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Neuroendocrine Tumours

Management Team

Neuroendocrine Tumours

Overview

Neuroendocrine tumours are tumours that arise from neuroendocrine cells found throughout the body. They can be benign or malignant and often produce hormones.

  • Flushing
  • Diarrhoea
  • Abdominal pain.

  • Blood and urine tests: To measure hormone levels.
  • Imaging studies: To locate the tumour.
  • Biopsy: To confirm the diagnosis.

  • Depends on the tumour type and location and may include surgery, medications to control hormone production, targeted therapies, or chemotherapy.
  • Regular monitoring is important to manage symptoms and detect any tumour recurrence or progression.
  • Early detection and personalised treatment improve outcomes for patients with neuroendocrine tumours.
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Nerve Conduction Velocity (NCV) Studies

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Nerve Conduction Velocity (NCV) Studies

Overview

It helps in evaluating conduction in various large peripheral nerves in our body. The latency, amplitude, and conduction velocity are calculated for the nerve under investigation. A decrease in recorded amplitude implies damage to the axons of the nerve (axonal neuropathy). If the myelin sheath surrounding the nerve gets affected, the nerve conducts the electrical impulse slowly and its conduction velocity decreases. This is seen in demyelinating disorders. Both motor and sensory component of the nerves are studied using this method.

  • Electrodes are placed on the extremities to be studied
  • The nerve is then stimulated via a stimulator by giving a low voltage current
  • An electrode placed at the other end of the nerve records the response

  • Diagnosis of peripheral neuropathy
  • Evaluation of nerve injury
  • Diagnosis of nerve compression or entrapment syndromes
  • Monitoring nerve function in chronic diseases such as diabetes and kidney diseases
  • Evaluation of numbness or weakness
  • Diagnosis of nerve root lesions
  • Pre-surgical evaluation
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Neuroendocrine Tumours (NET)

Management Team

Neuroendocrine Tumours (NET)

Overview

Neuroendocrine tumours arise from special type of cells, called neuroendocrine cells, located within the intestine. These cells are like nerve cells and hormone-making endocrine cells. Neuroendocrine tumours can arise in any part of the body and are rare tumours. Conventionally they are slow-growing and behave differently from the traditional cancers.

Neuroendocrine tumours can form in any part of the body including the lungs and respiratory tract. Neuroendocrine tumours of the intestinal tract may arise from the

  • Small intestine – duodenum, jejunum and ileum
  • Stomach
  • Appendix
  • Large intestine – colon
  • Rectum
  • Pancreas

The tumours may arise from specialised cells, which secrete specific chemicals (hormones). And the over secretion of these hormones can result 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, which do not secrete specific hormones and are referred to as “Non-functioning tumours”.

Depending on their biology and aggressiveness, neuroendocrine tumours are classified into Grades 1, 2, and 3.

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

The exact cause of NET is not fully known. The chances of tumour development increases in the following cases:

  • Genetic mutation: Variations in the genes and a family history increases the risk
  • Pre-existing conditions such as diabetes and peptic ulcer
  • Rare inherited diseases such as neurofibromatosis, multiple endocrine neoplasia and Von Hippel-Lindau syndrome

The symptoms differ based on functioning and non-functioning neuroendocrine tumours.

  • Functioning neuroendocrine tumours

    These secrete hormones which give rise to specific symptoms, based on the hormone secreted. Diagnosis is mainly based on clinical suspicion. Most common types include:

    • 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.
    • Gastrinoma: This is due to over secretion of acid in stomach. Normal gastric acid secretion leads to digestion of the ingested food and absorption in the stomach. However, when gastric acid is released in excessive amounts, this results in severe acidic symptoms, development of “stomach like” ulcers in the large intestine, small intestine, and food pipe (oesophagus). The lesion causing gastrinoma may be present within the pancreas or even in the small intestine (duodenum).
    • Carcinoid tumour: This is due to the release of another chemical–5 hydroxy tryptamine (5 HT)–this hormone typically causes diarrhoea, flushing, and intermittent abdominal pain

  • Non-functioning tumours

    These do not produce symptoms by virtue of over secretion of hormones, as hormones are not secreted by them. However they may produce the following symptoms:

    • Bleeding: presents as blood in vomit or dark stools– Malena. Often, patients simply present with anaemia (low haemoglobin) and further investigations lead to a diagnosis of tumour
    • Abdominal pain
    • Loss of appetite and weight loss

  • Blood tests: Specific blood tests aim to identify elevated levels of the hormones in the blood, such as insulin, gastrin, chromogranin, etc.
  • CT scan and imaging: Neuroendocrine tumours may be incidentally discovered during an ultrasound or CT scan performed for other reasons. Tumours have a characteristic appearance, which can raise suspicion of pancreatic neuroendocrine tumours.
  • DOTA scan: This is a specialised type of nuclear medicine scan, specific and sensitive for neuroendocrine tumours (NETs). The presence of dye uptake confirms the presence of a tumour.

