Porta Hepatis Paraganglioma: A Rare Case Report With Diagnostic Pitfalls

Paragangliomas are rare neuroendocrine tumors that infrequently localize to the porta hepatis, where they create significant diagnostic challenges by mimicking hepatocellular carcinoma or distinct mesenchymal neoplasms. We report the case of a 64‐year‐old woman presenting with vague abdominal pain and an enhancing porta hepatis mass compressing the inferior vena cava. An initial core needle biopsy, which suggested a benign mesenchymal tumor, underscores the potential for misdiagnosis in such atypical presentations. During surgery, the patient developed paroxysmal hypertension suggestive of occult catecholamine release. Subsequent histopathological examination revealed a classic zellballen pattern, with immunohistochemical positivity for Chromogranin A, Synaptophysin, and S100 definitively confirming a paraganglioma. The patient remains disease‐free at a 2‐year follow‐up. This case highlights the importance of considering paraganglioma in the differential diagnosis of porta hepatis masses and emphasizes the value of histopathology and immunohistochemistry for accurate diagnosis, particularly in patients who lack preoperative adrenergic symptoms.