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BMC Clin Pathol. 2018 Mar 1;18:3. doi: 10.1186/s12907-018-0070-7. eCollection 2018.

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

Author information

1
1The Jackson Laboratory for Genomic Medicine, 10 Discovery Drive, Farmington, CT 06032 USA.
2
2Department of Biostatistics, Yale University, New Haven, CT 06511 USA.
3
3Division of Vascular Surgery, The Second Affiliated Hospital of Dalian Medical University, Dalian, Liaoning Province 116000 China.
4
4Division of Hepatobiliary and Pancreatic Surgery, The Second Affiliated Hospital of Dalian Medical University, 467 Zhongshan Road, Dalian, Liaoning Province 116000 China.
5
5Dalian Medical University, Dalian, Liaoning Province 116000 China.
6
6Department of Epidemiology of Microbial Diseases, Yale School of Public Health, New Haven, CT 06520 USA.
7
8Division of Hepatobiliary and Pancreatic Surgery, Department of General Surgery, The Second Affiliated Hospital of Dalian Medical University, 467 Zhongshan Road, Dalian, Liaoning 116027 China.
8
Suite 200, 135 College St, New Haven, CT 06510 USA.
#
Contributed equally

Abstract

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.

KEYWORDS:

Immunohistochemical staining; Liver biopsy; Primary hepatic neuroendocrine carcinoma; Somatostatin analogue; Transcatheter arterial chemoembolization

Conflict of interest statement

The study was approved by The Second Affiliated Hospital of Dalian Medical University with the reference number 2017-20 under the fast approval track of The Second Affiliated Hospital of Dalian Medical University.Consent forms of the two deceased cases’ close relatives (in both cases, daughters) were provided.The authors declare that they have no competing interests.Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.

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