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Table of Contents
CASE REPORTS
Year : 2022  |  Volume : 14  |  Issue : 1  |  Page : 40-43

Primary Hepatic Neuroendocrine Tumour: A case report


Department of Pathology, NRI Medical College, Chinakakani, Guntur District, Andhra Pradesh, India

Date of Submission03-Feb-2022
Date of Acceptance20-May-2022
Date of Web Publication21-Jul-2022

Correspondence Address:
Renuka Venkata Inuganti
Department of Pathology, NRI Medical College, Chinakakani, Guntur District, Andhra Pradesh
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/njgh.njgh_4_22

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  Abstract 

Primary hepatic neuroendocrine tumors (PHNETs) are infrequent and rarely cause carcinoid syndrome unlike metastatic deposits in the liver from primary neuroendocrine tumors (NETs) in other parts of the gastrointestinal system. There are only about 61 cases of PHNET reported in the literature. We present a case of a 30-year-old woman with a PHNET in the left lobe of the liver for which a lobectomy was successfully done. This case was subjected to a thorough workup to exclude an occult extra hepatic NET.

Keywords: Liver tumor, neuroendocrine tumor, primary hepatic neuroendocrine tumor


How to cite this article:
Inuganti RV, Boregowda C, Vaddati T, Potti R. Primary Hepatic Neuroendocrine Tumour: A case report. Niger J Gastroenterol Hepatol 2022;14:40-3

How to cite this URL:
Inuganti RV, Boregowda C, Vaddati T, Potti R. Primary Hepatic Neuroendocrine Tumour: A case report. Niger J Gastroenterol Hepatol [serial online] 2022 [cited 2022 Nov 26];14:40-3. Available from: https://www.njghonweb.org/text.asp?2022/14/1/40/351553




  Introduction Top


Primary hepatic neuroendocrine tumors (PHNETs) are extremely rare with only about 60 cases reported in the literature. They arise between 8 and 83 years of age with a slight female prediction. They usually present with mass lesion effects or incidentally, and uncommonly with carcinoid syndrome symptoms.[1]


  Case history Top


A 30-year-old female patient presented with headache, loss of weight, and weakness for 3 months. She complained of pain in the right hypochondrium and epigastrium. There was no history of diabetes mellitus, hypertension, tuberculosis, or jaundice.

Blood workup was normal, and computerized tomography (CT) scan of the abdomen showed a large arterial phase enhancing hepatic mass of size 10 × 8.4 × 8.1 cm in the left lobe of the liver [Figure 1]. The differential diagnosis was a primary hepatocellular carcinoma versus metastatic deposit. All other organs were normal. Fine-needle aspiration was done and reported as malignancy; type was not specified. Alpha-fetoprotein (AFP) levels were within normal limits. We subjected the patient to a meticulous radiological and clinical examination in search of a primary neuroendocrine tumor (NET) and did not find any symptoms, signs, or any other evidence of a primary elsewhere.
Figure 1: CT scan showing a large mass in the left lobe of liver

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Gross appearance

We received a lobectomy specimen of the left lobe of the liver of size 14 × 11 × 6 cm [Figure 2]A. Cut section showed a circumscribed, lobulated, gray-white to gray-tan tumor of 9 × 6 cm with a pushing type of border. All margins appeared to be uninvolved by the tumor [Figure 2]B.
Figure 2: (a) and (b) Tumor left lobe of liver with cut sections showing a lobulated gray white tumor having a pushing type of border

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Microscopic appearance

Sections studied from the material received showed liver tissue with focal fatty change and a tumor distinctly delineated from the liver tissue [Figure 3]A. The tumor showed round cells in nested and trabecular patterns. Individual cells exhibit mild-to-moderate nuclear pleomorphism with salt-and-pepper type of chromatin, moderate-to-abundant cytoplasm, and moderate mitotic activity of 6–8/10 HPF (50 HPFs were counted) [Figure 3]B. The differential diagnoses offered were hepatocellular carcinoma, primary NET, and metastatic deposits from a neuroendocrine carcinoma (NEC). Immunohistochemical marker studies showed diffuse positivity for pancytokeratin, chromogranin, and synaptophysin. Ki 67 showed positivity in 10%–12% of cells [Figure 4]. The final diagnosis was signed off as a PHNET G2.
Figure 3: (a) Liver tissue with focal fatty change and tumor distinctly delineated from the liver tissue, with cells in nested and trabecular patterns (H&E, ×100). (b) Individual cells exhibiting mild-to-moderate nuclear pleomorphism, salt, and pepper type of chromatin and moderate cytoplasm (H&E, ×400)

