What Are Neuroendocrine Tumours That Celebrities Like Steve Jobs And Irrfan Khan Died Of?

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What Are Neuroendocrine Tumours That Celebrities Like Steve Jobs And Irrfan Khan Died Of?

What Are Neuroendocrine Tumours That Celebrities Like Steve Jobs And Irrfan Khan Died Of? (Image Credits: Instagram)

Known personalities like Steve Jobs and celebrities like Irrfan Khan have succumbed to death due to a rare type of cancer that originates from cells that belong to the neuroendocrine system, which produces and releases hormones into the bloodstream. These cells are dispersed throughout the body, notably in organs like the pancreas, lungs, gastrointestinal tract, and adrenal glands. Although rare, Neuroendocrine Tumours or NETs have been observed because of their slow-growing nature, and for this reason, diagnosis and treatment can be challenging if not taken care of early on. But what are NETs? How does this rare condition happen? We got in touch with Dr Aditya Gupta, Director- Neurosurgery, at Artemis Hospital Gurugram who shares insights on this rare condition.

What Are Neuroendocrine Tumours?

NETs are obtained from neuroendocrine cells, which are functional as nerves and hormone producers. Unlike regular cancer cells that grow aggressively and rapidly, many NETs grow very slowly, which sometimes delays the diagnosis. NETs can be benign or malignant; even benign NETs cause significant health issues due to overproduction of hormones.

“NETs secrete hormones like serotonin, gastrin, insulin, and so on, based on their location. This may cause different symptoms and, therefore, NETs come under the category of being functional or non-functional if it would produce symptoms by the release of hormones,” Dr Gupta explains.

Types Of Neuroendocrine Tumours

Dr Gupta shares that Neuroendocrine tumours are further subdivided based on their origin in the body:

– Gastrointestinal (GI) NETs: The most common NETs; primarily involve the small intestine, appendix and rectum; and may cause hormones that lead to carcinoid syndrome; symptoms include flushing, diarrhea, and breathing problems.

– PNETs: It arises in the pancreas and might make insulin, glucagon, and other hormones. Based on the nature of NET, it can cause variations in the blood glucose level, gastric disturbances, or hormonal disturbances.

– Lung NETs: Although rare, lung NETs can be a bit symptomatic, such as coughing, wheezing, and difficulty breathing. However, hormonal-type symptoms are less common in lung NETs, although sometimes they secrete hormones that can affect bodily functions.

– Other NETs: NETs may sometimes present in the adrenal glands as pheochromocytoma, or in the thyroid as medullary thyroid carcinoma and present their own problems and symptoms.

Symptoms Of Neuroendocrine Tumours

Symptoms of NETs vary dramatically depending on the location of the tumour and the ability of the tumour to produce hormones. Most frequently, symptoms consist of:

– Pain in the abdomen,

– Unintended loss of weight,

– Feeling tired,

– Flushing of the skin,

– Diarrhea, and shortness of breath in some people.

“However, because such symptoms are quite nonspecific or blamed on another disease process, NETs are usually diagnosed late, sometimes years after symptoms start,” he said.

Causes and Risk Factors

“The cause for NETs is not well known; however, there are genetic syndromes such as MEN1 and NF1, which show a high risk. Sometimes, increased family history predisposes a person to more NETs. There are other contributing factors, including age and status of health,” Dr Gupta said.

Diagnosis and Treatment

“It is difficult to diagnose NETs as they grow very slowly and cause nonspecific symptoms. Methods include imaging studies like CT scans, MRIs, and PET scans, as well as blood and urine tests that measure hormone levels. This establishes a diagnosis, identifies the type, and the grade of the tumour,” he said.

The treatment of NETs depends on characteristics, such as the site, size, and stage of the tumour. These may include the following:

Surgery: If diagnosed early, surgery can remove the tumour completely.

Medications: Certain drugs either inhibit hormone production (such as somatostatin analogues) to minimize symptoms.

Targeted Therapy and Chemotherapy: These therapies may be able to inhibit net growth in cases of advanced or aggressive NETs.

Radiation Therapy: This is very rarely utilised, but in some patients, it does help.

“A neuroendocrine tumour is a rare and complex cancer that is affecting thousands of people worldwide. Although a rarity, the knowledge of NETs is constantly expanding, with advances in diagnosis and treatment coming hand-in-hand with improved patient outcomes, and so an awareness of symptoms and risk factors may lead to earlier diagnoses and better management so that most people with NETs can be managed to live fulfilling lives,” Dr Gupta said.

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