[Durgical](https://durgical.com/en/) › [DurgiPedia](https://durgical.com/en/durgipedia/) › [General pathology](https://durgical.com/en/general-pathology/) › [Liver and gallbladder](https://durgical.com/en/general-pathology/areas/liver/) › Rare primary liver cancers

# Rare primary liver cancers

- Lesion

Besides the common liver cancers there are rare ones arising from vessels, mixed cell types or lymphocytes.

Updated Nov 12, 2026

## Key points

- Angiosarcoma: vinyl chloride (plastics industry), arsenic (pesticides, water), thorotrast (old contrast agent); very poor prognosis.
- Angiosarcoma cells are CD31, CD34 and ERG positive and line irregular blood spaces.
- Epithelioid hemangioendothelioma: multifocal, epithelioid endothelial cells in myxoid stroma; WWTR1-CAMTA1 fusion; intermediate behaviour.
- Combined hepatocellular-cholangiocarcinoma: both components in one tumour; behaves more like cholangiocarcinoma.
- Primary hepatic lymphoma is rare; secondary involvement by systemic lymphoma is much more common.

## What it is

A small proportion of primary liver malignancies are neither ordinary hepatocellular carcinoma nor cholangiocarcinoma. Combined hepatocellular-cholangiocarcinoma has both components and shares the risk factors of HCC. Angiosarcoma is an aggressive endothelial tumour linked historically to vinyl chloride, arsenic and thorotrast. Epithelioid hemangioendothelioma is a lower-grade vascular tumour. Lymphomas and other sarcomas can also arise in the liver.

## Step by step

- Carcinogens such as vinyl chloride are metabolized in the liver to reactive epoxides.
- These damage DNA of sinusoidal endothelial cells.
- Atypical endothelial cells proliferate along sinusoids, forming irregular vascular channels.
- The tumour bleeds, invades and metastasizes early.

## Rare primary malignant tumours of the liver

| Tumour | Cell of origin | Clues | Behaviour |
| --- | --- | --- | --- |
| Angiosarcoma | Endothelium | Vinyl chloride, arsenic, thorotrast; hemorrhagic masses | Highly aggressive |
| Epithelioid hemangioendothelioma | Endothelium | Multiple nodules; WWTR1-CAMTA1 fusion | Intermediate, variable |
| Combined hepatocellular-cholangiocarcinoma | Bipotent progenitor or divergent differentiation | Same risks as HCC | Aggressive |
| [Primary hepatic lymphoma](https://durgical.com/en/conditions/non-hodgkin-lymphoma/) | B or T lymphocytes | HCV, HIV, immunosuppression | Depends on type |

## Markers

- CD31
- CD34
- ERG
- CAMTA1 (nuclear)
- HepPar-1 and keratin 19 (combined tumour)

## Memory aid

Vinyl, arsenic, thorotrast: the three V-A-T poisons that turn liver vessels into angiosarcoma.

## Test yourself

Think of the answer, then tap the question to see it.

## Which three exposures are classically linked to hepatic angiosarcoma?

Vinyl chloride, arsenic and thorotrast.

## Which immunostains confirm an endothelial tumour?

CD31, CD34 and ERG.

## What gene fusion characterizes epithelioid hemangioendothelioma?

WWTR1-CAMTA1.

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## Connections

## Related 5

- [Hepatocellular carcinoma](https://durgical.com/en/general-pathology/hepatocellular-carcinoma/)
- [Cholangiocarcinoma](https://durgical.com/en/general-pathology/cholangiocarcinoma/)
- [Drug- and toxin-induced liver injury](https://durgical.com/en/general-pathology/drug-and-toxin-induced-liver-injury/)
- [Chemical carcinogens: initiation, promotion and the table to know](https://durgical.com/en/general-pathology/chemical-carcinogens-initiation-promotion-and-the-table-to-know/)
- [Heavy metals: lead, mercury, arsenic and cadmium](https://durgical.com/en/general-pathology/heavy-metals-lead-mercury-arsenic-and-cadmium/)

## Related conditions 3

- [Hepatocellular carcinoma](https://durgical.com/en/conditions/hepatocellular-carcinoma/)
- [Cholangiocarcinoma (bile duct cancer)](https://durgical.com/en/conditions/cholangiocarcinoma/)
- [Non-Hodgkin lymphoma](https://durgical.com/en/conditions/non-hodgkin-lymphoma/)

[← All of general pathology](https://durgical.com/en/general-pathology/)

Educational content on the mechanisms of disease; not a diagnostic or treatment guide and no substitute for a health professional's advice. No doses are given.

Sources: [Primary Liver Cancer Treatment (PDQ®) - NCI](https://www.cancer.gov/types/liver/hp/adult-liver-treatment-pdq) (National Cancer Institute, 2026).

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Representative model, for educational purposes only.

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Source: https://durgical.com/en/general-pathology/rare-primary-liver-cancers/
License: CC BY 4.0 (https://creativecommons.org/licenses/by/4.0/). Cite as: DurgiPedia (durgical.com), https://durgical.com/en/general-pathology/rare-primary-liver-cancers/