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Figure 4: (a) Diffuse cytoplasmic positivity (chromogranin, ×400). (b) Diffuse cytoplasmic positivity (synaptophysin, ×400). (c) Perinuclear granular cytoplasmic positivity (pan cytokeratin, ×400). (d) Nuclear positivity in 12%–15% of cells (Ki 67, ×400)

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  Discussion Top


PHNET is rare and was first described by Edmonson in 1958. It represents 0.3% of all carcinoids, and a total of 60 cases of PHNETs have been reported in the literature.[2] They arise between 8 and 83 years of age with a slight female predilection and usually present with mass lesion effects or incidentally, uncommonly with carcinoid syndrome symptoms.[1] In the present case, the patient was a young 30-year-old woman and presented with symptoms of headache, loss of weight, and weakness. Zhao et al.[3] reported two cases of NETs of grade3 with presenting features of abdominal pain and jaundice, whereas Chen et al.[4] reported cases of PHNET with the majority presenting with no symptoms.

On imaging, it would be difficult to differentiate PHNETs from other tumors with a rich blood supply, such as hepatocellular carcinoma, hepatic adenoma, and focal nodular hyperplasia.[4] In our case, a differential diagnosis of primary hepatocellular carcinoma versus metastatic deposit was offered.

The macroscopic appearance is usually that of a solitary, circumscribed, parenchymal mass, which averages 6.5 cm.[1] In our patient, the tumor was circumscribed, lobulated, and 9 × 6 cm with a pushing type of border. In the case series reported by Chen et al.,[4] only one case presented as multiple lesions involving both lobes.

On microscopy, PHNETs commonly show varied patterns of solid, nested, and trabecular patterns.[1] In our case, the latter two patterns predominated.

Immunohistochemistry (IHC) is a useful adjunct in the diagnosis of PHNETs. They include strong and diffuse positivity for the neuroendocrine markers chromogranin and synaptophysin. Cytokeratin shows a granular perinuclear staining pattern.[2] In our case, immunohistochemical tests showed similar results [Figure 4].

Neuroendocrine neoplasms of the digestive system are graded as G1, G2, or G3 based on proliferative activity as assessed by mitotic activity and the Ki 67 proliferation index.[5] Mitotic count is to be done in at least 50 HPFs, and Ki 67 index is the percentage of 500–2000 cells counted in areas of the strongest nuclear labeling.[5] In the present case, mitotic count was 6–8/10 HPF and Ki 67 index was 10%–12% and hence was graded as PHNET G2.

NET G3 also designated as NECs is further subtyped as small-cell NEC (SCNEC) and large-cell NEC (LCNEC) [Table 1]. We compared our case with other studies as shown in [Table 2].
Table 1: WHO grading of NETs of the digestive system (2019)[5]

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Table 2: Comparison with other studies

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  Conclusion Top


When patients have no history of hepatitis or cirrhosis, have normal serum AFP levels, and a large dominant hypervascular hepatic mass, PHNET should be suspected. However, since PHNET is rare, metastasis should be rigorously excluded before diagnosis. IHC aids in the diagnosis, and grading is important for the prediction of prognosis.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Klimstra DS Hepatic neuroendocrine neoplasms. In: Klimstra DS, Klöppel G, La Rosa S, Rindi G, editors. WHO Classification of Tumours of the Digestive System. 5th ed. Lyon, France: IARC; 2019. p. 263-64.  Back to cited text no. 1
    
2.
Shetty PK, Baliga SV, Balaiah K, Gnana PS Primary hepatic neuroendocrine tumor: An unusual cystic presentation. Indian J Pathol Microbiol 2010;53:760-2.  Back to cited text no. 2
    
3.
Zhao ZM, Wang J, Ugwuowo UC, Wang L, Townsend JP Primary hepatic neuroendocrine carcinoma: Report of two cases and literature review. BMC Clin Pathol 2018;18:3.  Back to cited text no. 3
    
4.
Chen Q, Chen X, Lu H, Zhao H, Zhao J, Bi X, et al. Primary hepatic neuroendocrine tumors: Retrospective analysis of seven cases and literature review. Transl Cancer Res 2018;7:428-40.  Back to cited text no. 4
    
5.
Klimstra DS, Klopell G, La Rosa S, Rindi G Classification of neuroendocrine neoplasms of the digestive system. In: Klimstra DS, Klöppel G, La Rosa S, Rindi G, editors. WHO Classification of Tumours of the Digestive System. 5th ed. Lyon, France: IARC; 2019. p. 16-9.  Back to cited text no. 5
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4]
 
 
    Tables

  [Table 1], [Table 2]



 

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Abstract
Introduction
Case history
Discussion
Conclusion
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