Supporting the

Neuroendocrine Cancer Community

Duodenal

Overview

 

The duodenum is the short tube that connects the stomach to the rest of the small bowel (aka small intestine). It is here that semi-digested food and stomach acid (chyme) is ‘processed’ so that nutrients can be absorbed as it passes through towards the large bowel. Key nutrients absorbed in the duodenum include iron, vitamins A and B1, calcium and other nutrients.

Causes and potential risk factors for Neuroendocrine Cancer

We do not know exactly what causes Neuroendocrine Cancer – however it is important to follow advice in leading a healthy lifestyle: eat healthily, exercise and avoid smoking and too much alcohol.

Most Neuroendocrine Cancers do not run in families; however, a number of rare conditions may increase the risk of them developing. Therefore, if other members of the family have been diagnosed with cancer, or have a known genetic condition, it is important that you tell your specialist team about not only your personal medical history, but also any family medical illnesses or conditions.

We recommend speaking to your GP, and specialist team, if you have a family history of MEN1, Von Hippel Lindau or other genetic condition. They may refer you for an NHS genetic test, which will tell you if you have inherited one of the cancer-risk genes – if this is not already known.

Neuroendocrine Cancer of the Duodenum is rare – and may not be associated with the above mentioned genetic alterations. However, if you do have a genetic alteration or strong family history of cancer – knowing this information and identifying your risk, may help your specialist team in planning your care and ensuring follow up is appropriate for you.

Further information on the genetic conditions mentioned here can be found at www.amend.org.uk

Duodenal

Symptoms that may or may not include Neuroendocrine Cancer associated syndromes
(Syndrome is where 2 or more related symptoms occur).

In Duodenal Neuroendocrine Cancer – you may hear the terms “Functioning” (meaning “with hormone related symptoms”) or “Non-functioning” (meaning “without hormone related symptoms”).

Nb. Functioning or non-functioning may also be terms used to describe whether these cancers show up on nuclear medicine imaging* (see diagnostic tests).

Many of Neuroendocrine Cancers of the Duodenum are non-functioning, meaning that they do not over produce hormones. Symptoms, if/when they occur, tend to be related to the size and / or position of the cancer and can include back pain, jaundice, stomach pain, nausea, vomiting and / or weight loss.

Functioning Duodenal Neuroendocrine Cancers can produce syndromes – due to producing too much of specific hormone. These include:

  • Gastrinoma (Gastrin): Zollinger-Ellison syndrome – acid reflux, heartburn, burping, stomach/chest pain, diarrhoea, anaemia
  • Somatostatinoma (Somatostatin): can cause symptoms of diabetes – like feeling tired, going pee a lot, dry mouth, nausea, weight loss and anaemia. They may also cause diarrhoea and / or steatorrhoea (fatty pale stools).

A very small percentage may also produce symptoms of Carcinoid Syndrome: dry flushing of face and torso but can occur over whole body, palpitations, headache and blood pressure alterations, diarrhoea, asthmatic-like wheezing, weight loss or gain, malnutrition and / or fatigue.

Other, rarer symptoms, including Paraneoplastic syndrome and oncological emergencies, (a specific set of health concerns that can occur in any cancer), such as raised calcium levels (Hypercalcaemia), may occur.

Further information about Neuroendocrine Cancer associated and Paraneoplastic Syndromes – including Oncological emergencies – can be found here.

 

Tests that may be used for the diagnosis and / or monitoring of Neuroendocrine Cancer of the Duodenum:

Blood and / or urine:

Full blood count
Liver and kidney function
B12/Iron/Ferritin/Folate
Chromogranin A
Urinary or serum 5HiAA (serotonin)
Gut hormone profile
Thyroid function
LDH
MEN1 screen

Consider Genetic testing ( eg MEN1, VHL, etc).

Scans:

CT chest, abdomen and pelvis and/ or CT chest & MRI abdomen and pelvis
Octreotide (SPECT) or Gallium-Dotatate PET/CT.
FDG-PET – if NEC or rapidly progressive disease seen or suspected.

Endoscopy:

Upper endoscopy – OGD +/- biopsy
Endoscopic Ultrasound (EUS) +/- biopsy

Pathology (what can be seen through special tests under a microscope):

Differentiation and cellular morphology
Synaptophysin
Chromogranin
Ki67
Gut hormone (optional) – even in absence of neuroendocrine associated syndrome
SSTR2a (optional).

The key aim of treatment, should be to help you have the best possible care and quality of life – by ensuring access to appropriate treatment, management of symptoms and addressing what’s most important to you
Treatment options will depend on the type, position and size of your cancer – and whether (and to where) it has spread.
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:

  • Surgery
  • Control of your disease, by slowing or stopping further growth and / or spread
  • Palliation, or easing, of any symptoms you may be experiencing.

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 or partially remove or bypass the primary duodenal neuroendocrine cancer and / or secondary sites of disease (metastases).

Primary surgery may be done by local resection or by endoscopy for very small, localised cancers. However, a more extensive surgery called a Whipples procedure or Pylorus-preserving Pancreaticoduodenectomy (PPPD) may be required to completely remove the cancer and nearby lymph nodes.

A Whipple procedure removes the head of the pancreas, the first part of the small intestine (duodenum), the lower end of the stomach including the pyloric sphincter, the gallbladder and the bile duct. The remaining organs are reattached to allow you to digest food normally after surgery.

Pylorus-preserving Pancreaticoduodenectomy (PPPD) is similar to a Whipple’s, however, the stomach and pyloric sphincter are left intact (not removed).

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:
Is sometimes given after surgery to kill any cancer cells that might remain there. It may also be used for cancer that has spread beyond the primary site, in particular if disease has spread to the bones – here it is used to help control growth of any spread and alleviate bone pain.

Endoscopic procedure:
Treatment given via endoscopy – for example, removal of a stomach, duodenal or rectal polyp.

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

Endoscopic procedure:
Treatment given via endoscopy – for example, removal of a stomach, duodenal or rectal polyp.

Irreversible electroporation (IRE also known as Nanoknife):
Is a relatively novel therapy, not widely available, that uses a strong electric current to kill cancer cells. It may be particularly useful in treating primary or secondary disease when surgery, or other ablation techniques, are risky because tumours are located too close to structures such as major blood vessels.

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 in our “Clinical Trials” factsheet. 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.

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.

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.

For patients with Grade 1 – 2 NET – who have had a complete (R0) or incomplete (R1) resection:

  • 3 – 9 monthly CT or MRI and biochemistry (type depending on pre-operative results) – extend interval as per stability of disease
  • Endoscopic surveillance 12-24 months
  • Functional imaging 18 – 24 months.

For Grade 3 NET/NEC:

  • Resected disease – Contrast CT scan every 3 – 6 months for 2 – 3 years, and then every 6 – 12 months
  • Residual / Advanced disease – Contrast CT scan every 2 – 3 months if on therapy
  • Endoscopic surveillance : as clinically indicated – 6 – 12 months
  • Functional imaging 18 – 24 months – or earlier if progression suspected
    Biochemistry if elevated at diagnosis.

Advanced disease: follow up as per guidelines – but should be guided by prognosis, expected treatment efficacy 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.

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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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