A few days ago, we wrote about the nearly “miraculous” cancer survivor stories of Valerie Harper and Dr. Feelgood frontman Wilco Johnson.
Given only a few months to live because of pancreatic cancer, Johnson rejected all treatments and went on the road to sing. Johnson was quoted as saying:
“The doctors have recently become curious as to why I’m not either dead or very, very ill.”
And apparently, now we know why. Wilco Johnson didn’t have adenocarcinoma of the pancreas. Recent studies show that he has a pancreatic neuroendocrine tumor (PNET)- the same type of tumor that killed Steve Jobs. PNETs arise from the islet cells of the pancreas and grow more slowly than adenocarcinomas.
And with this change of diagnosis, Johnson has changed his plans: BBC 6 Music tweeted:
“Wilko Johnson’s management have confirmed he has undergone, ‘A medical procedure that will see him out of action for the foreseeable future’.”
In an interview with Britain’s GQ, Johnson reveals to Tony Parsons that he “had an operation that removed his football-size tumour, as well as his entire pancreas, his spleen, part of his stomach, parts of both his small and large intestines, and involved the removal and reconstruction of blood vessels relating to the liver.”
In the same interview, he told Parsons that having a diagnosis of terminal cancer gave him a sense of “euphoria”:
“There was a shift of consciousness. I have never felt more alive. I have never known such ecstasies. I have never known such elation. I was living with idea that there was no future for me, that all I’ve got is the present time. Now. The future is yet to come and the past is irrevocable. I watched the snow fall in Kyoto and the sun was coming through the snow and turning it to gold and… I had this euphoria in the moment. And to live in the moment is something that’s very difficult to do. But I was forced to.”
This seems like the perfect time to review the difference between the two different types of pancreatic tumors.
The pancreas is a fish-shaped organ (with a head to the right and tail to the left) which lies behind the stomach. It is made up of two types of cells:
1. Exocrine cells: These cells produce a pancreatic juice that includes enzymes which aid in the digestion of food in the small intestine, breaking up proteins,carbohydrates and fats.
2. Endocrine cells: These are clustered in small groups (called the Islets of Langerhans) scattered throughout the pancreas. These cells produce important hormones such as insulin, glucagon, and somatostatin.
An islet cell tumor is a mass of abnormal cells that forms in the endocrine (hormone -producing) tissues of the pancreas. Islet cell tumors may be benign (noncancer) or malignant (cancerous). Islet cell cancer is rare- only about 5% of pancreatic cancers start here.
When islet cells in the pancreas become cancerous, they may make too many hormones. Islet cell cancers that make too many hormones are called functioning tumors. Other islet cell cancers may not make extra hormones and are called nonfunctioning tumors. Seventy-five percent of islet cell tumors are functioning tumors.
Functional Islet Cell Tumors are often named after the hormone they produce:
Surgery is the most common treatment of islet cell cancer. The doctor may take out the cancer and most or part of the pancreas. Sometimes the stomach is taken out (gastrectomy) because of ulcers. Lymph nodes in the area may also be removed and looked at under a microscope to see if they contain cancer.
If malignant cancer cells spread , they tend to go to the liver, and a portion of the liver may also be removed, if possible. Research has suggested that since the tumor is slow growing, even when present in the liver, that liver transplantation is a reasonable option for islet cell cancer patients with spread to the liver. (Steve Jobs underwent a liver transplant in 2009)
If the cancer is widespread, various forms of chemotherapy may be used to try and shrink the tumors.
The prognosis for islet cell tumors is generally better than adenocarcinomas. Average life expectancy has been stated as 5-10 years. They tend to be slower growing tumors. Also, because the hormones they produce cause symptoms, they are often caught at an earlier stage. Surgery may be curative if found before there is any spread elsewhere.
Pancreatic adenocarcinoma is a cancerous overgrowth of exocrine pancreatic cells (see above).
Inherited genetic disorders cause 5-10% of cases of pancreatic cancer and certain genes can inrease the lifetime risk up 80%.
Because pancreatic cancer is often advanced when it is first found, very few pancreatic tumors can be removed by surgery. The standard surgical procedure is called a pancreaticoduodenectomy (Whipple procedure).
When the tumor has not spread out of the pancreas but cannot be removed, radiation therapy and chemotherapy together may be recommended.
When the tumor has spread (metastasized) to other organs such as the liver, chemotherapy alone is usually used. The standard chemotherapy drug is gemcitabine, but other drugs may be used. Gemcitabine can help about 25% of patients.
Managing pain and other symptoms is an important part of treating advanced pancreatic cancer. Palliative care tams and hospice can help with pain and symptom management, and provide psychological support for patients and their families during the illness.