The present study aims to raise awareness about the necessity of a thorough differential diagnosis process when diagnosing paraganglioma as it is an extremely rare entity with a high malignant potential. A 64-year-old female patient was admitted to the hospital with symptoms of bowel obstruction and a palpable abdominal mass. Abdominal and pelvic computed tomography revealed a cystic heterogeneously enhanced mass. The tumor was surgically removed and then microscopically analysed. Microscopically, the mass was composed of nests of small polygonal and round cells with central vesicular nuclei, consistent with the diagnosis of mesenteric paraganglioma which was later confirmed by immunohistochemistry. The critical markers for paragangliomas, namely chromogranin A and S100, should be used as the first step in diagnosis, followed by other valuable immunohistochemical markers. A long-term follow-up is extremely important following the diagnosis of paraganglioma as all these tumors have a malignant potential.
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