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Epithelioid haemangioendothelioma of the liver: a plain guide | CION Cancer Clinics
Epithelioid haemangioendothelioma, or EHE, is a very rare cancer that starts in the blood-vessel cells of the liver. It often grows slowly, and many people have no symptoms when it is found. This page explains how EHE is confirmed, why doctors sometimes watch before treating, and which options exist, from surgery and transplant to drug treatment. At CION Cancer Clinics in Hyderabad, our oncologists explain liver cancer in plain words and plan care around you and your family.
On this page
The short answer
What is epithelioid haemangioendothelioma of the liver?
Epithelioid haemangioendothelioma, usually shortened to EHE, is a very rare cancer that starts in the cells lining the small blood vessels of the liver. It is a primary liver cancer, meaning it began in the liver. It is not the common liver cancer called hepatocellular carcinoma, and it is not cancer that spread from somewhere else.
Why it surprises everyone
EHE often grows slowly, and many people have no symptoms when it is found. It usually shows up as several small spots scattered through both sides of the liver, often on a scan done for another reason. Because it is so rare, the first scan report may call it something else, and the right name sometimes only appears after a biopsy.
Not the same as a haemangioma
The names sound alike, which frightens families. A liver haemangioma is a common, harmless tangle of blood vessels. EHE is a cancer, even though it often behaves gently for a long time.
If you were told about a spot on the liver and do not yet know whether it started there, read primary versus secondary liver cancer first.How it behaves
Is EHE slow-growing or aggressive?
It can be either. The team watches how it behaves in you rather than assuming one pattern.
Quiet for a long time
In many people the spots barely change from one scan to the next. Some people live with stable EHE for many years with scans alone.
Slowly growing
The spots grow or new ones appear over time. This usually prompts the team to discuss treatment rather than more watching.
More active disease
A smaller group has faster growth or symptoms. Doctors look for these signs in particular:
- Pain or a swollen abdomen
- Fluid in the abdomen or around the lungs
- Weight loss and tiredness
- Spots in the lungs, bones or lymph nodes
Spots in more than one organ
EHE can appear in the liver and the lungs at the same time. This does not always mean fast-moving disease, but it changes which treatments make sense.
Not sure whether this applies to you?
Ask an oncologistGetting the name right
How do doctors confirm it is EHE?
The scans
Ultrasound, CT or MRI shows several spots, often near the edge of the liver. On their own, scans can suggest EHE but cannot confirm it. CION arranges these scans through diagnostic partners.
A biopsy
A thin needle takes a small piece of one spot, guided by a scan. Unlike the common liver cancer, EHE cannot be named without tissue. See what a liver biopsy involves.
Special stains and a gene test
The pathologist uses stains that show blood-vessel cells, and often a test on the tissue for the gene change typical of EHE. This separates it from angiosarcoma and from spread of other cancers.
A second look at the slides
Because few pathologists see EHE often, a second opinion on the slides is reasonable and common.
The tumour board
Oncologists, surgeons and radiologists review the scans and report together and agree whether to watch or treat.
The options
What treatment options exist, and who does each suit?
There is no single standard plan for EHE. The tumour board weighs how many spots there are, whether they are growing, whether anything lies outside the liver and how well you are. Surgery and transplant are planned with partner hospitals; your team will tell you where any operation takes place.
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Commonly believed
What families often assume about EHE, and what is true
EHE often starts as many spots in the liver at once. That is how this tumour tends to appear, and it does not mean the same thing as many spots from a cancer elsewhere.
For stable EHE, watching is an active choice. It avoids treatment side effects while the disease is quiet, and treatment starts if scans show change.
EHE and haemangioma are different conditions with similar names. EHE needs a specialist plan and regular follow-up, even when it is quiet.
EHE is a different disease. Treatments are chosen from the small body of evidence on EHE itself, which is still thin.
