Introduction: Paragangliomas of the gallbladder are exceptionally rare. To date, only a few cases of this disease have been reported globally, and the majority were found incidentally during surgery. Although complete resection can achieve a curative effect, specific targeted drugs may have survival benefits for patients with potential recurrence and metastasis risks.
Case presentation: A 48-year-old woman was scheduled for anatomical central hepatectomy due to the discovery of a liver mass. Surprisingly, a gallbladder tumor accompanied by intrahepatic invasion was found rather than primary liver lesions during the operation. Postoperatively, the lesion was confirmed to be a paraganglioma originating from the gallbladder with intrahepatic invasion detectable on histopathology. After surgery, the patient was treated with a new targeted drug, surufatinib {200 mg, q.d. [quaque die (every day)]}, and no recurrence was observed during the regular follow-up.
Discussion: Gallbladder paraganglioma is rare and occult, and surgeons do not know it well, so it is easily misdiagnosed before surgery. Postoperative pathological examination is the gold standard for diagnosis.
Conclusion: Given that the tumor contained abundant blood sinuses, the early and continuous enhancement of dynamic enhanced CT scanning was its characteristic manifestation. We presented a case in which a primary gallbladder paraganglioma was identified accidentally in a patient who was misdiagnosed with a liver lesion before surgery. Based on our experience in this work, the en bloc resection technique in combination with surufatinib might have a survival benefit to patients at risk of potential recurrence or metastasis; however, further follow-up observations are needed.
Keywords: differential diagnosis; gallbladder paraganglioma; immunohistochemistry; neuroendocrine neoplasms (NENs); surufatinib.
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