PDF(Pubmed)
Abstract：
For pheochromocytoma and paraganglioma (PPGL), the efficacy of percutaneous ablative therapies in achieving control of metastatic tumors measuring <3 cm had been demonstrated in only few reports, and intraoperative radiofrequency ablation (RFA) of locally invasive primary PPGLs has not been reported. We presented the case of a 31-year-old man who had a 9-cm functioning unresectable PPGL. He was treated with 13 cycles of cytotoxic chemotherapy without objective tumor response, according to the Response Evaluation Criteria in Solid Tumors (RECIST). Subsequently, magnetic resonance imaging revealed a 9.0 × 8.6 × 6.0-cm retroperitoneal mass that extended to the inferior portion of the inferior vena cava, the inferior mesenteric artery, and the infrarenal aorta. Biochemical evaluation demonstrated high level of plasma normetanephrine (20.2 nmol/L, normal range <0.9 nmol/L). Genetic investigation showed the germline pathogenic variant c.1591delC (p. Ser198Alafs*22) in the SDHB gene. I131-metaiodobenzylguanidine scintigraphy was negative and Ga68-dotatate PET-CT scan showed high tumor uptake without distant metastases. On open laparotomy, tumor debulking was not possible. Therefore, intraoperative RFA was performed by a highly experienced team of interventional radiologists. At 12 months after the RFA, the tumor volume decreased from 208 to 45 mL (78%), plasma normetanephrine decreased from 20.2 to 2.6 nmol/L (87%), and the doxazosin dose was reduced from 16 to 8 mg/day. To our best knowledge, this was the first report on intraoperative RFA that markedly reduced the size of a large primary unresectable PPGL, along with clinical and biochemical responses.
摘要：
对于嗜铬细胞瘤和副神经节瘤（PPGL），只有少数报道证明了经皮消融治疗在控制<3厘米的转移性肿瘤方面的疗效，术中射频消融（RFA）的局部有创原发性PPGL尚未报道。我们介绍了一个31岁男子的病例，该男子的PPGL功能为9厘米，无法切除。他接受了13个周期的细胞毒性化疗，没有客观的肿瘤反应，根据实体瘤疗效评估标准（RECIST）。随后，磁共振成像显示一个9.0×8.6×6.0厘米的腹膜后肿块，延伸到下腔静脉的下部，肠系膜下动脉,和肾下主动脉.生化评估显示血浆去甲肾上腺素水平较高（20.2nmol/L，正常范围<0.9nmol/L)。遗传调查显示种系致病变异c.1591delC（p。Ser198Alafs*22)中的SDHB基因。I131-间碘苄基胍闪烁显像阴性，Ga68-dotatatePET-CT扫描显示肿瘤摄取高，无远处转移。在开腹手术中，肿瘤减积是不可能的。因此,术中RFA由经验丰富的介入放射科医师团队进行.RFA后12个月，肿瘤体积从208毫升减少到45毫升（78％），血浆去甲肾上腺素从20.2降至2.6nmol/L（87％），多沙唑嗪的剂量从16毫克/天减少到8毫克/天。据我们所知，这是术中RFA的第一份报告，该报告显着减少了大型无法切除的原发性PPGL的大小，以及临床和生化反应。
