原发性肝脏神经内分泌癌骨、脑转移患者MDT诊治报道

MDT diagnosis and treatment of primary hepatic neuroendocrine carcinoma with bone and brain metastases

原发性肝脏神经内分泌癌属于罕见肿瘤。本例患者发病后曾行肝脏肿瘤切除术和右侧第10肋骨肿瘤切除术,经术后病理组织学和免疫组织化学检查确诊。术后1月出现多发脑转移,经第1次多学科讨论,先行替尼泊苷联合顺铂方案化疗5周期,辅以现代化中药制剂榄香烯乳注射液治疗,再序贯行头颅伽玛刀放疗,颅内病灶得以有效控制,神经系统症状消失,客观疗效达到部分缓解。后颅内病灶进展,采用多种细胞毒药物、分子靶向药物治疗,颅内病灶稳定较长时间。后颅内病灶再次进展,进行第2次多学科讨论,给予全脑放疗,再次获得控制(部分缓解)。目前患者仍然带瘤生存,生活质量良好,从诊断至今生存期已达37月,充分体现多学科诊治团队能够优化罕见肿瘤的治疗方案,有计划、合理的综合治疗对于改善患者的生活质量,延长生存起关键作用。

Primary hepatic neuroendocrine carcinoma is a rare tumor. In our case,the patient received hepatic tumor and right 10 th rib tumor resection,and was diagnosed as primary hepatic neuroendocrine carcinoma with rib metastasis by postoperative pathology and immunohistochemistry. However,one month after surgery,multiple brain metastases appeared.According to multidisciplinary team（ MDT） discussion,the patient was treated with teniposide and cisplatin chemotherapy for 5 cycles,supplemented with Chinese medicine elemene emulsion,and sequential head gamma knife therapy. With these remedies,the intracranial lesions were effectively controlled and the neurological symptoms were significantly alleviated. The patient was evaluated as partial response. After a while,her intracranial lesions progressed. Multiple cytotoxic drugs and molecular targeted drug therapy were taken and the intracranial lesions maintained stable for a relatively long period of time.Afterwards,the intracranial lesion progressed again. A second multidisciplinary discussion was held and agreed on whole brain radiotherapy. After the therapy,the patient once again achieved partial remission. Up to now,it has been 37 months since diagnosis. The patient is still alive with tumor and her life quality is satisfactory. Our case effectively demonstrated the advantages of MDT in optimizing treatment scheme,improving the quality of life and prolonging survival period for rare tumor.