Nakatake Richi, Ishizaki Morihiko, Matui Kosuke, Yanagimoto Hiroaki, Inoue Kentaro, Kaibori Masaki, Kawaguchi Yusai, Kon Masanori
Department of Surgery, Kansai Medical University, 2-5-1 Shinmachi, Hirakata, Osaka, 573-1010, Japan.
Surg Case Rep. 2017 Sep 11;3(1):102. doi: 10.1186/s40792-017-0378-z.
Primary hepatic neuroendocrine carcinomas are extremely rare. Because of the rarity of PHNEC, its clinical features and treatment outcomes are not well understood. A proper diagnosis and the correct therapeutic approach therefore remain clinically challenging.
A 67-year-old man was admitted to our department because of a liver tumor. Computed tomography revealed a single liver tumor 50 mm in diameter and located in the S3 region. Biopsy and imaging findings resulted in a diagnosis of primary hepatic neuroendocrine carcinoma. Left lateral segmentectomy was performed. Immunohistochemically, the tumor cells were positive for synaptophysin, chromogranin A, and CD56. Ki-67 was positive in > 90% of the tumor cells. The final diagnosis was primary hepatic neuroendocrine carcinoma. The patient suffered two episodes of lymph node recurrence. Nonetheless, the tumor was excised to prolong survival. Thus, after lymphadenectomy, he received adjuvant chemotherapy for 6 months. Two years after surgery, the patient remains alive and in good general condition.
In most cases, primary hepatic neuroendocrine carcinoma, while extremely rare, has a poor prognosis. At present, surgical resection is a priority for curative treatment, but in patients with recurrence, combined therapies are recommended.
原发性肝神经内分泌癌极为罕见。由于原发性肝神经内分泌癌罕见，其临床特征和治疗结果尚不明确。因此，正确诊断和正确的治疗方法在临床上仍然具有挑战性。
一名67岁男性因肝脏肿瘤入住我科。计算机断层扫描显示肝脏有一个直径50毫米的单发肿瘤，位于S3区。活检和影像学检查结果诊断为原发性肝神经内分泌癌。进行了左外侧叶切除术。免疫组织化学检查显示，肿瘤细胞突触素、嗜铬粒蛋白A和CD56呈阳性。Ki-67在>90%的肿瘤细胞中呈阳性。最终诊断为原发性肝神经内分泌癌。该患者出现了两次淋巴结复发。尽管如此，为延长生存期，仍切除了肿瘤。因此，在淋巴结切除术后，他接受了6个月的辅助化疗。手术后两年，患者仍然存活，一般状况良好。
在大多数情况下，原发性肝神经内分泌癌虽然极为罕见，但预后较差。目前，手术切除是根治性治疗的首选，但对于复发患者，建议采用联合治疗。
