Department of General Surgery, Hamad General Hospital, Doha, Qatar.
Weill Cornell Medical College Qatar, Qatar Foundation, Doha, Qatar.
World J Surg Oncol. 2019 Jul 20;17(1):126. doi: 10.1186/s12957-019-1666-9.
The pure large cell type is a rare variant of primary neuroendocrine carcinoma of the gallbladder. Few reports have mentioned extended survival. Although a multimodal treatment has been described in the treatment of such rare disease, redo liver resection has not yet been mentioned.
A 67-year-old lady was found to have poorly differentiated, high grade, pure large cell neuroendocrine tumor of the gallbladder after cholecystectomy for gallstones. After the diagnosis, staging workup showed a lesion in segment IVB/V of the liver, and chromogranin was elevated (982 mcg/L). The patient underwent central inferior hepatectomy and wedge excision of a lesion in segment III (discovered intra-operatively), with hilar lymphadenectomy. Three months after the first liver resection, she developed a new liver lesion II/III and underwent left lateral liver resection. The patient remained disease-free for 4 months following the second liver resection but then developed recurrent liver disease and was started on chemotherapy. Further progression led to multi-organ failure and death at 26 months from initial diagnosis.
This is the first reported repeat liver resection in such a rare disease that has led to extended overall survival. We suggest that a group of selected patients with this rare malignancy, and liver-limited disease, may benefit from repeated liver resection.
单纯大细胞型是胆囊原发性神经内分泌癌的罕见变异型。鲜有报道提及延长生存时间。尽管已描述了治疗这种罕见疾病的多模态治疗方法,但尚未提及再次肝切除术。
一位 67 岁女性因胆囊结石行胆囊切除术,术后诊断为分化差、高级别、单纯大细胞神经内分泌胆囊肿瘤。确诊后,分期检查显示肝 IVB/V 段有病变,嗜铬粒蛋白升高(982mcg/L)。患者接受了中下肝切除术和 III 段楔形切除术(术中发现),并进行了肝门淋巴结清扫术。第一次肝切除术后 3 个月,患者出现新的 II/III 肝病变,行左外侧肝切除术。第二次肝切除后 4 个月,患者无疾病复发,但随后出现复发性肝疾病并开始化疗。进一步进展导致多器官衰竭,从最初诊断到死亡历时 26 个月。
这是首例报道的此类罕见疾病重复肝切除术,导致总生存时间延长。我们建议,对于一组具有这种罕见恶性肿瘤和肝局限性疾病的特定患者,重复肝切除术可能会获益。