Background: Resection of a retrocaval paraganglioma is technically challenging due to limited tumor accessibility and proximity to the vena cava.
Case presentation: A large, malignant paraganglioma was found behind the retrohepatic segment of the inferior vena cava of a 60-year-old male. During resection of this rare paraganglioma, the left lateral lobe of the liver, a portion of the caudate lobe of the liver, and the gallbladder were also removed. Unfortunately, the patient died six months after surgery due to hepatic metastasis.
Conclusion: This case demonstrates that a partial hepatectomy may be necessary to improve tumor accessibility during resection of a retrocaval paraganglioma, particularly if the tumor is proximal to the vena cava. Furthermore, palliative treatments may help prevent tumor recurrence and metastasis of malignant paragangliomas.