Ann Hepatobiliary Pancreat Surg.  2018 May;22(2):159-163. 10.14701/ahbps.2018.22.2.159.

Long term recurrence in primary liver neuroendocrine tumor: Report of a single case and review of literature

• Pirozzolo
• Cona C
• Rizzo M
• Shala F
• Berisha S
• Recordare

• KMID: 2412432
• DOI: http://doi.org/10.14701/ahbps.2018.22.2.159

Abstract

Primary liver neuroendocrine tumors (PLNETs) are rare tumors of the liver. They share some common characteristics with neuroendocrine tumors (NETs) of the extrahepatic bile ducts, such as slow rise, hormonal, and histological features. Nevertheless, they possess some peculiarities and the major feature is the difference in the metastatic potential between PLNETs and NETs. PLNETs have less metastatic potential compared with NETs, which is the main factor based on which differential diagnosis between the two groups is achieved. There exists few reports disease's long-term outcome, especially about the recurrences management. We report the case of a 52-year-old woman admitted to hospital for jaundice and presence of liver mass. She underwent extended right hepatectomy and subsequently, PLNET was revealed. After 9 years, a new mass was discovered in the remnant liver, far from the resection line, and was surgically removed. Histological examination confirmed a PLNET recurrence. The patient is alive and doing well after a year of surgery. We conducted a review of the literature on recurrent PLNETS. Five papers followed our inclusion criteria and included 10 patients. Clinical presentation was mostly nonspecific in included cases and no carcinoid syndrome was reported. Median overall survival and median disease-free survival periods were 22 and 5 months, respectively. The primary disease was treated with surgical resection in all the included cases and recurrent diseases were mostly treated with non-surgical techniques (mainly transarterial chemoembolization). In conclusion, more studies should be conducted in order to have significant data about this uncommon neoplasm. Finally, considering the lack of data on long-term outcome, a long and accurate follow-up should be considered.

Keyword

Carcinoid tumor; Neuroendocrine tumor; Liver neoplasms; Neoplasm recurrence, local

MeSH Terms

Bile Ducts, Extrahepatic
Carcinoid Tumor
Diagnosis, Differential
Disease-Free Survival
Female
Follow-Up Studies
Hepatectomy
Humans
Jaundice
Liver Neoplasms
Liver*
Middle Aged
Neoplasm Recurrence, Local
Neuroendocrine Tumors*
Recurrence*

Figure

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  Fig. 1 Computed tomography scan portal phase. The scan shows an hypodense lesion of the segment V and IVb (T) involving the right branch of the portal vein. There is a slight enlargement of the intrahepatic bile ducts. The main trunk of the portal vein is patent. Based on imaging, an extended right hepatectomy was planned to remove the tumor.

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  Fig. 2 Photograph of the resected specimen after extended right hepatectomy. The segment IVa was spared. V: stump of the right hepatic vein. P: right portal pedicle. T: tumor. The gallbladder was left on site, and the tumor appears on the surface of the liver in the floor of segment V.

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  Fig. 3 Magnetic resonance imaging finding of T2 weighted scan 9 years after the first resection. A hyper intense lesion involving segment III and part of segment II is evident on T2 phase. The tumor is in close relationship with the left portal branch. A portal branch for segment II is involved in the tumor, as well as the distal part of the left hepatic vein. The neoplasm goes toward the caudate lobe.

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  Fig. 4 Intraoperative finding of non-anatomical liver resection. The intraoperative picture shows the liver remnant and the section plan. The main left portal branch was left, and its branch for segment II tied at its origin. In the right side the stumps of the resected distal left hepatic vein are shown.

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