  • Surgery remains the mainstay of treatment for all neuroendocrine tumours.

    Depending on the location of the tumour, the operation indicated would include removal of the affected organ with an adequate surrounding tissue (margin), which may include:

    • Pancreatic surgery: Whipple procedure or distal pancreatectomy
    • Small bowel: Removal of a segment of intestine
    • Stomach: A part or portion affected by the tumour is removed from the stomach. Also known as Gastrectomy.
    • Colectomy: Part of the large intestine is removed
    • NETs may also spread to other parts of the body – e.g. liver.
    • Where possible all attempts to remove the part of the liver bearing the tumour should be performed. This is potentially curative and has achieved very good long-term results.
  • Small asymptomatic non-functioning NETs may be kept under observation and surveillance only after thorough investigation and discussion.
  • Non-surgical therapy
    • Chemotherapy: When surgery is not possible, certain medications are available, which will slow down the progression of the disease and also control the symptoms.
    • Somatostatin analogues: These causes inhibition of release of the hormones and hence, help in controlling the symptoms. The somatostatin analogues also prevent the spread of tumour.
    • Ablation: Radiofrequency ablation or microwave ablation is performed on the disease that spread to the liver or any other organ (e.g. lung).
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Nonalcoholic Fatty Liver Disease (NAFLD)

Management Team

Nonalcoholic Fatty Liver Disease (NAFLD)

Overview

Nonalcoholic Fatty Liver Disease (NAFLD) refers to the accumulation of excess fat in the liver in people who consume little or no alcohol. It is a spectrum of liver conditions, which can progress from simple fatty liver (steatosis) to more severe forms like nonalcoholic steatohepatitis (NASH), cirrhosis, and even liver cancer.

The exact cause of NAFLD is not fully understood, but several factors contribute to its development:

  • Insulin resistance: The liver becomes more likely to store fat when the body becomes resistant to insulin. This is often associated with obesity, type 2 diabetes, and metabolic syndrome.
  • Obesity: Excess body fat, especially abdominal fat, significantly increases the risk of NAFLD.
  • Poor diet: Diets high in sugars, refined carbohydrates, and unhealthy fats can contribute to fat buildup in the liver.
  • Genetics: Genetic factors can predispose individuals to develop NAFLD, though the exact genes involved are still being studied.
  • Metabolic syndrome: Conditions like high blood pressure, high cholesterol, and high triglycerides can increase the likelihood of developing NAFLD.
  • Medications: Certain medications, such as corticosteroids, some cancer drugs, and anti-retroviral drugs, can contribute to the development of fatty liver.

Several factors increase the likelihood of developing NAFLD:

  • Obesity: Overweight individuals are at higher risk, especially those with a higher amount of abdominal fat.
  • Type 2 diabetes: Insulin resistance associated with diabetes often contributes to the development of fatty liver disease.
  • High cholesterol or triglycerides: People with dyslipidaemia (abnormal levels of fats in the blood) have an increased risk.
  • Sedentary lifestyle: Lack of physical activity can contribute to obesity and insulin resistance, both of which are risk factors for NAFLD.
  • Age: The risk increases with age, especially in individuals over 40.
  • Gender: Men are more likely to develop NAFLD, but postmenopausal women also have an elevated risk.
  • Genetics: Family history of NAFLD or other liver diseases increases the risk.
  • Other medical conditions: Conditions such as polycystic ovary syndrome (PCOS), hypothyroidism, and sleep apnea can increase the risk.

