Sugino Teruaki, Ando Ryosuke, Unno Rei, Iida Keitaro, Naiki Taku, Hamamoto Shuzo, Mizuno Kentaro, Okada Atsushi, Umemoto Yukihiro, Kawai Noriyasu, Tozawa Keiichi, Hayashi Yutaro, Inaki Anri, Kayano Daiki, Kinuya Seigo, Yasui Takahiro
Department of Nephro-urology, Nagoya City University Graduate School of Medical Sciences, 1, Kawasumi, Mizuho-cho, Mizuho-ku, Nagoya, 467-8601, Japan.
Department of Nuclear Medicine, Faculty of Medicine, Institute of Medical, Pharmaceutical and Health Sciences, Kanazawa University, Kanazawa, Japan.
BMC Res Notes. 2017 Dec 19;10(1):750. doi: 10.1186/s13104-017-3095-6.
Pheochromocytomas are rare neuroendocrine tumors, with a malignancy frequency of approximately 10%. The treatment of malignant pheochromocytoma is palliative, and the traditional management strategy has limited efficacy. Furthermore, no clear criteria exist for the treatment of metastatic pheochromocytoma, especially for unresectable lesions. We report a case of complete remission of metastatic pheochromocytoma in I-metaiodobenzylguanidine (MIBG) scintigraphy after a single session of I-MIBG therapy.
A 61-year-old woman had a right adrenal grand tumor and lymph node metastasis on the hilum of the right kidney, both of which incorporated MIBG. After surgery, immunostaining of a tumor specimen showed expression of the tumor makers chromogranin and synaptophysin. One year postoperatively, abdominal computed tomography revealed a local recurrence and retroperitoneal lymph node swelling. The local recurrence was positive for MIBG uptake, whereas the swollen retroperitoneal lymph nodes were negative. She underwent surgery again, but the local recurrence was unresectable because of rigid adhesion to the surrounding tissue. Immunostaining of an intraoperatively extracted swollen retroperitoneal lymph node showed expression of tumor markers. The patient then underwent a single session of I-MIBG therapy (7.4 GBq, 200 mCi), after which the residual lesions no longer incorporated MIBG, and a complete response in I- metaiodobenzylguanidine (MIBG) scintigraphy was achieved. The I-MIBG treatment was repeated 6 months later. None of the lesions were positive for MIBG uptake.
I-MIBG therapy efficaciously treats unresectable lesions that are positive for MIBG uptake.
嗜铬细胞瘤是罕见的神经内分泌肿瘤，恶性频率约为10%。恶性嗜铬细胞瘤的治疗是姑息性的，传统管理策略疗效有限。此外，对于转移性嗜铬细胞瘤的治疗，尤其是不可切除病变，尚无明确标准。我们报告1例转移性嗜铬细胞瘤经单次131I-间碘苄胍（MIBG）治疗后在MIBG闪烁扫描中完全缓解的病例。
一名61岁女性右肾上腺有巨大肿瘤，右肾门有淋巴结转移，二者均摄取MIBG。手术后，肿瘤标本免疫染色显示肿瘤标志物嗜铬粒蛋白和突触素表达。术后1年，腹部计算机断层扫描显示局部复发和腹膜后淋巴结肿大。局部复发处MIBG摄取呈阳性，而肿大的腹膜后淋巴结为阴性。她再次接受手术，但由于与周围组织紧密粘连，局部复发灶无法切除。术中取出的肿大腹膜后淋巴结免疫染色显示肿瘤标志物表达。然后患者接受了单次131I-MIBG治疗（7.4GBq，200mCi），之后残留病变不再摄取MIBG，并在131I-间碘苄胍（MIBG）闪烁扫描中获得完全缓解。6个月后重复进行131I-MIBG治疗。所有病变MIBG摄取均为阴性。
131I-MIBG治疗对摄取MIBG阳性的不可切除病变有效。
