Supporting the

Neuroendocrine Cancer Community

Secondary Neuroendocrine Cancer

Metastatic disease.

 

Overview

Secondary cancer is a term used to describe cancer cells that have spread, from the place where they started (primary site), to another part of the body (secondary site).

If these cells form a single secondary tumour, that tumour is called a metastasis, if they form more than one tumour at one or more secondary sites, these secondary tumours are called metastases.

Metastases are formed of the same kind of cancer cells as seen at the primary site – so if a Small Bowel Neuroendocrine Cancer cell goes to the liver, the metastasis formed is still a Small Bowel Neuroendocrine Cancer (as it is made up of cancer cells from the primary tumour and not cells from the secondary site) and not Liver Cancer. Liver cancer starts within the cells of the liver and is a completely different type of cancer.

The spread of Neuroendocrine Cancer is not always a sign of rapid or aggressive cancer. If a very small, slow growing cancer is present then the passage of time can increase the risk of a cancer cell breaking away to enter the blood or lymphatic circulation. Some primary sites can be so small they may not be visible on even the best of scans. If you have secondary cancer and the primary site cannot be clearly identified by scans or other tests, you may be told you have a Cancer of Unknown Primary (CUP).

How does Secondary Cancer occur?

To understand how cancer spreads we need to understand how cancer cells develop and what is in the body that allows it to travel elsewhere.

In terms of cancer development, normal cells have a set of rules that regulate growth and behaviour, in cancer the control signals go wrong and the rules are forgotten!

Normal cells:

  • Develop and grow in a controlled manner
  • Do what they are programmed to do, then die off to be replaced by new cells
  • If damaged – try to repair themselves or die off when they can’t
  • Tend not to separate and travel to other parts of the body
  • If they do grow too big for where they are – tend to push against neighbouring cells, rather than through them.

Cancer cells:

  • Have an uncontrolled development and growth
  • Forget what they are programmed to do, do not die off and new cells continue to develop
  • Have a lack of cell repair and replacement / death, so abnormal cells continue to grow
  • Can break away from where they start to grow and travel to other parts of the body and settle (metastasise)
  • When they do grow too big for where they are, will invade (grow through) neighbouring cells and structures.

Cancer develops when the body’s normal control mechanism stops working.

Cancer cells do not die and instead grow out of control, forming new, abnormal cells. These extra cells form a mass of tissue, called a tumour.

As tumours form and grow, cancer cells can grow into surrounding structures and some may break away from the tumour and enter either the blood stream and/or the lymphatic system.

Secondary cancer may be present at the time that Neuroendocrine Cancer is discovered – indeed this is quite common and true for more than half of those diagnosed.

For many, the primary disease may have been present for a number of months or even years, with no signs or symptoms, until the disease has spread elsewhere. It may not be until symptoms, caused by the secondary tumour, occur that a suspicion that something is wrong occurs.

For example; in Neuroendorine Cancer of the Small Bowel it is often the presence of a mesenteric mass (lymph node/tissue mass near the small bowel) or liver metastases that causes symptoms that leads to diagnosis.

There are a number of tests that can be carried out to confirm a diagnosis of Primary and / or Secondary Neuroendocrine Cancer.

These include blood and urine tests, as well as scans and other investigations, such as a biopsy (taking a sample of the cancer to examine it under a microscope).

The type of tests you may have will be based on the type of Neuroendocrine Cancer you are thought to have or are known to have – and whether there is/was any evidence of secondary disease at the time of first diagnosis.

As with more common cancers, the earlier in the development of a primary cancer it is diagnosed, the higher the possibility of cure is. However, for Secondary Neuroendocrine Cancer there is no cure – but, this does not necessarily make this a terminal diagnosis.

No cure (incurable) is NOT the same as ‘terminal’, and many people living with Neuroendocrine Cancer are doing just that – living, and many are living long and well, with the support of their families, support network and specialist Neuroendocrine Cancer team.

Treatment will depend on the type (grading, functionality, etc.), position and size of your Primary Neuroendocrine Cancer. And the extent of your Secondary Cancer – where and how widely it has spread and overall how much cancer is present is important to know.

