Neuroendocrine tumors
"After stabilizing the patient for inappropriate hormone secretion, if there are no metastases, surgery should be attempted with curative intent, and if there are metastases, adjuvant therapy should be added".
DR. JORGE BAIXAULI
SPECIALIST. GENERAL AND DIGESTIVE SURGERY DEPARTMENT
What is a neuroendocrine tumour?
Neuroendocrine tumours are a rare type of cancer that forms in certain cells of the digestive system and pancreas. These cells have the ability to produce hormones that regulate various body functions.
Approximately 70% of these tumours occur in the digestive tract and 20% in the pancreas. They are divided into functioning, if they produce hormones that cause symptoms, and non-functioning, if they do not produce hormones or do not cause obvious symptoms. They can also be benign or malignant, depending on whether they have spread to other parts of the body.
In most cases, these tumours are malignant and can spread to the lymph nodes, liver (where metastases are most common), bone, lungs or brain. However, some, such as insulinoma, are usually benign.
These tumours are difficult to diagnose because they are usually very small. At the Cancer Center Clinica Universidad de Navarra we have a Radiopharmaceuticals Laboratory, the only one of its kind in Spain, capable of synthesising the necessary compounds to diagnose these tumours with maximum accuracy using imaging tests.
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What are the symptoms of neuroendocrine tumours?
Clinical manifestations are very heterogeneous, as tumours may remain asymptomatic for years or present obstructive symptoms (abdominal pain, nausea, vomiting, cholestasis).
They may also present metastases, which are found accidentally or manifest themselves in typical syndromes due to hormonal hypersecretion.
In most cases, the risk of developing metastases is increased due to the vagueness of the symptoms and the delay in diagnosis (average 3-10 years). The most common symptoms are:
- Diarrhoea
- Abdominal pain
- Nausea
- Heat waves
- Profuse sweating
Do you have any of these symptoms?
If you suspect that you have any of the above symptoms,
you should consult a medical specialist for a diagnosis.
Intestinal neuroendocrine tumours
Intestinal neuroendocrine tumours of the small intestine have an incidence of 0.15-0.5 cases/100,000 per year. Until the development of metastases they are usually asymptomatic or may present with symptoms of obstruction due to the local fibrotic reaction or, rarely, to the tumour mass itself.
At this stage, the typical picture is that of carcinoid syndrome which occurs in 18% of patients with ileal carcinoid and is characterised by hot flushes, diarrhoea, abdominal pain.
Less common are lacrimation, profuse sweating, telangiectasias, cardiac fibrosis and pelagroid skin manifestations due to niacin deficiency.
Pancreatic neuroendocrine tumours
Endocrine tumours of the pancreas can present as typical syndromes caused by hormonal hypersecretion (insulinoma, gastrinoma, VIP-oma (VIP: vasointestinal polypeptide, glucagonoma and somatostatinoma).
40-50% of them are non-functioning or secrete peptides of low biological impact, such as pancreatic polypeptide and neurotensin. Approximately 50% of cases already have metastases at the diagnostic stage.
Insulinoma and gastrinoma are the most frequent pancreatic NETs. 90% of insulinomas are benign, small in size (>90% are < 2 cm) and single (6-13% are multiple).
4-6% are associated with MEN-1 (multiple endocrine neoplasia type 1). Clinically they are characterised by fasting hypoglycaemia and neuroglycopenic symptoms. In general, hypoglycaemia-induced catecholamine release causes symptoms such as sweating, tremor and palpitations.
The chronic hypergastrinaemia caused by gastrinoma results in marked gastric hypersecretion that eventually leads to peptic ulcer disease (which is often refractory and severe), diarrhoea and gastro-oesophageal reflux disease (Zollinger and Ellison syndrome).
How are neuroendocrine tumours diagnosed?
The Clínica is the only Spanish hospital with the capacity to synthesise and apply 18 different radiopharmaceuticals, which allows for a more precise diagnosis of complex diseases.
For the diagnosis and monitoring of these tumours, there are several tumour markers in the circulation or urine. Gastrointestinal tumours are usually studied by endoscopic techniques and endoscopic ultrasound.
Tumours of the small bowel may require, in addition to conventional radiographs (barium contrast bowel studies), the use of newer techniques such as double balloon enteroscopy and capsule videoendoscopy.
Both carcinoid and pancreatic tumours can be studied by computed tomography and magnetic resonance imaging, which allow the extent of metastases to be defined and the response to treatment to be assessed.
How are neuroendocrine tumours treated?
Medical treatment depends on how highly or poorly differentiated the tumour is. Functioning tumours are usually well differentiated and the first therapeutic goal is symptom control. Due to the slow growth of these tumours, with a relatively long life expectancy, it is important to ensure that the patient has a good quality of life.
Treatment of gastrinomas is based on the use of proton pump inhibitors, in doses ranging from 40-60 mg/day to 120 mg/day. Insulinomas are treated with diazoxide associated with hydrochlorothiazide; if this treatment is not effective, calcium channel blockers, ß-blockers and glucocorticoids can be used.
For other well-differentiated tumours, treatment consists of SSA (somatostatin analogues), interferon and, more recently, targeted therapy. SSAs allow control of hormone-induced symptoms and should be used both preoperatively and in inoperable tumours.
In practice, octreotide and lanreotide are used. Interferon A in monotherapy or combined with SSA can be used to inhibit hormone hypersecretion and stabilise the disease. The indications for interferon are the same as for SSA in gastrointestinal NETs, except for carcinoid crises.
In cases with advanced disease, treatment with somatostatin analogues, interferons, both, or even chemotherapy or radiotherapy with octreotide derivatives can be performed when the octreoscan is positive.
Where do we treat it?
The Gastrointestinal Cancer Area
of the Cancer Center Clínica Universidad de Navarra
The Gastrointestinal Cancer Area is composed of a multidisciplinary team of experts in the diagnosis and treatment of diseases of the digestive tract.
It includes specialists in the digestive system, radiology, pathological anatomy, surgery and medical and radiotherapeutic oncology and nursing support.
What diseases do we treat?
Why at the Clinica?
- Integral evaluation of the patient.
- Cutting edge technology.
- Expert professionals who are a national reference.
Our team of experts in neuroendocrine tumours
