How is Wilko Johnson Like Steve Jobs?

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.

What is the pancreas and what does it do?

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.

Islet Cell (Neuroendocrine) Cancer of the Pancreas

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:

  • Gastrinoma -The tumor makes large amounts of a hormone called gastrin, which causes too much acid to be made in the stomach. Ulcers may develop as a result of too much stomach acid.
    • Symptoms:  Abdominal pain, diarrhea, ulcers in the stomach and small bowel and occasionally vomiting blood
  • Insulinoma -Overproduction of the hormone insulin and causes the body to store sugar instead of burning the sugar for energy. This causes too little sugar in the blood, a condition called hypoglycemia.
    • Symptoms: Feeling tired or weak, shakiness, sweating, headache, hunger, nervousness, double or blurry vision, and fast or pounding heartbeat
    • If your blood sugar gets too low, you may faint, have a seizure, or even go into a coma.
  • Glucagonoma – This tumor makes too much of the hormone glucagon and causes too much sugar in the blood, a condition called hyperglycemia.
    • Symptoms: Diabetes (present in most of the patients), inflamed mouth and tongue, and a crusty or scaly skin rash on the face, abdomen, buttocks, or feet – which may be filled with clear fluid or pus

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

Pancreatic adenocarcinoma is a cancerous overgrowth of exocrine pancreatic cells (see above).

Pancreatic cancer is the fourth leading cause of cancer death for both men and women.

  • Pancreatic cancer is one of the most deadly of all types of cancer.
  • This year 44,030 Americans will be diagnosed with pancreatic cancer and about 37,660 will die from it.
  • Despite the high mortality rate, the federal government spends woefully little money on pancreatic cancer research.

Pancreatic cancer is treatable when caught early; the vast majority of cases are not diagnosed until too late.

  • Five-year survival rates approach 40% if the cancers are surgically removed while they are still small and have not spread to the lymph nodes.

Pancreatic cancer is difficult to diagnose

  • There is no reliable screening test for the early detection of pancreatic cancer.
  • Symptoms are often vague and easily confused with other diseases:
    • Dark urine and clay-colored stools
    • Fatigue and weakness
    • Jaundice (a yellow color in the skin, mucus membranes, or eyes)
    • Loss of appetite and weight loss
    • Nausea and vomiting
    • Pain or discomfort in the upper part of the belly or abdomen
    •  Back pain
    • Blood clots
    • Diarrhea
    • Indigestion

Who Has the Greatest Risk?

Inherited genetic disorders cause 5-10% of cases of pancreatic cancer and certain genes can inrease the lifetime risk up 80%.

  • People with two or more relatives who have had pancreatic cancer
  • Cigarette smokers
  • People of Ashkenazi Jewish descent
  • Have the BRCA2, p16, STK11 gene mutation or chronic pancreatitis (inflammation of the pancreas)
  • Are over the age of 50

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.

Michele R. Berman, M.D. was Clinical Director of The Pediatric Center, a private practice on Capitol Hill in Washington, D.C. from 1988-2000, and was named Outstanding Washington Physician by Washingtonian Magazine in 1999. She was a medical internet pioneer having established one of the first medical practice websites in 1997. Dr. Berman also authored a monthly column for Washington Parent Magazine.

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