Being straight with you
What this page cannot tell you
It cannot tell you whether the spots on your scan are EHE. Only a biopsy read by an experienced pathologist can do that. It also cannot tell you how your own disease will behave. That becomes clear over time, from comparing scans.
The evidence is thin
EHE is so rare that most of what doctors know comes from small groups of patients and case reports, not large trials. Good specialists will say so openly. Ask your team what each recommendation is based on, and whether a clinical trial might be relevant later.
Who this page does not apply to
It is not about the common liver cancer, about cancer that spread to the liver from the bowel, breast or elsewhere, or about a harmless haemangioma. It is not about children's liver tumours either; for those, see hepatoblastoma in children.
If you have a report that mentions EHE, the helpline can arrange for the CION team to review it with you.Questions we are asked
Common questions about liver EHE
Is epithelioid haemangioendothelioma cancer?
Yes. It is a rare cancer of blood-vessel cells. It sits between harmless vascular lumps and aggressive vascular cancers, and many people have slow-growing disease. It still needs a specialist plan and regular scans, even when no treatment is given straight away.
Can EHE of the liver be treated successfully?
Many people do well for a long time. When the disease can be fully removed by surgery or transplant, the aim can be curative. When it cannot, treatment aims to control it. Your team will explain which aim applies to you, without promising an outcome.
Why did my scan report not say EHE?
Because it is rare and looks like other conditions on a scan. Reports often describe several liver spots and suggest spread from elsewhere. The name usually comes from the biopsy and the special stains, which is why the tissue matters so much.
Do I need a liver transplant?
Only some people do. Transplant is considered when many spots fill the liver, surgery cannot remove them and there is no spread elsewhere. It is assessed by a licensed transplant centre, and CION cannot influence the waiting list in any way.
Is there a tablet or injection for EHE?
Some drugs have been tried for growing EHE, but the evidence comes from small studies. Some are given only within trials, and availability in India varies. Your oncologist will explain what is realistic in your case and what is still experimental.
Did something I did cause this?
No known habit, food or infection causes EHE. It is linked to a gene change inside the tumour, which is not inherited and is not passed to your children. Nothing you ate or drank brought it on.
How often will I need scans if we are watching?
Scans are usually closer together at first, then spaced out if nothing changes. Your team sets the timetable for you. Keep every scan and report together so each new one can be compared with the last.
Should I get a second opinion?
It is reasonable for a tumour this rare. Ask for the biopsy slides to be reviewed by a pathologist experienced in vascular tumours, and for the scans to be read again. The helpline can tell you how to bring your reports to a CION centre.
Meet CION's liver cancer specialists. Bring your scan reports and liver blood tests.
Our medical and surgical oncologists plan liver cancer care together, weighing the tumour and how well your liver works before choosing surgery, a liver-directed procedure or medicines.
Dr. C. Raghavendra Reddy
MBBS(Gold Medal), DNB(General Medicine), DM(Medical Oncology)(Gold Medal)
Dr. Bharati Devi Gorantla
MBBS, MD(General Medicine), DM(Medical Oncology)(Adyar,Chennai), ECMO, MRCP SCE(UK)
Dr. Owais Mohammed
MBBS, MD (General Medicine), DrNB (Medical Oncology), ECMO, MRCP SCE (Medical Oncology) (UK)
Dr. Muralidhar Muddusetty
MBBS (AIIMS), MS (Surgery) (AIIMS), DNB (Surgical Oncology), MRCS (Edinburgh)
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Sources
- National Cancer Institute — What Is Liver Cancer?
- Cancer Research UK — Liver cancer
- MedlinePlus — Liver biopsy
- Cancer Research UK — Secondary liver cancer
This page is general information, not a prescription. Do not change or stop any treatment based on what you read here. If anything is worrying you, contact your own treating team — or call our helpline and we will help you reach the right specialist.
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Have a report that mentions EHE?
Send us your scans and biopsy report and the team will go through them with you and explain the next step. One helpline serves every CION centre.