Lesser-known cancers deserve far more attention
By Nancy Maes
Some cancers, such as breast, colon and lung, are well known. And like principal actors in a movie, they get a great deal of time in the media spotlight. Meanwhile the lesser-known cancers are like the character actors and, therefore, receive far less attention. But these unfamiliar cancers should not be underrated.
Before Joyce Jann was menopausal, she began experiencing symptoms similar to menopause. In particular, she gained about 40 pounds, located mostly in the abdominal area, although her weight had always been stable. And she constantly felt extremely fatigued.
When she went to see her regular obstetrician/gynecologist, she was told her that the weight gain was due to hormonal changes and that she should eat less, do more sit-ups and be more active. When her health didn’t improve, she returned to see her doctor as many as a dozen times asking to be checked again. She even requested a CA125 blood test that would test for ovarian cancer, but the OB/GYN refused because “she didn’t feel it was necessary,” says Jann.
Frustrated, Jann insisted on having multiple ultrasounds, something her doctor reluctantly agreed to. Following one of those ultrasounds, Jann’s suspicions were confirmed, and she was diagnosed with stage-four ovarian cancer. But during surgery, which included a hysterectomy, the doctor determined that she did not, in fact, have ovarian cancer but a rare, slow-growing incurable cancer that had started in her appendix, which was also removed during surgery, called mucinous adenocarcinoma, also known as pseudomyxoma peritonei.
“I learned the importance of listening to my own body and becoming my own advocate,” says Jann, who owns the Inn at Union Pier in Michigan with her husband.
She eventually underwent a second surgery, during which the tumors were debulked and her spleen and the top lining of her stomach were removed. This resulted in many complications.
Through their research, her husband and three daughters found Dr. Armando Sardi, at Mercy Medical Center in Baltimore, a surgeon who specializes in her type of cancer. In 2008, he performed a 13-hour surgery on Jann to repair the damage from the previous surgery and to remove the tumors, her gallbladder, the pyloric part of her stomach, half of her bladder and part of her urinary tract. During the operation, she also underwent a treatment called hyperthermic intraperitoneal chemotherapy (HIPEC), which bathes the inside of the abdominal cavity with heated chemotherapy to remove any microscopic tumors that might remain.
“As far as I know, the longest a person has lived with this cancer is 15 years, and that person had eight surgeries,” says Jann. While her cancer is considered incurable, she is in the wait-and-watch period of the disease.
Sardi, head of the division of surgical oncology at Mercy in Baltimore, says, “The diagnosis of this type of cancer, which spreads to the lining of the abdominal cavity from a primary cancer that can originate in the appendix, the colon, the stomach or the ovaries, is sometimes very difficult. And in women, it is often mis-diagnosed and mistreated as ovarian cancer, so patients often get the wrong operations and get chemotherapy, which doesn’t work very well. [As a result], many people come to us after multiple surgeries and failed chemotherapy treatments. The heat of the HIPEC kills the cancer cells and also enhances the effect of the chemotherapy when given during surgery.”
Sardi has been doing surgery combined with HIPEC since 1994 and, working with his team of specialists in the field, does about 50 to 70 of the procedures a year. He points out that there are about 100 physicians worldwide experienced in this type of surgery combined with HIPEC.
“The sooner a patient comes to us, the better the results because we will be able to protect as many organs as possible. We have patients who have been told they were going to die who are still alive 19 years later,” he says. “The treatment requires a great deal of expertise, so it’s important for patients to ask the doctor how long he has been doing it.” Sardi is currently working on a number of research projects about this type of cancer and its treatment including ways to better diagnose it.
Pancreatic cancer is also on the list of lesser-known cancers and made the headlines when actors Michael Landon and Patrick Swayze died of the disease in 1991 and 2009, respectively, while Apple visionary Steve Jobs succumbed to a rare form of it in 2011. But it deserves more attention beyond when famous people die.