In the early stages, NAFLD often does not cause noticeable symptoms. However, as the condition progresses, some individuals may experience:

  • Fatigue
  • Abdominal discomfort or pain (especially in the upper right side of the abdomen)
  • Weight loss (in advanced stages)
  • Enlarged liver (hepatomegaly), which can be detected during a physical examination.
  • Jaundice in advanced cases with cirrhosis.
  • However, many individuals with NAFLD may remain asymptomatic until the disease reaches more severe stages, such as cirrhosis.

  • Blood tests: These can check for liver enzyme levels (ALT, AST) and other markers of liver function. While abnormal results may indicate liver damage, they are not conclusive for diagnosing NAFLD.
  • Imaging tests:
    • Ultrasound: The most common method for detecting fat in the liver. It’s non-invasive and can show liver enlargement or fatty changes.
    • CT Scan or MRI: These can also detect fat accumulation and assess liver structure.
  • Liver biopsy: If NASH or cirrhosis is suspected, a liver biopsy may be performed to assess the extent of liver damage. A biopsy is the gold standard for diagnosing NASH and determining the severity of liver damage.
  • FibroScan (Transient Elastography): This test measures liver stiffness, which is an indicator of fibrosis (scarring of the liver). It can help assess the extent of liver damage.

There is no FDA-approved medication specifically for treating NAFLD, but management focuses on lifestyle changes, monitoring, and treating related conditions.

  • Weight loss:
    • Diet: A balanced, healthy diet is essential. Low-calorie, low-fat diets, and diets high in fruits, vegetables, and whole grains are recommended. Reducing sugar intake, especially refined carbs, is important.
    • Exercise: Regular physical activity can help reduce liver fat, improve insulin sensitivity, and reduce obesity.
  • Control of risk factors:
    • Diabetes management: Keeping blood sugar levels under control is crucial.
    • Lowering cholesterol/triglycerides: Statins or other lipid-lowering medications may be prescribed if high cholesterol or triglycerides are present.
    • Blood pressure control: Managing hypertension can reduce the risk of complications.
  • Medications:
    • While no specific drugs for NAFLD exist, certain treatments may be used to manage related conditions:
    • Vitamin E may be prescribed for individuals with NASH (though it’s not suitable for everyone).
    • Pioglitazone (a diabetes medication) can sometimes be used in NASH with insulin resistance.
    • Avoiding alcohol and hepatotoxic drugs: People with NAFLD should avoid alcohol, which can worsen liver damage. Additionally, certain drugs that can stress the liver should be avoided.
  • Liver transplant: In cases of cirrhosis or end-stage liver failure, a liver transplant may be necessary.

Healthy lifestyle choices are the most effective way to prevent NAFLD. This includes maintaining a healthy weight, eating a balanced diet, exercising regularly, and managing underlying conditions such as diabetes and high blood pressure.

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Neuroendocrine Tumours of the Duodenum

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Neuroendocrine Tumours of the Duodenum

Overview

NETs can also arise in the duodenum, the first part of the small intestine. These tumours are often associated with Zollinger-Ellison syndrome (gastrin-secreting tumours), which can cause peptic ulcers, diarrhoea, and gastroesophageal reflux disease (GERD).

  • Abdominal pain, often related to peptic ulcers or gastritis.
  • Diarrhoea due to gastrin secretion.
  • Gastrointestinal bleeding, manifested as melena or hematemesis.
  • Weight loss or nausea if the tumour is large or metastasises.

  • Endoscopy: To identify the tumour and obtain a biopsy for histopathological analysis.
  • Somatostatin receptor scintigraphy: To assess the extent of disease and metastasis.
  • Gastrin levels: To diagnose gastrinoma in the case of excessive gastrin production.

  • Endoscopy:
    • The primary diagnostic tool for visualising tumours in the oesophagus, stomach, and duodenum.
    • Biopsy is essential to confirm the diagnosis of a neuroendocrine tumour, as well as to classify it as functional or non-functional.
  • Imaging:
    • CT scans, MRI, and positron emission tomography (PET) scans are used for staging, detecting metastasis, and assessing the size of the tumour.
    • Somatostatin receptor scintigraphy (SRS) or octreotide scanning may be used to detect tumours that overexpress somatostatin receptors.
  • Laboratory tests:
    • Plasma or urinary chromogranin A (CgA): A marker for neuroendocrine tumours, though not specific to the GI tract.
    • Gastrin levels: In suspected gastrinoma.
    • 5-HIAA (5-hydroxyindoleacetic acid): A metabolite of serotonin, elevated in functional neuroendocrine tumours like carcinoid tumours.

