular mass in the retroperitoneum and typical clinical symptoms of hypertension, headache and palpitations. Treatment involves surgical resection, after accurate preoperative management. Genetic counselling is required, allowing a personal and genotype-based follow-up.
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