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Rare disease remains hidden

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Elaine Yauin Beijing

The recent death of Apple co-founder Steve Jobs has turned the spotlight on pancreatic cancer, which claims about 400 lives every year in Hong Kong. The type of cancer that Jobs had - a pancreatic neuroendocrine tumour (PNET) - is actually a rare form of the cancer. Only eight to 10 new cases of PNET occur in Hong Kong every year, compared to 400 for pancreatic cancer, the sixth leading cause of cancer deaths locally.

Dr Lo Chung-yau, a specialist in endocrine surgery, says PNET is a less lethal form of pancreatic cancer. 'PNET grows relatively slowly,' he says. 'If the cancerous cells have not spread to other parts of the body, 60 per cent of patients will still be alive five years after diagnosis; 20 to 30 per cent of patients can still live 10 years after diagnosis.'

If the cancerous cells spread, however, the survival rate drops to 20 per cent five years after diagnosis, says Lo. However, this rate is still higher than that for the common form of pancreatic cancer - adenocarcinoma - which is close to zero. Jobs was diagnosed at the age of 48 and died at 56.

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Because of the slow growth of PNET, the window for treatment is broad, so it's not difficult to cure, according to Dr Peter Teo Man-lung, a specialist in clinical oncology. The flip side, however, is that the lack of clear symptoms usually delays treatment, says Lo.

Because the pancreas lies deep inside the body, it's often difficult to diagnose tumours early because they have yet to interfere with the function of nearby organs such as the stomach, liver or gall bladder. 'While getting a liver scan is easy, normal medical check-ups can't reveal PNET,' says Lo.

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If the tumour is located in the head of the pancreas, it will cause an obstruction in the bile duct. The patient's face will turn yellow, and they will have an infection that doctors can pick up. But the chance of having obvious symptoms is lower if the tumour is located in the tail, farthest from the duodenum.

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