help for cancer patients after diagnosis

After Diagnosis

After diagnosis

EHE is ultra-rare, and like all ultra-rare diseases, there is a lot we don’t know about the disease.  EHE is so rare that it may even be new to your oncology team. Many oncologists, and associated scanning and imaging departments, have not encountered the disease before. This can lead to patients facing difficult diagnoses, leaving them feeling frightened, isolated and scared post-diagnosis. The first thing we want to stress therefore is that you are not alone. There are many other people with EHE and they are readily available through our global patient network and social media sites. Help and support, information and experience can be found there.

It is possible that you have already been diagnosed with multiple tumours, often referred to as ‘multi-focal’. This is not uncommon with EHE. This may have been described to you as a ‘grade 3 or grade 4’ cancer but the traditional grading system is not appropriate for EHE as the number of tumours is not necessarily indicative of the severity of the disease you have. Some of our members with multiple tumours have been living with the disease for many years.

In addition to its rarity, because the disease is heterogeneous and its natural history is not well understood, the management and treatment of this disease is difficult. Confident prognosis is not possible with different patients responding differently to the same drug regimen.

Accessing EHE and sarcoma knowledge and experience

If you are based in the UK and have recently been diagnosed with EHE, or it has been suggested as a possible diagnosis, it is important that you are referred to a recognised sarcoma unit, if you are not already being treated at one. Your medical team will refer you to the appropriate sarcoma centre for your area. EHE is a very rare form of sarcoma however so it is important therefore that you are treated by specialists in sarcoma who will be connected into the sarcoma network and will be able to seek EHE experience from other UK specialists when and if required.

Although EHE is ultra-rare, there are specialists familiar with the disease who will be able to help you and your oncology team. There is also a growing body of evidence and clinical and research papers relating to EHE. Our own patient community can also provide a huge amount of information and experience that can be invaluable. It is important to find the most current information, because the more informed you are, the less you will fear your disease, and the better prepared you are to discuss your situation and possible treatment plans with your medical team.

Please see our FURTHER INFORMATION page in this section for sources of information.

How will my disease progress?

This is a very difficult question to answer. Haemangioendotheliomas are often indolent (slow growing) but can be aggressive. Almost 50% of EHE cases will involve multiple sites. Tumours can occur anywhere in the body but more typically include sites in the liver, lungs, bone, lymph nodes, and skin. Where EHE involves tumours at multiple sites some tumours may disappear on imaging but reappear later. Some tumours may grow and subsequently regress. In some cases of EHE, tumours may show no adverse symptoms for many years. Indolent forms of this cancer can however become aggressive, but we do not yet understand why or when this will happen. Surgical intervention can remove tumours successfully but in some cases haemangioendotheliomas may recur at the original site, or nearby. Notably, some cases of haemangioendothelioma have been known to go into spontaneous remission. In summary, the progress of EHE is currently unpredictable.

What treatment should I expect?

This is not a question we can answer. From the information above it will be clear that each patient with EHE will have their own unique disease progression. If you have been diagnosed with EHE then you need to ensure that your medical team either have EHE experience or have contacted specialists with EHE experience as we have described above. These specialists together with your own medical team will be able to advice and provide the best treatment plan for your specific circumstances. See our FURTHER INFORMATION page for links to other sites that provide useful information.

The ESMO consensus of experts

The above information is only a summary of some of the key facts about EHE. For more detailed information about the disease and its treatment, please see the ESMO Consensus Paper page in this section of the website. This paper was published in 2021 and represents the consensus of over 85 highly-experienced sarcoma specialists from across Europe, the UK, North America and Asia. Chaired by Dr Silvia Stacchiotti, and produced under the auspices of the European Society of Medical Oncology (ESMO), this paper is the only such recording of clinical understanding and agreed treatment options for the disease.