Retroperitoneal paraganglioma presenting as acute pancreatitis

Abstract

Paragangliomas are rare neuroendocrine tumors that arise from the chromaffin cells of the
autonomic nervous system paraganglia. The estimated incidence is 0.8 per 100,000 people. The
initial manifestation of functioning paragangliomas is hypertension in 90% of cases. We present a rare case of a 55-year-old male patient who was previously asymptomatic and presented with clinical criteria consistent with acute pancreatitis, with no evidence of any other etiology. The imaging tests detected a para-aortic retroperitoneal mass displacing the distal pancreas. During open surgery, a tumor compromising the left adrenal gland was removed. During the tumor manipulation, the patient exhibited a significant increase in blood pressure, which subsided after the tumor was resected.
Histopathological examination confirmed a paraganglioma with a mutation in the SDHB gene. After a two-year period of clinical observation, there was no evidence of local or metastatic tumor recurrence.