A paraganglioma is a rare neuroendocrine neoplasm that may develop at various body sites (including the head, neck, thorax and abdomen). About 97% are benign and cured by surgical removal; the remaining 3% are malignant because they are able to produce distant metastases. 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. We report a rare case of retroperitoneal mass presented to be a non-functioning Paraganglioma.
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