Actor Irrfan Khan has finally revealed on Twitter that he is suffering from neuroendocrine tumours also known as NETs or carcinoid tumours. It was barely a fortnight ago when Irrfan had stunned the country with his revelation that he is suffering from a “rare disease.” And despite the actor’s pleas to stop unnecessary speculations about his health condition, many websites and news agencies spread rumours that he was suffering from Glioblastoma Multiforme (GBM) Grade IV, a disease known by its ominous sobriquet “Death on Diagnosis.” Today, the actor has put these speculations to end and disclosed the rare disease that he is afflicted by.

Is it the same as brain tumour?

In a subtle jibe to all the rumour mongers who said he was suffering from brain tumour, Irrfan mentioned in his tweet that the word ‘neuro’ does not always mean brain. In fact, the disease refers to tumours that originate in the nervous system (neuro) and the endocrine glands (endocrine), which explains the name. These tumours can either migrate to the rest of the body or can stay in its site of origin. Dr Kaustubh Mahaja, Consultant Neurologist, S. L Raheja Hospital- A Fortis Associate says, "It is, in fact, a contradiction to presume it to be directly associated with the brain. Cases in which these tumours are malignant, they evolve rapidly as they spread to other parts, including the brain. Symptoms may be neurogenic like dizziness or loss of consciousness, which are purely due to the excess hormones secreted which have functions with the brain.”

Is it malignant/cancerous?

Not all neuroendocrine tumours are malignant. Some of them can be non-cancerous or benign says Dr Akshay Shah, Consultant Medical & Hemato-Oncologist and Stem Cell Physician, S.L Raheja Hospital- A Fortis Associate. "Neuroendocrine tumour is a very broad term which is used for tumours arising from neural and endocrine tissues. It can range from benign tumours to frank malignancy," he adds. And at this point, we don't know whether Irrfan’s tumours malignant or benign.

Are there types of neuroendocrine tumours?

There are various types of neuroendocrine tumours, which can migrate to and grow in various parts of the body. The tumours are them categorised according to the sites they migrate to. But broadly, neuroendocrine tumours are categorised either as carcinoid and pancreatic neuroendocrine tumours. Additionally, there’s another category called Pheochromocytoma, which is very rare.

Which parts of the body are neuroendocrine tumours found?

In most cases, these tumours appear first in the lungs or the gastrointestinal tract. Carcinoid tumours originate in the digestive system, lungs, appendix thymus, brain, bone, gonads (testes and ovaries) and skin. Pancreatic Neuroendocrine Tumors are slow-growing tumours that take a very long time to develop. Pheochromocytoma is a rare type that develops in the adrenal glands.

What are the symptoms of neuroendocrine tumours?

"Since they can occur in any part of the body-- commonest being gastrointestinal tract, pancreas and the lungs-- symptoms depend on the organ involved," says Dr Shah. Common symptoms include flushing, diarrhoea, abdominal cramps, bronchospasm, hypertension, hypoglycemia (deficiency of blood glucose) or hyperglycemia (high blood glucose), headache and dizziness.

How rare are neuroendocrine tumours?

Irrfan has already said that he is suffering from a rare disease. Although these tumours comprise less than two percent of cancers than that originate in the stomach, they are more frequent than the stomach and pancreatic cancers combined. The National Cancer Institute has revealed a five-fold increase in the incidence of these tumours. In the last 15 years, the incidence of NET has increased considerably.

What are the causes of neuroendocrine tumours?

The causes of neuroendocrine tumours are still unknown. Some factors can increase your risk of the contracting it like a family history of the disease, excessive exposure to sunlight, smoking, having underlying diseases like pernicious anemia, etc. Dr Shah says, "It is more common in males as compared to females. Factors like underlying HIV infection and  exposure to arsenic have also been postulated in its occurrence." The doctor says that genetic factors such as  MEN 1 (MultipleEndocrine Neoplasia) and MEN 2 Syndrome can also increase your risk.

Are neuroendocrine tumours fatal?

Whether NET would turn fatal or not entirely depends on what stage the disease is in. Like all cancers, early detection will increase chances of survival. The size and location of the tumour will also decide whether the person would survive the disease or not. In the later stages, when the tumour has spread to the nearby lymph nodes, chances of survival are slim.

How are neuroendocrine tumours treated?

The good news is that neuroendocrine tumours are curable and that patients can live for years. Dr Shah explains that diagnosis of neuroendocrine tumours requires a multidisciplinary approach like blood markers, biopsy and specialized PET Scan. "Treatment depends on the staging of the tumour.The treatment options include observation, surgery, hormonal agents, chemotherapy drugs and radiopeptide therapy. But predicting the course of the disease is difficult.

For now all we know is the name of the disease. So it’s impossible to discern the site of the tumour, the stage or the progression of his disease. All we can do as fans and well-wishers is to pray and hope for his speedy recovery.

(The above story first appeared on LatestLY on Mar 17, 2018 09:21 AM IST. For more news and updates on politics, world, sports, entertainment and lifestyle, log on to our website latestly.com).