Ann Hepatobiliary Pancreat Surg.  2020 Feb;24(1):17-23. 10.14701/ahbps.2020.24.1.17.

Primary hepatic neuroendocrine tumours of liver- a rarity: Single centre analysis of 13 patients

• Parray A
• Patkar S
• Goel M

• KMID: 2471184
• DOI: http://doi.org/10.14701/ahbps.2020.24.1.17

Abstract

BACKGROUNDS/AIMS
Primary hepatic neuroendocrine tumours (PHNETs) are a rarity and this rarity imparts management complexities.
METHODS
A retrospective analysis of prospectively maintained liver database from 2009 to 2018 was performed and patients with PHNETs were identified and studied for clinical, imaging and pathological features, surgical outcomes, disease free and overall survival.
RESULTS
Thirteen patients of PHNET were identified following rigorous investigational protocols, which constituted 0.6% of all liver tumours (2095) in our series. The median age of patients was 50 years (14-65), with male to female ratio of 9:4. Eight patients (62%) underwent hepatic resections as primary treatment, while 5 (38%) patients received peptide receptor radiotherapy, trans-arterial chemotherapy, trans-arterial radiotherapy or a combination of these. In the surgical group at a median follow up of 36 months (range 5-114 months), 4 (50%) patients were alive without disease and disease free survival was 20 months. Median OS in surgical group was 47 months (40-53, 95% confidence interval) that was better but not statistically significant from that of non-surgical treatment group (36 months).
CONCLUSIONS
PHNETs are rare tumours that require multidisciplinary treatment approach. Liver directed surgery centred management leads to better clinical outcomes in these selected patients.

Keyword

Rare liver tumours; NETs of liver; Primary NETs of liver

MeSH Terms

Disease-Free Survival
Drug Therapy
Female
Follow-Up Studies
Humans
Liver
Male
Prospective Studies
Radiotherapy
Receptors, Peptide
Retrospective Studies
Receptors, Peptide

Figure

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  Fig. 1 Different MDCT characteristics of PHNETs. (A) Multilobulated cystic mass with rim enhancement. (B) Multiple hypodense cystic masses. (C) Arterially enhancing solid mass. (D) Hypoenhancing solid mass. (E) Metabolically active DOTANOC lesion in right lobe.

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  Fig. 2 Kaplan Meier survival curves. (A) Survival as a function of tumour grade. (B) Survival as function of Ki67 index.

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