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恶性嗜铬细胞瘤是起源于嗜铬组织的罕见恶性肿瘤,是否发生转移是判断其良恶性的标准。本例患者以多发肝、肺、骨转移就诊,查多项肿瘤标志物、胃镜、胸腹强化CT、PET-CT均难以确诊,行肝转移瘤穿刺活检,病理示:(肝右叶)恶性神经内分泌肿瘤,结合右侧肾上腺嗜铬细胞瘤切除术病史及免疫组织化学结果,考虑为恶性嗜铬细胞瘤肝、肺、骨转移。目前恶性嗜铬细胞瘤尚无标准治疗,文献报道使用CVD方案(环磷酰胺、长春新碱、达卡巴嗪)和靶向药物舒尼替尼能取得一定治疗效果。本例患者接受2个周期CVD化疗及1个疗程索坦治疗后,疾病仍缓慢进展。通过多学科讨论,认为131I-MIBG(131I-间位碘苄胍)可以作为该患者下一步治疗的选择。
Malignant pheochromocytoma is a rare malignant tumor originating from chromogranin. It is the standard to judge whether it is metastatic or not. The patients with multiple liver, lung and bone metastases treatment, check a number of tumor markers, endoscopy, chest and abdomen enhanced CT, PET-CT are difficult to diagnose, liver metastases biopsy, pathology showed: (right lobe) malignant Neuroendocrine tumors, combined with right adrenal pheochromocytoma resection history and immunohistochemical results, considered as malignant pheochromocytoma liver, lung and bone metastases. Currently no standard treatment of malignant pheochromocytoma, reported in the literature using the CVD regimen (cyclophosphamide, vincristine, dacarbazine) and targeted drug sunitinib can achieve some therapeutic effect. The patients underwent two cycles of CVD chemotherapy and one course of treatment with sultam, the disease is still slow progress. Through a multidisciplinary discussion, 131I-MIBG (131I-m-metronidazole) is considered as the next choice of treatment for this patient.