It may depend on whether you had Secondary Cancer at the time of diagnosis – or whether it has developed since – and whether you have already had treatment. If so, what effect did the treatment have – on both you and the cancer.

It will also depend on whether you have any other health concerns and / or illnesses and your general health and fitness.

One or more of the approaches below may be suggested:

  • Removal of all or part of the cancer
  • Control of disease, by slowing or stopping the growth of cancer
  • Monitoring or Surveillance
  • Palliation, or easing of, symptoms

Surveillance:
Monitoring through clinic review, bloods and scans, can be used to assess how well treatment is working or in periods between treatments (which may be months/years).

As not everyone will need to be on treatment – surveillance can be used to check your cancer and general health for any signs of change that may mean that a treatment might need to be considered. All treatments have possible side-effects, therefore, it is important to know when treatment may be helpful for you or not.

Surgery:
To remove, partially remove or bypass neuroendocrine cancer and / or secondary sites of disease (metastases).

Non-surgical treatments:

Somatostatin analogues (SSAs):
Can be used to help regulate the secretion of hormones if abnormal levels are being produced. SSAs may also be used to slow down growth rate in low to moderate grade ‘well-differentiated’ neuroendocrine cancer (NET).

Chemotherapy:
Can be given orally (in tablets) or Intravenously (through a vein) to slow tumour growth or try to reduce tumour size. This may be the first line therapy in high grade disease – particularly “poorly-differentiated’ NEC or in combination with other treatments. Chemotherapy may also be used to increase tumour cell sensitivity to radiation therapies.

Targeted Molecular Therapies:
Can be given orally (in tablets) or Intravenously (through a vein) to slow tumour growth or try to reduce tumour size.

Peptide receptor radionuclide therapy (PRRT):
May also be called Radioligand Therapy – uses targeted radiation to treat neuroendocrine cancer cells. Can be used in some patients who have had a ‘positive’ Octeotide or Gallium scan (‘receptor positive’ disease).

Radiotherapy:
Can be used alongside other treatments such as chemotherapy, after surgery or on its own. It can be particularly useful for treating a single site painful bone metastasis.

Endoscopic procedure:
Treatment given via endoscopy – for example, insertion of a stent to keep an airway, bile duct or part of the bowel open, if it has become blocked or narrowed by a cancer that cannot be removed.

Interventional radiology:
Through techniques such as embolisation or ablation – can be used to treat Neuroendocrine Cancer that may have spread to the liver and / or lung(s).

Clinical Trials:
Clinical research and safe new treatment development is essential to provide best care for those with Neuroendocrine Cancer – we need to know that treatments not only work but work safely. There are several phases of trial therapy – further information can be found HERE. Each trial will have specific criteria in regards to patient suitability – this can be discussed with your clinical team. You do not have take part in a trial – participation is voluntary.

Bone-strengthening treatment:
Such as Bisphosphonates or other agents may be used in the management of bone metastases.

Symptom Control:
Managing symptoms, including pain, is an important part of total care – and therefore occurs throughout care, not just at ‘end-of-life’. Symptom control or ‘palliation’ refers to what is used to alleviate or reduce the impact your cancer, other health issues and /or treatments may be having on you and your physical and mental health. It can include anything from simple medication and / or a combination of some of the treatments mentioned above to counselling and practical support.

This is not an exhaustive list – and other treatments may be offered that are tailored specifically to you. Your symptoms, your cancer, your overall health and, importantly, your choice.

All treatments that will not cure should be focused on your benefit (what you will get from them) and your quality of life.

There are expert agreed guidelines regarding how and when follow up should occur, however, in practice this varies and often with good reason – follow up should be expert informed & evidence /research based but also tailored to you and what is appropriate for your best care.

Advanced disease:
Follow up as per guidelines – but should be guided by prognosis (life-expectancy), expected treatment outcome and treatment related toxicity.

Your health, well-being, physical activity, informed choice and preference for ongoing care as well as aim of treatment should be reviewed and discussed to best plan care.

About Us

Neuroendocrine Cancer UK is a UK wide charity solely dedicated to providing support and information to those affected by Neuroendocrine Cancer.

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