As millions of Irrfan Khan fans were left stunned at his death, leading health experts on Wednesday tried to explain the rare neuroendocrine tumour (NET) the actor came to know about in 2018 and underwent chemotherapy and treatment in the US.
Neuroendocrine tumour, which can occur anywhere in the body, is a condition in which an abnormal tissue growth arises in the hormone producing nervous cells (or neuroendocrine cells).
Most NETs occur in the lungs, appendix, small intestine, rectum and pancreas, and can be non-cancerous or malignant.
"The NETs begin in the specific cells called neuroendocrine cells. Neuroendocrine cells have characteristics similar to that of hormone-producing cells and nerve cells, and are found throughout the body," Dr P.N. Renjen, Senior Consultant (Neurology), Indraprastha Apollo Hospitals, New Delhi, told .
The NETs don't generally show signs and symptoms at first. "The symptoms depend on the tumour's location and whether it produces excess hormones. In general, neuroendocrine tumour signs might include pain due to its growth, a sense of growing lump under the skin, weight loss without exercise or diet and fatigue," Renjen said.
The NET's incidence is just 2 per 1 lakh population in India, and accounts for 0.5 per cent of all malignancies.
According to Dr Rajendra Kumar, Additional Director (Radiation Oncology) at Fortis Hospital in Shalimar Bagh, the incidence of the NET is 5-6 per one lakh population, globally.
The cause of most of the malignancies is unknown. "There are very few chances of the NET running in the family, but that doesn't mean it will not happen. The NET's possibility is equal among men and women. The prognosis depends upon the variety and grading of the tumour," Kumar said.
The survival of the tumour depends on the pathological variety and its grading. It can be of a low grade, intermediate grade and high grade.
Speaking about the cure, the doctor said although removing the entire tumour was not possible, a debulking surgery was sometimes recommended. This surgery removes as much of the tumour as possible and may give some relief from symptoms.
However, it generally doesn't cure a NET.
The chances of survival in early-stage NET are 70-100 per cent, but as the stage and grading increase, the survival chances decrease.
On diagnosis and treatment, doctors said the treatment was dependent upon the tumour's location. "If the organ is resectable, surgery should be done. If the tumour is of high grade, chemotherapy is advised," Kumar said.
( With inputs from IANS )