  • Surgical resection is the mainstay of treatment for localised NETs, particularly when the tumour is small and confined to the stomach, duodenum, or oesophagus.
  • Endoscopic treatments such as endoscopic mucosal resection (EMR) may be considered for smaller, non-invasive tumours.
  • Chemotherapy, targeted therapies, and somatostatin analogues (octreotide) are options for more advanced, metastatic, or functional NETs, particularly those associated with carcinoid syndrome or Zollinger-Ellison syndrome.
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Neuroendocrine Tumours of the Stomach

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Neuroendocrine Tumours of the Stomach

Overview

NETs of the stomach, also called gastric neuroendocrine tumours, are most often found in the antrum (the lower part of the stomach). Gastric NETs are usually associated with gastritis or chronic Helicobacter pylori infection, but they can also arise in the setting of other conditions such as gastrinoma or Multiple Endocrine Neoplasia Type 1 (MEN1).

  • Type 1 Gastric NETs:
    • These are the most common type and are typically benign and associated with chronic atrophic gastritis (often due to H. pylori infection).
    • Patients with type 1 gastric NETs often have hypergastrinemia (elevated levels of the hormone gastrin) due to decreased acid secretion.
    • They are usually small and asymptomatic but can cause anaemia or nausea if bleeding or ulceration occurs.
  • Type 2 Gastric NETs:
    • Type 2 NETs are associated with gastrinoma and Zollinger-Ellison syndrome (a condition where tumours in the pancreas or duodenum produce excess gastrin).
    • These tumours are more likely to be malignant and require careful management.
  • Type 3 Gastric NETs:
    • These are sporadic and are usually more aggressive, with a higher likelihood of metastasis. These tumours are non-functional (they don't secrete hormones) and are more likely to be diagnosed when they cause bleeding or obstruction.

  • Abdominal pain, indigestion, or nausea.
  • Gastric bleeding (manifesting as melena or hematemesis).
  • Weight loss or anaemia in advanced cases.
  • In some cases, symptoms related to gastrinoma, such as diarrhoea, may occur.

  • Endoscopy: A key diagnostic tool to visualise the tumour.
  • Biopsy: Histologic examination confirms the neuroendocrine nature of the tumour.
  • Gastrin levels: For gastrinoma or when gastritis is suspected.
  • Somatostatin receptor scintigraphy (SRS) or PET scans: Useful for detecting metastasis.

  • Surgical resection is the mainstay for localised tumours.
  • Endoscopic resection (e.g., EMR) may be considered for smaller, well-differentiated tumours.
  • In cases of gastrinoma, treatment with proton pump inhibitors (PPIs) to reduce acid secretion and manage symptoms is important.
  • Somatostatin analogues (such as octreotide) can be used to control symptoms of hormone-producing tumours.
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Neuroendocrine Tumours of the Oesophagus

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Neuroendocrine Tumours of the Oesophagus

Overview

NETs of the oesophagus are rare but can occur in both the upper and lower portions. They are typically non-functional (i.e., they do not produce hormones that cause symptoms), although in some cases, they can produce serotonin or other substances.

Patients with oesophageal NETs may present with dysphagia (difficulty swallowing), chest pain, weight loss, or gastrointestinal bleeding if the tumour causes ulceration.

Diagnosis is typically made via endoscopy and biopsy. Endoscopic ultrasound (EUS) may also be helpful to assess tumour depth and local invasion.

Treatment usually involves surgical resection if the tumour is localised. Endoscopic mucosal resection (EMR) or endoscopic submucosal dissection (ESD) may be options for smaller, superficial tumours. For more advanced disease, chemotherapy or somatostatin analogues (like octreotide) may be used.

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Nephrectomy

Management Team

Nephrectomy

Overview

This is a surgical procedure in which a part of a kidney or the complete kidney is removed. Nephrectomy is normally used to remove cancerous or non-cancerous tumours from the kidney.

The length of your hospital stay and recovery time will vary based on your overall health and the type of nephrectomy. After the procedure, it is worthwhile to keep the following in mind:

  • A catheter may be placed for a short time in the bladder to drain urine from the body
  • Light, everyday activities can be resumed a short time after the surgery. However, it is recommended to avoid strenuous activity or heavy lifting for at least 6 weeks after surgery
  • After recovery, you can expect to return to your usual routine and activities; nephrectomy typically does not affect your quality of life

Nephrectomy is categorised into two main types —radical and partial. The complete kidney is removed during a radical nephrectomy. In a partial nephrectomy, only a part of the kidney is removed, and the healthy tissue is left in place.

