Aretha Franklin, the Queen of Soul, with such signature hits as "(You Make Me Feel) Like A Natural Woman," "Respect," and "Chain of Fools," has been recovering in the Detroit area from surgery for what published reports have identified as pancreatic cancerr, one of the most deadly malignancies.
Without more information, it's hard to judge how the diva might fare, because not all pancreatic tumors are alike. In about 90 percent of cases, the term "pancreatic cancer" means adenocarcinoma of the pancreas, a tumor in the duct of the organ that kills more than half of patients within 5 months; fewer than 4 percent are alive after five years, according to American Cancer Society figures. However, when these cancers can be identified and surgically removed before they spread to other organs, doctors do use the word "cure." The five-year survival rate for pancreatic tumors caught early is about 40 percent. A small number of pancreatic cancers are neuroendocrine tumors, which are generally slower-growing and less likely to metastasize; survival can be years.
That Franklin underwent surgery would appear to be a good sign, as surgeons don't operate on pancreatic tumors unless they're confident they can remove them completely. Just 10 percent to 20 percent of the 40,000 pancreatic cancers diagnosed each year are operable, because the pancreas is located near several major blood vessels that feed other organs, said Dr. J. Marc Pipas, director of GI oncology at the Norris Cotton Cancer Center at Dartmouth-Hitchcock Medical Center in Lebanon, N.H. Although the national standard of care has been to operate first on pancreatic tumors, then follow up with chemotherapy and radiation, Dartmouth and M.D. Anderson Cancer Center in Houston have been leaders in pre-treating pancreatic tumors with radiation and chemotherapy to shrink them as much as possible to increase the odds of successful surgery. "An incomplete resection doesn't help you live longer," Pipas said.
Part of the theory of pre-treating the tumor is that when surgery is performed first, "about 1 in 4 patients can never get chemoradiation because they're so beat-up after surgery," he said. The weight loss, poor healing and extreme fatigue often leave them too weak to withstand an extensive operation that removes parts of the pancreas, intestine, bile duct, gallbladder and stomach.
The 68-year-old Franklin, who lives in Bloomfield Hills, Mich., was hospitalized in October at Sinai-Grace Hospital in the Motor City, and was ordered by her doctors to cancel appearances for six months. She has several known risk factors for pancreatic cancer, including being obese, African American, over 55 (the median age in many pancreatic cancer studies is 65) and diabetic, although it's unclear whether she has Type 1 or Type 2 diabetes. (Doctors don't know whether it's the elevated blood sugar levels or the impaired function of the insulin-secreting pancreas itself that accounts for the pancreatic cancer risk among diabetics). Other known risk factors for pancreatic cancer include smoking, which doubles or triples risk; family history; chronic inflammation of the pancreas, cirrhosis of the liver and workplace exposures to pesticides, dyes and chemicals. About 10 percent of cases are linked to genetic mutations associated with certain cancers of the breast and ovaries, skin, and some colorectal cancers. Researchers also suspect there may be associations with ulcer-causing Helicobacter pylori infections and high-fat diets.
Ranked No. 1 on Rolling Stone magazine's list of the 100 greatest singers of all time, Franklin has kept her illness largely private, although a cousin reportedly told the National Enquirer that she was suffering from pancreatic cancer, which in 2009 claimed the life of actor Patrick Swayze. However, other high-profile patients, like Supreme Court Justice Ruth Bader Ginsburg and Apple CEO Steve Jobs, have survived.
Pancreatic cancer occurs in an oblong organ about 6 inches long lying deep in the abdomen, behind the stomach, small intestine, liver, and bile ducts. It contains the islet cells that secrete the important hormone insulin, which breaks down sugar to fuel the brain and body. The pancreas contains other cells that release enzymes that digest food.
Pancreatic cancer is the No. 4 cause of cancer in this country. In 2010, more than 43,000 people will be diagnosed with it and more than 36,000 will die from it, the American Cancer Society estimates. It is one of the most dreaded cancers because by the time it's found, it's most often advanced. Plus, there are no reliable ways to screen for it.
Some blood tests can detect chemicals made by pancreatic tumor cells, such as CA 19-9, but the tests have limited value and largely are used to help doctors determine if treatment is working, or to detect recurrence. Pipas and his colleagues at Dartmouth have submitted for publication a study that looked at CA 19-9 levels in close relatives of pancreatic cancer patients, and sent those family members with high levels for endoscopic ultrasound exams to look for tumors. Pipas said the findings were "not conclusive, but suggestive." He said endoscopic ultrasound has allowed Dartmouth cancer specialists to pick up tumors when they are just 10 to 15 millimeters in size.
Tests for another protein, carcinoembryonic antigen (CEA) can be used to identify advanced pancreatic cancer.
Further contributing to low rates of early diagnosis is that symptoms of pancreatic cancer tend to be vague, such as pain in the back or abdomen, unexplained weight loss or loss of appetite and the inability to digest fatty foods. A more specific symptom is jaundice, the yellowing of the skin and whites of the eyes when bilirubin, which is made in the liver, builds up in the blood because a pancreatic tumor is blocking the bile duct and keeping bilirubin from moving into the intestines.
According to the National Cancer Institute, the chance of recovering from pancreatic cancer depends on whether the tumor can be surgically removed; its size; whether it has spread beyond the pancreas; and whether it's a first-time diagnosis or a recurrence. Once the cancer has spread to other organs, it's no longer considered curable, and doctors use a variety of approaches to control symptoms and complications.
At Dartmouth, doctors are treating pancreatic cancer with five-day-a-week external beam radiation called intensity-modulated radiotherapy (IMRT) and twice-weekly low doses of the chemotherapy drug gemcitabine (Gemzar). "Gemcitabine for years was the standard of care in advanced disease," Pipas said. Doctors then discovered that it powerfully sensitizes cancer cells to the effects of radiation, preventing the cells from repairing themselves after sustaining radiation damage, so they began using lower doses in concert with radiation for six weeks. So far, in studies of more than 100 patients, "what we have shown is we can downsize many of these tumors, and we can downstage them, meaning we can pull them off vessels. The majority of people we treat in this fashion are able to go on and get a surgery."
Researchers are making inroads with targeted therapies and vaccines, too. Some of the targeted therapies inhibit certain growth factors, including those that encourage the sprouting of new blood vessels that nourish tumors. Researchers at Dartmouth and Stanford universities are collaborating on studies of a new drug called an anti-connective tissue growth factor antibody. This targets some of the connective tissue around tumor cells, and when given together with Tarceva, which inhibits epidermal growth factor receptor, and the chemo drug gemcitabine, hits tumors with a triple punch.
At the Johns Hopkins Kimmel Cancer Center in Baltimore, researchers have been fine-tuning and developing vaccines to stimulate patients' own immune systems to mount attacks on pancreatic tumor cells in newly diagnosed patients as well as those with advanced disease.
At the American Society for Clinical Oncology meeting last summer, French researchers presented results of a randomized clinical trial in which Folfirinox, a combination of four chemotherapy drugs, improved survival of patients with metastatic pancreatic cancer. It yielded a median survival of 11 months compared with about 7 months with gemcitabine. At the time, Dr. Andrew Ko, writing in the online publication HemOnc Today, said the findings "may be practice-changing in terms of establishing a new chemotherapy regimen as front-line treatment of metastatic pancreatic cancer." He called the Phase III study "the most positive …we have ever seen in advanced pancreatic cancer" with results "too striking to ignore."