Neuroendrocrine Tumours

These are a special type of tumour – these arise in a special type of cells – located and found within the INTESTINE – these cells are called the neuroendocrine cells.

The neuroendocrine cells are like nerve cells and are like hormone making endocrine cells. Neuroendocrine tumours can arise virtually in any part of the body and by itself, they are rare tumours.

Conventionally they are slow-growing and behave differently from the traditional cancers as we know them.

The tumours prize from specialised cells which secrete specific chemicals ( hormones ). The over secretion of these hormones results in specific symptom complexes ( syndromes ) which can be attributed to the action of these hormones. Tumours associated with over secretion of these hormones and specific syndromes are called “Functioning tumours”. There are Neuroendocrine tumours ( NET ), which do not secrete specific hormones and are referred to as “Non-Functioning Tumours”.

NEUROENDOCRINE TUMOURS OF THE INTESTINAL TRACT MAY ARISE FROM THE

1. SMALL INTESTINE – duodenum, jejunum and ileum

2. STOMACH

3. APPENDIX

4. LARGE INTESTINE – COLON

5. RECTUM

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6. PANCREAS

Neuroendocrine tumours can virtually form in any part of the body – including the lungs and the respiratory tract.

Symptoms and presentation.

Functioning Neuroendocrine tumours secrete hormones which give rise to specific symptoms, based on the hormone secreted. A high index of clinical suspicion is required to make a diagnosis.

eg. [ more common ones ]

1. Insulinoma : Secondary to over-release of insulin from the pancreas, this leads to recurrent and repeatedly low sugar levels – thereby leading to symptoms of hypoglycaemia, and neurological symptoms due to constant low blood sugar.

2. Gastrinoma : This is due to over secretion of gastric acid in the stomach. the normal acid secretion leads to digestion and absorption of food in the stomach. however, when gastric acid is released in excessive amounts, this results in severe acid symptoms, development of “stomach like” ulcers in the intestine – small intestine, food pipe ( oesophagus ). the lesion causing gastrinoma may be present within the pancreas or even in the small intestine ( duodenum ).

3. Carcinoid Tumour : This is due to the release of another chemical – 5 hydroxy Tryptamine ( 5 HT ) – this hormone typically causes diarrhea, flushing, and intermittent abdominal pain

Non Functioning Tumours

These do not produce symptoms by virtue of hormone oversecretion as hormones are not secreted by them

However they may produce the following symptoms

  • Bleeding: presents as vomiting with blood in vomiting or dark stools – Malena. Many times, patients simply present with anaemia -: low haemoglobin and further investigations lead to a diagnosis of GIST
  • Abdominal pain.
  • Loss of appetite and weight loss.
  • Incidental ( by chance ) discovery on an ultrasound or CT scan done for any other reason.

depending on their biology and aggressiveness neuroendocrine tumours are classified into 3 grades

Grade 1
Grade 2
Grade 3.

Grade 3 tumours are also called Neuroendocrine Carcinomas ( NEC ) and are known to be locally aggressive and spread to other organs as well. Grade 1 and Grade 2 tumours have more benign behaviour and are slow-growing.

Diagnosis

  • Blood tests – specific blood tests directed towards identifying the elevated levels of the hormone in the blood are diagnostic. eg: insulin, gastrin, chromogranin, etc.
  • CT scan and imaging – the tumours have a characteristic appearance which raise the suspicion of Pancreatic Neuroendocrine tumours.
  • DOTA scan: this is a specialised type of nuclear medicine scan, specific and sensitive for NET -the presence of pick up of the dye confirms the presence of tumour.

Treatment

Surgery remains the mainstay of treatment of all Neuroendocrine tumours : NET

1. Small asymptomatic non-functioning NETs may be kept under observation and surveillance only after thorough investigation and discussion.

2. Surgery remains the mainstay of treatment of NETS- removal of the tumour through a standard operation is curative.

Depending on the location of the tumour – the operation indicated would include removal of the affected organ with an adequate surrounding tissue ( margin ).

  • Pancreatic surgery – Whipple procedure or distal pancreatectomy
  • Small bowel – removal of a segment of intestine
  • Stomach – gastrectomy – removal of part or portion of the stomach
  • Colectomy – removal of part of the large intestine

3. NETs may also spread to other parts of the body – eg: Liver

– Where possible all attempts to remove the part of the liver bearing the tumour should be done. this is potentially curative and achieved very good long term results.

Non-Surgical Therapy

1. Chemotherapy – where surgery is not possible, certain medications are available, which slow down the progression of the disease and also control symptoms.

2. Somatostatin analogues – These cause inhibition of release of the hormones and hence help in controlling the symptoms – the somatostatin analogues also prevent spread of the tumour.

3. Ablation – Radiofrequency Ablation or Microwave ablation of the disease spread to the liver or any other organ eg: Lung, etc.

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