According to the Pancreatic Cancer Action Network, it is the fourth leading cause of cancer deaths, which ranks it before prostate cancer. The American Cancer Society estimated there would be more than 43,000 cases in 2012 and more than 37,000 deaths. Nationally the overall five-year survival rate is 6 percent. The majority of cases (53 percent) are not diagnosed until the cancer has metastasized, and in that case, the five-year survival rate is 1.8 percent.
Dr. Gerard Aranha, a surgical oncologist at Loyola University Medical Center, says it is important to detect the disease early. Screening is recommended for those who have a history of pancreatic cancer in their family and for those who develop diabetes suddenly, which can indicate a problem in the pancreas. Whipple surgery, named for Dr. Allen Whipple who improved the procedure, is a treatment that has successful results.
“Most people think of pancreatic cancer as a death sentence, but the Whipple procedure can be performed on patients in stages one and two of the disease,” says Aranha. The surgeon removes the head of the pancreas, the lower part of the stomach, the bile duct, including the gall bladder, and the first foot of the small intestine, and reconstructs the digestive tract. Aranha has performed more than 400 Whipple procedures with a survival rate of 63 percent after the first year, 35 percent after the third year and 20 percent after 5 years or more.
“There is growing evidence to suggest that in [stage three], which is the stage that most patients present [signs of the disease], giving chemotherapy and radiation therapy before surgery to shrink the tumor will increase overall survival,” Aranha says. Loyola is currently conducting a clinical trial on a vaccine for pancreatic cancer patients that might create antibodies that will prevent the cancer cells from growing. Aranha points out that funding is needed for research to find new drugs to treat the disease.
Cancer of the esophagus, which affects about 16,000 people in the United States each year, is another one that has remained largely in the shadows even though there have been major changes in the treatment of the disease.
“In the last 20–30 years, there has been a shift [in prevalence] from a type called squamous cell carcinoma, [which] is mostly due to alcohol and smoking, to adenocarcinoma, which is found mostly in people who have acid reflux, so we can screen those patients to catch the cancer early and cure it,” says Dr. Marco Patti, professor of surgery and director of the Center for Esophageal Diseases at University of Chicago Medicine (UCM). Patti performs minimally invasive surgery to remove the cancer, a procedure that results in a quicker recovery, a shorter hospital stay and less scarring.
He points out the importance of a specialized medical team. The mortality rate is about 18–20 percent for patients who have the surgery at hospitals that do a small number of the procedures and about 1–2 percent at hospitals that have a specialized esophageal team that does a large number of surgeries. Although funding for research on esophageal cancer is scarce, Patti says that UCM is conducting clinical trials to assess the effects on survival rates of doing chemotherapy and radiation therapy before the surgery.
Jann has gained many insights from her experience in battling cancer. “I had to learn to ask for and accept help,” she says. “It helps to have a good support group including your family, but also people who are trained to provide help that you can go to with your fear, your pain, your feeling of being alone and the extreme depression that sets in when you have a new normal and you’re limited in your lifestyle.”
Jann found support from the PMP Pals Network that put her in touch through the Internet with other people around the world affected by pseudomyxoma peritonei, and she contacted the palliative care center at a local hospital that helped her with pain management, counseling and spiritual care. She also bought herself a puppy, which she finds very therapeutic. And she created a video and a website for all the hospitals in Florida, where she and her husband own a home, to help educate the public about palliative care for patients in all stages of all serious diseases. “Getting involved with other people in order to get the focus off yourself is the best medicine,” she says. Jann’s realizations can be salutary lessons for patients battling any form of cancer.
Much like actors in secondary parts who don’t get star billings but are recognized for the significance of their roles, these lesser-known cancers deserve equal attention so that people can be better informed about symptoms and new developments in diagnosis and treatments. And with more attention will hopefully come additional financial support necessary for research to improve survival rates.
Published in Chicago Health Winter/Spring 2013