There are three approaches to a nephrectomy:

  • Open: This is a major surgical procedure in which a surgeon opens up the abdomen and obtains a full view of the internal organs
  • Laparoscopic: This is a technique that uses a thin tube called a laparoscope to perform a minimally invasive surgery in the abdomen
  • Robotic: This is a robot-assisted surgical procedure to remove a part of or the complete kidney
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Nutcracker Oesophagus

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Nutcracker Oesophagus

Overview

This condition also known as hypercontractile oesophagus or jackhammer oesophagus is characterised by excessively strong (and prolonged) oesophageal contractions, resulting in pain and dysphagia (difficulty swallowing). The contractions may be normal in frequency but are excessively forceful.

  • Neuromuscular dysfunction: Disrupted neural regulation of the oesophagus, including impaired inhibitory neurotransmission, may lead to hypercontractility.
  • Chronic acid reflux
  • Psychological stress
  • Oesophageal inflammation: Conditions like eosinophilic esophagitis or general esophagitis may disrupt normal motility.
  • Genetic predisposition
  • Gastroesophageal reflux disease (GERD)
  • Ageing
  • Dietary triggers: Certain foods and drinks, especially those that are spicy or acidic, may trigger or worsen symptoms.

  • Dysphagia (difficulty swallowing)
  • Intense chest pain that is occasionally confused with a heart attack
  • Sensation of food getting stuck in the throat
  • Heartburn (burning sensation in the chest)

  • Oesophageal manometry: This is the primary diagnostic modality for nutcracker oesophagus. Diagnosis is based on the detection of hypercontractile contractions.
  • Oesophageal high-resolution manometry (HRM): This is a more advanced form of manometry. It can provide a more detailed, topographic map of pressure changes in the oesophagus and may be particularly helpful in differentiating nutcracker oesophagus from other oesophageal motility disorders.
  • Endoscopy: It is typically normal in nutcracker oesophagus and is performed to exclude other structural or inflammatory causes (e.g., tumours, strictures, or GERD-related esophagitis).

  • Medications: Various medications are used to relax the oesophagus
    • Calcium channel blockers
    • Nitrates
    • Botulinum toxin injections
    • Prokinetic medications (e.g., metoclopramide, domperidone)
    • Antidepressants (to regulate oesophageal nerve activity)
  • Surgical myotomy: In some cases, myotomy (a procedure involving trimming the circular fibres of the lower oesophageal sphincter (LES) to facilitate movement of food along the food pipe may be considered (if conservative measures fail).
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Neck Pain

Management Team

Neck Pain

Overview

Neck pain may present as mild discomfort, which improves with gentle stretching, while some patients may experience severe pain that may not respond to simple analgesics. Although this condition is self-limiting, patients may seek medical advice for rapid rehabilitation.

  • This condition may arise in all age groups. Although unusual in the paediatric age group, children require medical attention for further evaluation.  
  • Adults may experience neck pain, which might be related to poor posture, excessive screen time, long duration of desk work, or sometimes without any predisposing cause.  
  • Older individuals may experience neck pain because of spinal degeneration.

  • Patients complain of pain in the neck. Sometimes this pain may radiate to the shoulders or arms.  
  • Some patients may experience tingling and numbness in the upper limb or weakness of muscles.  
  • Some patients may complain of severe nagging pain, which may not be relieved even after rest and medications.

  • A thorough clinical examination to rule out that the pain is not being referred from any adjacent joint.  
  • A comprehensive clinical neurological examination to rule out any pinch on the nerve tissue.
  • X-ray of the neck to look for degenerative changes and rule out any fractures, malalignment, or abnormal soft tissue shadows.

  • A short course of anti-inflammatories, analgesics, and muscle relaxants.
  • Local cold fomentation may help in relieving pain and improve rehabilitation.
  • Once the pain reduces, advanced physiotherapy should improve the range of motion, and the strengthening of muscles may be started.
  • Correction of posture and lifestyle modifications are an integral part of treatment.
  • Severe cases may require cervical discectomy.
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