Liver & intrahepatic bile ducts
Hepatocellular carcinoma
Lymphocyte rich
Editorial Board Member: Raul S. Gonzalez, M.D.
Editor-in-Chief: Debra Zynger, M.D.
Topic Completed: 28 May 2019
Minor changes: 1 August 2020
Copyright: 2019, PathologyOutlines.com, Inc.
PubMed Search: Lymphoepithelioma-like hepatocellular carcinoma
Table of Contents
Definition / general | Essential features | Terminology | ICD coding | Epidemiology | Clinical features | Radiology images | Prognostic factors | Case reports | Treatment | Gross description | Microscopic (histologic) description | Microscopic (histologic) images | Positive stains | Negative stains | Molecular / cytogenetics description | Sample pathology report | Differential diagnosis | Board review style question #1 | Board review answer #1 | Board review style question #2 | Board review answer #2Cite this page: Fels Elliott DR, Gill RM. Lymphocyte rich. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/livertumorlelhcc.html. Accessed August 6th, 2020.
Definition / general
- Rare variant characterized by pleomorphic tumor cells in a dense lymphoid stroma
Essential features
- Cords of pleomorphic epithelial cells in a dense lymphoid stroma
- Very rare variant of hepatocellular carcinoma (HCC)
- Most cases negative for Epstein-Barr virus (EBV)
Terminology
- Previously called Lymphoepithelioma-like variant
ICD coding
- ICD-10: C22.7 - Other specified carcinomas of liver
Epidemiology
- Very rare tumor with limited clinical and outcome data
- Based on a review of 42 cases reported in the literature, incidence is 3 - 7% of HCC cases; there appears to be a male predominance (M:F = 2.8:1) with mean age 58 years (range 39 - 81 years) (Am J Surg Pathol 2015;39:304, Hepatology 1998;27:407, Histopathology 2000;37:523)
Clinical features
- Approximately half of cases have underlying cirrhosis or chronic infection with hepatitis B / C (Am J Surg Pathol 2015;39:304)
Radiology images
Images hosted on other servers:
CT scan
Prognostic factors
- May have better prognosis in comparison to classic HCC (Am J Pathol 2017;187:1438, Am J Surg Pathol 2015;39:304)
- Majority of patients present with low stage disease, single lesion, without vascular invasion (Am J Surg Pathol 2015;39:304)
- Prognostic factors for HCC: stage (TNM), single lesion versus multifocal, size, vascular invasion, portal vein thrombosis, severity of underlying liver disease (Liver Int 2009;29:502, J Surg Oncol 2018;117:644)
Case reports
- 37 year old woman with chronic hepatitis B and C infection (Medicine (Baltimore) 2017;96:e9416)
- 39 year old woman with liver transplant for end stage liver disease secondary to chronic hepatitis C (Diagn Mol Pathol 2004;13:183)
- 47 year old woman with abdominal pain (World J Gastroenterol 2008;14:4694)
- 64 year old man with cirrhosis (Arch Pathol Lab Med 1999;123:441)
- 81 year old woman with chronic hepatitis C infection (World J Gastroenterol 2015;21:10468)
Treatment
- Surgical resection
- Radiofrequency ablation
- Transarterial chemoembolization (TACE)
- Transplantation (e.g. Milan criteria, modified by some institutions, Nat Rev Gastroenterol Hepatol 2017;14:203)
Gross description
- Majority present as a solitary lesion (Am J Surg Pathol 2015;39:304)
Microscopic (histologic) description
- Cords and trabecula of pleomorphic epithelial cells in a dense lymphoid stroma
- May have focal syncytial growth
Microscopic (histologic) images
Contributed by Ryan M. Gill, M.D., Ph.D.
Dense lymphoid infiltrate
Inconspicuous tumor cells
Cytokeratin
Positive stains
- Cytokeratin
- Hepatocellular markers (arginase1, glypican 3, HepPar1) variably positive
- Majority of lymphocytes are CD3+ / CD8+ cytotoxic T cells (Am J Surg Pathol 2015;39:304)
Negative stains
Molecular / cytogenetics description
- Most cases negative for EBV in situ hybridization (ISH); EBV positivity reported in one study (Diagn Mol Pathol 2004;13:183)
Sample pathology report
- Liver, mass, core biopsy:
- Lymphoepithelioma-like hepatocellular carcinoma (see comment)
- Comment: The tumor shows histologic features of lymphoepithelioma-like carcinoma with pleomorphic tumor cells arranged in nests and cords within a dense lymphoid stroma. The tumor cells are positive for pancytokeratin, HepPar1 and glypican 3, and negative for CK7, CK19 and mucicarmine. EBV in situ hybridization is negative.
Differential diagnosis
- Lymphoepithelioma-like cholangiocarcinoma:
- Classic HCC with increased lymphocytes:
- Tumors with only mild to moderate increase in lymphocytes should be classified as conventional HCC
- Lymphoma (primary hepatic lymphoma or secondary involvement by systemic disease):
- Dense infiltrate of atypical B or T cells
- No intermixed neoplastic hepatocytes
- Positive hematolymphoid markers (depends on the entity), negative cytokeratin and hepatocellular markers
- Inflammatory myofibroblastic tumor:
- Tumor of myofibroblasts with plasma cells, lymphocytes and eosinophils
- ALK+ spindle cells (subset)
- IgG4 related disease:
- Abundant IgG4+ plasma cells, systemic disease, elevated serum IgG4
- Inflammatory variant of angiomyolipoma:
- Inflammatory pseudotumor-like follicular / fibroblastic dendritic cell sarcoma:
- Inflammatory pseudotumor (nonneoplastic):
- Mixed inflammation, granulomas, necrosis / abscess, rule out AFB, T. pallidum, parasites
- No intermixed neoplastic hepatocytes
Board review style question #1
Which variant of hepatocellular carcinoma is shown in the image?
- Fibrolamellar
- Lymphoepithelioma-like
- Sarcomatoid
- Scirrhous
- Undifferentiated
Board review answer #1
Board review style question #2
Which of the following features is characteristic of Lymphocyte rich hepatocellular carcinoma?
- Composed of pleomorphic epithelial cells in a dense lymphoid stroma
- Cytokeratin is usually negative
- Has a worse prognosis in comparison to classic HCC
- It is one of the more common variants of HCC
- Most cases are positive for Epstein-Barr virus in situ hybridization
Board review answer #2
A. Composed of pleomorphic epithelial cells in a dense lymphoid stroma
Reference: Lymphocyte rich hepatocellular carcinoma
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Reference: Lymphocyte rich hepatocellular carcinoma
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