Liver Transplantation in Primary Hepatic Carcinoid Tumor: Case Report and Literature Review

doi:10.1016/j.transproceed.2009.03.011

Transplantation Proceedings

Volume 41, Issue 4, May 2009, Pages 1386-1389

https://doi.org/10.1016/j.transproceed.2009.03.011 Get rights and content

Abstract

Ninety percent of all carcinoid tumors develop in the gastrointestinal tract. Although the liver is a usual site for metastases, primary hepatic carcinoid tumors (PHCTs) are extremely rare. The diagnosis is based on histopathologic characteristics and on exclusion of a nonhepatic primary tumor. While liver transplantation (OLT) is a well-established surgical treatment in selected cases of unresectable metastatic carcinoid tumor, its use in PHCT has not been widely described. We report the case of a 50-year-old woman with unresectable PHCT treated with OLT. After 64 months, disease recurred in the liver and mesentery. Laparotomy with multiple radiofrequency ablations of liver lesions and resection of peritoneal deposits was performed; however, in the postoperative period, a fatal myocardial infarct occurred. Our case is the fourth one reported in literature. It confirms long-term survival after OLT in patients with unresectable PHTCs.

Section snippets

Case Report

A 50-year old woman initially examined in a district general hospital for possible uterine fibrosis had incidentally diagnosed PHTC 16 years ago when an ultrasound scan and a subsequent CT scan of the abdomen showed a 9-cm tumor in the left hepatic lobe and multiple cystic lesions in the right lobe. Results of liver function tests and tumor markers (carcinoembryonic antigen (CEA), carbohydrate antigen 19-9 (CA 19-9), and α-fetoprotein (AFP)] were all within normal range. Findings in a

Discussion

The introduction of OLT to routine surgical practice has provided an extremely valuable therapeutic tool for treatment of selected hepatobiliary tumors, accounting for 13% (7659 of 58,916) of all indications for OLT in Europe between 1988 and 2006 (www.eltr.org). However, only a highly selected patient population benefits from OLT. When a potentially curative treatment is proposed to patients with malignant neoplasms, the key question is whether the tumor will recur, and when. Therefore,

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Cited by (13)

21 years of evolution of primary hepatic neuroendocrine neoplasm in a patient with primary sclerosing cholangitis: A case report
2023, International Journal of Surgery Case Reports
Primary hepatic neuroendocrine neoplasms(PHNEN) are among the rarest primitive neuroendocrine neoplasms. Main prognostic factor is histological. We report an unusual case of a PHNEN with 21 years of evolution in the setting of a primary sclerosing cholangitis(PSC).
A 40 year old man presented in 2001 with clinical signs of obstructive jaundice. CT-scan and MRI showed a 4 cm large hypervascular proximal hepatic mass evoking hepatocellular carcinoma(HCC) or cholangiocarcinoma. Exploratory laparotomy found an aspect of advanced chronic liver disease of the left lobe. Extemporaneous biopsy of a suspicious nodule showed signs of cholangitis. Left lobectomy was performed and postoperatively the patient received ursodeoxycholic-acid and biliary stenting.
After 11 years of follow-up, jaundice reappeared with a stable hepatic lesion.A percutaneous liver biopsy was done. Pathology showed a G1 neuroendocrine tumor. Endoscopy, imagery and Octreoscan were normal, supporting the diagnosis of PHNEN. PSC was diagnosed on tumor-free parenchyma. The patient is on liver transplantation waiting list.
PHNENs are exceptional. Pathology findings, endoscopy and imagery are necessary to rule out an extra hepatic NEN with liver metastasis. While G1 NEN are known for their slow evolution, this 21 year latency is extremely rare. The presence of PSC adds to the complexity of our case. Surgical resection is recommended when possible.
This case showcases the extreme latency of some PHNEN as well as possible overlap with PSC. Surgery is the most recognized treatment. Liver transplantation seems to be necessary for us, as the rest of the liver shows signs of PSC.
Primary hepatic neuroendocrine carcinoma: A postoperative diagnosis
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Liver Transplantation for High-Grade Primary Hepatic Neuroendocrine Tumor with Diffuse Liver Metastasis
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2020, Evaluation and Management of Liver Masses
Primary hepatic neuroendocrine carcinoma: Report of two cases and literature review
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Case reportLiverLiver Transplantation in Primary Hepatic Carcinoid Tumor: Case Report and Literature Review

Abstract

Section snippets

Case Report

Discussion

Lancet

J Comput Tomogr

J Gastrointest Surg

J Hepatol

Transpl Immunol

An analysis of 8305 cases of carcinoid tumours

Cancer

Hepatic resection and transplantation for primary carcinoid tumours of the liver

Ann Surg

Results of liver transplantation in the treatment of metastatic neuroendocrine tumours: a 31-case French multicentric report

Ann Surg

Liver transplantation for metastatic neuroendocrine carcinoma: an analysis of 103 patients

Transplantation

Case report
Liver
Liver Transplantation in Primary Hepatic Carcinoid Tumor: Case Report and Literature Review