Irrfan suffering from neuroendocrine tumour: Here's what experts have to say about it

CHENNAI: After actor Irrfan Khan revealed that he was diagnosed with neuroendocrine tumour (NETs), which is rare neoplasm, it is to be noted that the annual incidence of neuroendocrine tumours is approximately 2.5-5 per 100,000 throughout the world, which that indicates that incidence of the 
disease is on a rise.

Neuroendocrine tumors (NETs) are usually abnormal growths that develop in neuroendocrine cells and get distributed to other body parts as the disease advances. Most NETs are slowly proliferating and clinically silent, and so are often diagnosed at a stage with advanced disease based on tumour location and whether it is functional or non functional. At the time of diagnosis, more than 50 percent of NETs have already spread to other parts of the body.

While clinical and pathologic features of NETs are unique to the site of origin and treatment is mostly decided based on the site of origin, other characteristics are common. NETs are also sometimes referred to as carcinoid tumours.

“NETs usually appear in the lungs or the gastrointestinal tract, including the stomach, intestines, pancreas, colon, appendix and rectum. NETs are often asymptomatic in the early stage. When present, symptoms appear similar to those caused by other common conditions. Therefore, NETs are at times misdiagnosed as irritable bowel syndrome (IBS),” says Dr KK Agarwal, former president of Indian Medical Association.

Medicos say that the primary treatment for NET patients is the resection of tumour, with symptom control and the limitation of tumour progression as secondary goals.

FDA approves drug for treatment of specific NETs

The Food and Drug Administration recently approved Lutathera (lutetium Lu 177 dotatate) for the treatment of gastroenteropancreatic neuroendocrine tumors (GEP-NETs).

A radioactive drug, or radiopharmaceutical, has been approved for the treatment of GEP-NETs for the first time that consists of synthetic hormone for patients with advanced endocrine tumours. It is estimated that approximately one out of 27,000 people are diagnosed with GEP-NETs per year.

Lutathera is a radioactive drug that works by binding to a part of a cell called a somatostatin receptor, which may be present on certain tumours. After binding to the receptor, the drug enters the cell allowing radiation to cause damage to the tumour cells.

Officials at FDA’s Oncology Center of Excellence said that the approval of Lutathera was supported by two studies. The first was a randomised clinical trial in 229 patients with a certain type of advanced somatostatin receptor-positive GEP-NET. Patients in the trial either received Lutathera in combination with the drug octreotide or octreotide alone. The study measured the length of time the tumours did not grow after treatment (progression-free survival) indicating lower risk of the tumour.

The second study was based on data from 1,214 patients with somatostatin receptor-positive tumours, including GEP-NETS, who received Lutathera at a single site in the Netherlands. 

Complete or partial tumour shrinkage was reported in 16 percent of a subset of 360 patients with GEP-NETs who were evaluated for response by the FDA, says 
a release by FDA’s Center for Drug Evaluation and Research.