Primary Retroperitoneal Paraganglioma Simulating a Pancreatic Mass: A Case Report and Review of the Literature

Abstract

Paragangliomas are extra-adrenal tumors of the autonomic nervous system and may be found within the skull base, neck, chest, and abdomen. When presenting within the abdominal cavity, they may arise as a primary retroperitoneal neoplasm and can mimic vascular malformations or other conditions related to specific retroperitoneal organs such as the pancreas, kidneys, or adrenals. Retroperitoneal paragangliomas are mostly benign with good prognosis; however, they can present with abdominal pain, palpable mass, or hypertensive episodes. Patients should be initially evaluated with catecholamine levels, followed by computed tomography or magnetic resonance imaging to locate the primary lesion. Surgical excision remains the mainstay of treatment, although advanced disease and proximity to vital organs can make excision difficult or impossible. This case report describes a patient who initially underwent work up for a suspected pancreatic head mass which was discovered to be a retroperitoneal paraganglioma by frozen section.