Zhao Zi-Ming, Wang Jin, Ugwuowo Ugochukwu C, Wang Liming, Townsend Jeffrey P
1The Jackson Laboratory for Genomic Medicine, 10 Discovery Drive, Farmington, CT 06032 USA.
2Department of Biostatistics, Yale University, New Haven, CT 06511 USA.
BMC Clin Pathol. 2018 Mar 1;18:3. doi: 10.1186/s12907-018-0070-7. eCollection 2018.
Primary hepatic neuroendocrine carcinoma (PHNEC) is extremely rare. The diagnosis of PHNEC remains challenging-partly due to its rarity, and partly due to its lack of unique clinical features. Available treatment options for PHNEC include surgical resection of the liver tumor(s), radiotherapy, liver transplant, transcatheter arterial chemoembolization (TACE), and administration of somatostatin analogues.
We report two male PHNEC cases and discuss the diagnosis and treatment options. Both cases presented with abdominal pain; case two also presented with symptoms of jaundice. The initial diagnosis for both cases was poorly differentiated grade 3 small-cell neuroendocrine carcinoma, based on imaging characteristics and the pathology of liver biopsies. Final diagnoses of PHNEC were arrived at by ruling out non-hepatic origins. Case one presented with a large tumor in the right liver lobe, and the patient was treated with TACE. Case two presented with tumors in both liver lobes, invasions into the left branch of hepatic portal vein, and metastasis in the hepatic hilar lymph node. This patient was ineligible for TACE and was allergic to the somatostatin analogue octreotide. This limited treatment options to supportive therapies such as albumin supplementation for liver protection. Patient one and two died at 61 and 109 days, respectively, following initial hospital admission.
We diagnosed both cases with poorly differentiated grade 3 small-cell PHNEC through imaging characteristics, immunohistochemical staining of liver biopsies, and examinations to eliminate non-hepatic origins. Neither TACE nor liver protection appeared to significantly extend survival time of the two patients, suggesting these treatments may be inadequate to improve survival of patients with poorly differentiated grade 3 small-cell PHNEC. The prognosis of poorly differentiated grade 3 small-cell PHNEC is poor due to limited and ineffective treatment options.
原发性肝神经内分泌癌（PHNEC）极为罕见。PHNEC的诊断仍然具有挑战性，部分原因是其罕见性，部分原因是缺乏独特的临床特征。PHNEC的可用治疗选择包括肝肿瘤的手术切除、放疗、肝移植、经动脉化疗栓塞（TACE）以及生长抑素类似物的给药。
我们报告两例男性PHNEC病例，并讨论诊断和治疗选择。两例均表现为腹痛；病例二还出现黄疸症状。根据影像学特征和肝活检病理，两例的初步诊断均为低分化3级小细胞神经内分泌癌。通过排除非肝源性得出PHNEC的最终诊断。病例一右肝叶有一个大肿瘤，患者接受了TACE治疗。病例二双侧肝叶有肿瘤，侵犯肝门静脉左支，并在肝门淋巴结转移。该患者不符合TACE治疗条件，且对生长抑素类似物奥曲肽过敏。这使得治疗选择限于支持性治疗，如补充白蛋白以保护肝脏。患者一和患者二分别在初次入院后61天和109天死亡。
我们通过影像学特征、肝活检的免疫组化染色以及排除非肝源性的检查，将两例均诊断为低分化3级小细胞PHNEC。TACE和肝脏保护似乎均未显著延长这两名患者的生存时间，提示这些治疗可能不足以改善低分化3级小细胞PHNEC患者的生存。由于治疗选择有限且无效，低分化3级小细胞PHNEC的预后较差。
