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What type of cancer has plummeted in African-Americans, increased in whites, and, in only two generations, has become the fastest-rising cancer in the United States?
Few people would guess it is cancer of the esophagus – the foot-long, muscular tube that channels food from the mouth to the stomach. The disease is relatively rare, and low in the public's awareness, but it is often deadly, and its incidence is rising at the rate of 1 percent a year.
"It has not been a glamorous disease getting a lot of research dollars, because traditionally it affects people with less socioeconomic power and has had a grim outlook," says Peter Enzinger, MD, clinical director of the Gastrointestinal Oncology Center, who sees about 80 percent of the 120 new esophageal cases a year at Dana-Farber/Brigham and Women's Cancer Center (DF/BWCC).
Esophageal cancer has undergone a rapid evolution in the past 40 years, and the changes continue. Previously it was a disease found largely among the poor and disproportionately in African-Americans, and was linked to a lifestyle of heavy smoking and drinking. Survival rates were extremely low.
Today, incidence among African-Americans has dipped dramatically, while it has risen steadily in white males. The role of smoking and drinking is much smaller.
Now, the most important risk factors are obesity and the presence of gastroesophageal reflux disease (GERD) – irritation of the lower esophagus by acid stomach contents flowing backwards.
Even the biology of the tumors has turned upside down.
"When I started my training, almost all the esophageal cancer we saw was squamous cell carcinoma," observes Charles Fuchs, MD, director of DF/BWCC's gastrointestinal oncology disease center.
"Now we see very little of it – it's becoming a rare disease. At the same time, there's been an extraordinary rise in adenocarcinomas of the esophagus." In fact, deaths from this type of esophageal cancer rose from 2.5 to 20 per million between 1975 and 2005, according to a Dartmouth Medical School report.
But with steady gains in surgical techniques and post-surgical care, and more effective chemotherapy and radiation, "over the past 20 to 30 years, there has been a statistically significant improvement in survival for this disease," Enzinger says.
Both the old attitudes and the new trends are reflected in an episode of esophageal cancer that began for Irena Cade, of Amherst, Mass., in 2004, when she was 64.
Cade, a retired Hampshire College employee, was diagnosed some six months after she first went to see her doctor because of persistent trouble and pain in swallowing. The physician initially treated her for reflux disease with acid-blocking drugs, then antibiotics, but her symptoms didn't improve, and she continued to lose weight.
Finally an endoscopy – an examination of the inside of the esophagus with a thin, lighted tube – revealed the tumor.
The news left her stunned. "I started putting my things in order," she says. "I was sure I was going to die."
Her local doctor didn't hold out much hope, either. Apart from experienced teams of gastrointestinal cancer specialists at centers like DF/BWCC, many physicians don't realize that an esophageal cancer diagnosis is not a death sentence.
Even the most up-to-date treatments can be rough on patients, "but I had total trust in my doctors," says Cade, who was cared for at DF/BWCC with a team led by Enzinger.
Five years after surgery, radiation, and chemotherapy, Cade says, "I feel perfectly normal, and Dr. Enzinger says the cancer isn't likely to recur."
Cancer of the esophagus is a stealthy disease with few symptoms in its earliest, most curable stages. When symptoms do appear – those experienced by Cade are textbook warning signs – they may be initially ignored or deemed unlikely to be caused by cancer.
By then, the tumor will often have penetrated deep into the wall of the esophagus and in some cases will have spread to the network of bean-sized lymph nodes in the chest and neck, from where it is prone to start metastasizing to other organs.
As the link with acid reflux disease has become clearer, patients with severe, longstanding reflux symptoms are increasingly advised to have an endoscopy. However, most people with acid reflux won't develop esophageal cancer, and routine endoscopies aren't considered cost-effective because the disease is so uncommon.
Occasionally, an esophageal tumor may be discovered during follow-up of a patient with another type of cancer.
For example, J. Banks Hyde, a Washington, D.C., businessman who was diagnosed and treated for tongue cancer in 2007, had a routine scan in January 2009 that he says "lit up" where a tumor had developed in his esophagus.
Banks was successfully treated by surgeon Scott Swanson, MD, at Dana-Farber/Brigham and Women's Cancer Center (DF/BWCC).
He says that, in a sense, his bout with tongue cancer "saved my life, because it led to finding the cancer in my esophagus."
When worsening symptoms bring a patient to the doctor's office, the first diagnostic test is an X-ray, conducted while the patient swallows a barium contrast solution which can detect even small, early cancers in the esophagus. Additional tests are carried out with endoscopy, ultrasound, and CT scans to further pinpoint the tumor and determine whether cancer cells have spread to the lymph nodes and nearby organs.
Treatment of esophageal cancer is a highly interdisciplinary effort involving surgeons, radiation experts, and chemotherapy specialists working in close consultation, says Harvey Mamon, MD, PhD, clinical director of Radiation Oncology at DF/BWCC.
"If there is minimal invasion of the tumor into the esophageal wall, and no lymph nodes are involved, the patient may go straight to surgery," Mamon explains.
Surgery alone may be sufficient for very early, shallow tumors within the esophagus, and has good success rates. At the other extreme, if scans show that the cancer has spread to the patient's liver, lungs, or bone, it is labeled Stage 4 and the treatment of these metastatic cases will likely be limited to palliative therapy to make the patient more comfortable.
In less-advanced Stage 2 and 3 cancers, the tumor penetrates into but not through the esophagus' muscular wall, with cancer cells found in lymph nodes but no other organs. Here, the usual approach is a combined attack with radiation, drugs, and surgery.
A common regimen, says Enzinger, is a course of 28 daily radiation treatments carried out over 5½ weeks, with simultaneous weekly chemotherapy infusions to shrink the tumor. The patient then has a break of one or two months before surgery (Enzinger helped develop a widely used chemo combination for esophageal cancer).
Surgery is appropriate in about 30 percent of esophageal cancer cases, says Swanson, leader of DF/BWCC's Lowe Center for Thoracic Oncology.
Surgeons at DF/BWCC, led by Raphael Bueno, MD, carry out more than 100 operations for esophageal cancer a year, he says – the highest volume in New England and one of the highest nationwide.
Minimally invasive "keyhole" surgery can be substituted for the more traumatic "open" operation in some patients. Instead of large cuts, surgeons make a number of smaller incisions.
Swanson and Jon Wee, MD, a minimally invasive thoracic surgery specialist at DF/BWCC, say that in experienced hands, the keyhole procedure enables patients to recover more quickly at home and get back to full activity in a much shorter period compared to the traditional "open" operation.
Banks, the Washington businessman, is grateful that he was able to have the keyhole procedure done by Swanson and Wee in April 2009.
"I was in pain only for the first couple of days," Banks said in July. "Now I am back to playing golf two to three times a week – and playing well."
While esophageal cancer remains a daunting foe, researchers have grounds for optimism. Although no screening test is on the horizon, Fuchs is hopeful that current studies may lead to identification of high-risk groups.
"If we can understand the risk factors and the genetics of esophageal cancer better, we might be able to ascertain whom we should send for an upper endoscopy and follow them at regular intervals," he says.
Swanson is encouraging, as well, noting that combination therapy is increasing cure and survival rates.
"And we're doing it with less disruption of people's lives," he adds. "We need to remain very proactive with this disease now that we have more effective diagnostic and treatment options. The earlier we detect the problem, the more opportunity we have for an outstanding outcome."
Fall/Winter 2009 Table of Contents
In this video, Ed shares his experience with esophageal cancer.