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Pheochromocytoma (PHEO) is a rare neuroendocrine that tumor originated from the adrenal medulla that secrets catecholamines. Tumors from extra-adrenal chromaffin tissues are called extra-adrenal PHEO or paraganglioma (PGL). To our knowledge, adrenal PHEO and subclinical PGL with inferior vena cava (IVC) invasion had been sporadically reported, while functional PGL with IVC tumor thrombus has not been publicly reported yet. Perioperative management of those diseases is less well established because of their multidisciplinary nature and rarity. We herein present a case of primary malignant PGL with IVC invasion. A 16-year-old female patient with a history of severe paroxysmal hypertension was admitted to Peking Union Medical College Hospital on suspicion of retroperitoneal mass. In-house diagnostic work-up revealed a malignant PGL with IVC invasion, inferior mesenteric artery encasement and, aorta engagement. Multi-disciplinary discussions were held and careful preoperative preparation plans were made. After everything was ready, the functional PGL and tumor thrombus were completely resected, then a reconstruction of IVC was performed. The patient was discharged on postoperative day 14 and all her clinical symptoms disappeared afterward. No evidence of tumor residual or metastasis was found in the subsequent six months of follow-up. Gene tests were made for her and her family. Albeit its rarity, functional PGL with IVC invasion is not unresectable, a multi-disciplinary task force should be established to settle down every detail. We recommended 3-dimensional imaging reconstruction for gaining a better anatomic understanding. Literature reviews showed that complete resection is the premise of a good prognosis. In particular cases, complementary or alternative therapy like chemotherapy and 131 I-metaiodobenzylguanidine might help, family hereditary genetic tests are advised as well.

Functional paraganglioma with tumor thrombus in the inferior vena cava, first case report