What Irrfan Khan's Story Can Help Us Understand About Neuroendocrine Tumors

What he made public

Irrfan Khan was one of India's most admired actors. Abroad he was known for Slumdog Millionaire, Life of Pi and Jurassic World. In 2018 he told the public that he had a neuroendocrine tumor. The BBC called it a rare cancer. It starts in cells that release hormones into the blood. He was later treated at a hospital in London.

The BBC reported that he died on 29 April 2020, aged 53. He had spent several days in intensive care at a hospital in Mumbai with a colon infection.

He did not say where in his body the tumor was, and this page does not guess. What follows is about the disease he named, not about his care.

A tumor type that hides in plain sight

Neuroendocrine cells sit all through the body. They take a signal from a nerve and answer by putting a hormone into the blood. NCI calls this network the diffuse neuroendocrine system. A neuroendocrine tumor, or NET, grows from one of those cells.

NETs are uncommon. NCI's summary for clinicians gives a worldwide rate of about 2 per 100,000 people a year. That is roughly 0.5% of all new cancers. The average age at diagnosis is 61.4.

Where a NET starts follows the shape of the gut before birth. NCI groups them this way:

• Foregut, up to a quarter of cases: lung, thymus, stomach, or the top of the small bowel
• Midgut, up to half of cases: small intestine, appendix, or the first part of the colon
• Hindgut, about 15%: lower colon or rectum
• The rest: pancreas, gallbladder, liver, kidney, ovary or testis

The pancreatic kind has its own name. NCI calls it a pancreatic neuroendocrine tumor, or islet cell tumor. It is far less common than ordinary pancreatic cancer. NCI states that it also carries a better outlook. NCI adds that although a pancreatic NET can look identical to a gut carcinoid tumor down the microscope, its biology differs, so it is treated and studied as a separate entity.

Why they take so long to name

Most NETs grow slowly, and slow growth is what hides them. NCI notes that many are found by chance, during tests done for something else.

Two things give one away. The first is size. A tumor big enough to press on something causes symptoms, and which ones depends on where it sits. The list includes belly pain, constipation, diarrhea, nausea, bloating, weight loss you cannot explain, and blood in the stool. It also includes jaundice, which means yellowing of the skin and the whites of the eyes.

The second is hormones. NCI splits NETs in two. A functional tumor pours out an active hormone. A nonfunctional one does not. A functional tumor gives itself away through what its hormone does. An insulinoma makes insulin, so blood sugar drops too low. A gastrinoma makes gastrin, and the stomach then floods with acid. That causes ulcers that keep coming back, plus diarrhea. Doctors call that picture Zollinger-Ellison syndrome.

Carcinoid syndrome is the version most people have heard of. It usually means the tumor has reached the liver. NCI explains why. Hormones from a gut tumor drain through the liver first, and the liver breaks them down. It takes liver spread for enough hormone to reach the rest of the body. The signs are flushing or warmth in the face and neck, diarrhea, belly pain, bloating and wheezing. Our page on carcinoid syndrome goes further.

All of that looks like other things: irritable bowel, menopause, asthma, ulcers. NCI's clinical summary notes long delays between a first symptom and an answer.

How they are found and treated

Imaging does most of the work. NCI lists CT, MRI, and endoscopic ultrasound, in which a small probe is passed into the gut. There are also somatostatin-receptor scans. They use a tracer that sticks to a receptor most NETs carry on their surface. Blood or urine tests may measure the tumor's hormone, or a general marker called chromogranin A.

Surgery is the only treatment NCI describes as curative. It is often used even after the cancer has spread, because taking out bulk can quiet hormone symptoms. For a slow-growing NET that has spread, NCI states that careful watching may be the best plan, with no treatment until symptoms need relief. Drugs that block hormone release fill in around that. Radiation has a small role, mainly for bone pain. Our guide to neuroendocrine tumor treatment sets out the order.

Some NETs run in families. NCI names two inherited syndromes: multiple endocrine neoplasia type 1 (MEN1) and neurofibromatosis type 1 (NF1). Stomach conditions such as atrophic gastritis and pernicious anemia also raise risk.

When to get checked

Take these to a doctor, above all if they have run for weeks:

• Flushing of the face and neck in episodes, with no fever
• Diarrhea most days for more than four weeks, and above all with flushing or wheezing
• Stomach ulcers that keep coming back, or reflux that returns after treatment
• Spells of shaking, sweating, confusion or fainting that eating fixes
• Belly pain, bloating or weight loss you cannot explain
• Blood in the stool, or yellow skin or eyes

Nearly all of these have ordinary causes. It is the pattern and the persistence that earn a closer look. Our page for people newly diagnosed with a neuroendocrine tumor covers the first questions.

What this does not mean

• The one medical fact Khan made public was the tumor type. Nothing here describes his treatment, his stage or his outlook.
• The BBC reported a colon infection during his last hospital stay. That is what was reported, not a claim about what his cancer did.
• "Neuroendocrine tumor" is a family, not one diagnosis. Some are watched for years. Some are fast.
• Slow-growing does not mean harmless.
• One person's course predicts nothing about anyone else's.

Sources

• BBC News, Irrfan Khan: Slumdog Millionaire and Life of Pi actor dies — https://www.bbc.com/news/world-asia-india-52468343
• NCI PDQ, Gastrointestinal Neuroendocrine Tumors Treatment (Patient Version) — https://www.cancer.gov/types/gi-neuroendocrine-tumors/patient/gi-neuroendocrine-treatment-pdq
• NCI PDQ, Gastrointestinal Neuroendocrine Tumors Treatment (Health Professional Version) — https://www.cancer.gov/types/gi-neuroendocrine-tumors/hp/gi-neuroendocrine-treatment-pdq
• NCI PDQ, Pancreatic Neuroendocrine Tumors (Islet Cell Tumors) Treatment (Patient Version) — https://www.cancer.gov/types/pancreatic/patient/pnet-treatment-pdq
• NCI PDQ, Pancreatic Neuroendocrine Tumors (Islet Cell Tumors) Treatment (Health Professional Version) — https://www.cancer.gov/types/pancreatic/hp/pnet-treatment-pdq

How this article was prepared

An AI-assisted editorial system helped prepare this page. No named medical reviewer has reviewed it unless one is listed.

The National Cancer Information Foundation publishes Cancer Explained. This page is for learning. It is not medical advice and does not suggest a test or treatment.

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