1 in 20 million
Fewer than 250 cases of glucagonoma have been identified worldwide since 1942, and the American Association of Endocrine Surgeons estimates that about one in 20 million people annually will develop it. The tumor has no known cause, but in some cases a family history of cancer may be involved. (After his diagnosis, Williams discovered that his grandmother died of pancreatic cancer at age 68, although its specific type is not known.) Glucagonomas result from the overproduction of the hormone glucagon; this excess disrupts the production of insulin, which regulates blood sugar. About 70 percent of glucagonoma patients have NME, and many also lose weight, as did Williams, who had thought his gradual 30-pound weight loss was caused by the stress of dealing with the rash.
Surgery to remove the tumor is the preferred treatment because glucagonomas do not respond well to chemotherapy. Because such cancers, which grow slowly, tend to be detected only after they have spread beyond the pancreas, surgery cures only about 20 percent of such patients. Poligone said that he and his colleagues were pessimistic; Williams had had symptoms for more than six years, and they thought it was highly likely that his cancer had spread.
Poligone said he lined up an oncologist and a surgeon and then broke the news to Williams, who did not understand the gravity of his diagnosis at first.
“I really didn’t relate ‘tumor’ with ‘cancer’ until they told me I was going to be treated at the cancer center,” he recalled.
On Jan. 22, 2011, Williams underwent surgery. The results were the best anyone could have hoped for: The cancer had not spread to his lymph nodes or to his liver, and doctors believed they removed it all. Four weeks later, his rash was gone; subsequent tests have found no sign of cancer.
“We were very surprised it was contained,” Poligone recalled, adding that doctors believe his surgery was curative. Poligone said that a dermatopathologist who went back and examined all of Williams’s biopsy slides did not find specific evidence of NME. Other than the rash and weight loss, Williams had none of the other classic symptoms that might have prodded doctors earlier to suspect pancreatic cancer, nor did he seem seriously ill.
Instead, he seemed mostly eager to be rid of the rash. “He was, like, ‘You’re cutting into my golf time and work time, get me back,’ ” Poligone recalled, adding “I take the most solace that [his case] had an excellent outcome.”
Williams said that he remembers feeling increasingly tired but not really sick. Only in retrospect, he said, did he realize “how weak I’d become,” adding, “I feel like a new person.”
Sometimes he ponders the six years he spent seeing doctor after doctor about his miserable rash. “You think, ‘Geez, why didn’t they run this test two years earlier?’
“But then you think, ‘Hey, they figured it out.’ ”
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