原发性肝脏神经内分泌癌2例报告并文献复习。

Q2 Medicine
BMC Clinical Pathology Pub Date : 2018-03-01 eCollection Date: 2018-01-01 DOI:10.1186/s12907-018-0070-7
Zi-Ming Zhao, Jin Wang, Ugochukwu C Ugwuowo, Liming Wang, Jeffrey P Townsend
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引用次数: 22

摘要

背景: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,部分原因是其缺乏独特的临床特征。PHNEC可用的治疗方案包括肝肿瘤手术切除、放疗、肝移植、经导管动脉化疗栓塞(TACE)和给予生长抑素类似物。病例介绍:我们报告两例男性PHNEC病例,并讨论诊断和治疗方案。两例均表现为腹痛;病例2也有黄疸症状。根据影像学特征和肝活检病理,两例患者的初步诊断均为低分化3级小细胞神经内分泌癌。PHNEC的最终诊断是通过排除非肝脏来源得出的。病例1为右肝叶大肿瘤,经TACE治疗。病例二表现为双肝叶肿瘤,侵犯肝门静脉左支,肝门淋巴结转移。该患者不适合接受TACE治疗,并且对生长抑素类似物奥曲肽过敏。这限制了支持治疗的选择,如补充白蛋白以保护肝脏。患者1和患者2分别在初次入院后61天和109天死亡。结论:我们通过影像学特征、肝活检免疫组化染色和排除非肝脏来源的检查,诊断这两例为低分化3级小细胞PHNEC。TACE和肝脏保护均未显着延长两例患者的生存时间,提示这些治疗可能不足以提高低分化3级小细胞PHNEC患者的生存。低分化3级小细胞PHNEC由于治疗方案有限且无效,预后较差。
本文章由计算机程序翻译,如有差异,请以英文原文为准。

Primary hepatic neuroendocrine carcinoma: report of two cases and literature review.

Primary hepatic neuroendocrine carcinoma: report of two cases and literature review.

Primary hepatic neuroendocrine carcinoma: report of two cases and literature review.

Primary hepatic neuroendocrine carcinoma: report of two cases and literature review.

Background: 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.

Case presentation: 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.

Conclusions: 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.

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来源期刊
BMC Clinical Pathology
BMC Clinical Pathology Medicine-Pathology and Forensic Medicine
CiteScore
3.30
自引率
0.00%
发文量
0
期刊介绍: BMC Clinical Pathology is an open access journal publishing original peer-reviewed research articles in all aspects of histopathology, haematology, clinical biochemistry, and medical microbiology (including virology, parasitology, and infection control). BMC Clinical Pathology (ISSN 1472-6890) is indexed/tracked/covered by PubMed, CAS, EMBASE, Scopus and Google Scholar.
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