In September 2005, my husband William was diagnosed with adenocarcinoma of the esophagogastric junction (AEG). Less than 10 months later, he died from surgical complications. William had a longstanding history of acid reflux and Barrett’s esophagus (a complication of gastroesophageal reflux disease), which we thought was well controlled. After his death, when I began more in-depth research about this silent killer, I recognized the need for greater public and healthcare-professional awareness of this devastating disease. Consumer education, media attention, and researchers have focused extensively on breast cancer in women, while AEG in white American males gets little attention.
Early diagnosis may bring a more positive outcome, but postoperative quality of life declines severely. For instance, eating—for most people, a pleasurable and social function—becomes difficult due to swallowing problems and smaller gastric capacity. Patients have trouble maintaining optimal nutrition.
Esophageal cancer is three to four times more prevalent in men than women. Squamous-cell carcinoma is the most common esophageal cancer in African-American males, whereas adenocarcinoma is more common in white males.
Esophageal cancer incidence is growing at an alarming rate. In the western world, AEG is the most rapidly increasing cancer. In white American males, its incidence is climbing faster than that of any other cancer. Since the mid-1970s, it has risen steadily by 5% to 10% per year.
According to the American Cancer Society (ACS), esophageal cancer kills approximately 14,000 Americans each year. Worldwide, it’s is the sixth leading cause of death, with 400,000 cases diagnosed annually. Esophageal cancers cause more deaths globally than breast, lung, or colorectal cancer. AEG has a mortality rate of 87%.
Survival rates for esophageal cancer have improved since the 1950s but remain low. Today, only about 17% of whites and 12% of African-Americans survive at least 5 years after diagnosis; these rates apply to all disease stages.
Early diagnosis may lead to higher survival rates but doesn’t guarantee longer survival. Survival depends on an individualized therapeutic approach based on tumor type and staging results, along with the extent and type of treatment complications. Complications stemming from leaks at the anastomotic site almost always are fatal.
Research shows that reflux symptoms and Barrett’s esophagus relate closely to an increased adenocarcinoma risk. Most esophageal adenocarcinomas arising in the lower third of the esophagus result from preexisting Barrett’s esophagus.
Typically, AEG progresses as shown in the box below:
Adenocarcinoma incidence in patients with Barrett’s esophagus is thought to be less than 5% or 10%. But this is just an estimate; the true incidence of Barrett’s esophagus is unknown.
Surgical resection is the optimal approach in patients with early resectable AEG who have no evidence of metatastic disease and are physically fit enough to undergo the difficult surgery. The ideal approach is complete resection of the tumor and its lymphatics to provide the best potential for long-term survival.
Preoperative staging is extremely important in determining appropriate treatment. Endoscopy with biopsy is the mainstay of diagnosis. Modalities used to assess disease extent are spiral computed tomography (CT); endoscopic ultrasound (EUS), including EUS fine-needle aspiration; and positron emission tomography. The patient’s physical fitness and expected ability to withstand surgery also must be considered.
Generally, AEG is treated as follows:
- Stage I tumors with no metastasis warrant limited resection of the proximal stomach and distal esophagus.
- In more advanced but resectable stage I tumors, the tumor and its lymphatics are removed completely.
- In Stage II or III tumors, total gastrectomy with trans-hiatal resection of the distal esophagus may be done, along with en-bloc removal of the lymphatic drainage system.
- For locally advanced tumors when complete surgical removal is questionable, multimodal treatment trials should be considered. Optimal treatment is controversial. Chemoradiation or surgery alone are acceptable standards of care, with treatment selection based on the patient’s tumor stage and comorbidities.
- Metastatic or unresectable disease is incurable. Chemotherapy is palliative, with the goal of improving quality of life and easing dysphagia in about 60% to 80% of patients. Median overall survival is 4 to 8 months. Chemotherapy doesn’t yield a survival benefit in advanced disease, although it does in metastatic gastric cancer. Neoadjuvant and adjuvant multimodalities and definitive chemoradiotherapy are promising but need further investigation.
Surgical approaches and complications
Surgical approaches include the left thoraco-abdominal, Ivor Lewis, transhiatal, right thoracotomy/transhiatal and retrosternal approaches. Regardless of the technique used, esophagogastric resection is difficult and fraught with complications. In many cases, comorbidities complicate the postoperative course.
One of the newest surgical modalities for AEG is the minimally invasive DaVinci Robotic technique. Data suggest it significantly reduces recovery time, pain, trauma, and infection risks. Yet it’s no panacea. Critical factors for good patient outcomes include the surgeon’s experience using the robot for complex surgeries, critical-care nurses’ experience caring for patients who’ve undergone robotic surgery, anesthesia care, and hospital volume in handling complicated cases. Little data exist on the costs and benefits of robotic surgery for this type AEG cancer compared to conventional techniques.
In elderly patients undergoing complex surgeries such as esophagogastrectomy and pancreatectomy, mortality relates directly to how many such procedures are done at the hospital in question. Generally, mortality is lower when complex procedures are performed at high-volume hospitals and the patient receives care from an experienced surgical team. Between 1988 and 1998, mortality rates for esophageal resection in low-volume centers ranged from 9.2% to 20.3%; high-volume centers had lower mortality rates, ranging from 2.5% to 8.4%. Such organizations as the LeapFrog Group (a patient-safety program) recommend that esophageal resection be performed only at high-volume centers—those with least 20 resections per year.
Efforts to enhance surgical outcomes typically focus on improving preoperative patient selection and reducing postoperative complications. Only a few studies have examined how complications associated with surgical technique relate to outcomes in cancer patients who’ve had esophagogastrectomies.
One of the most serious complications of esophagogastric resection is an anastomotic leak—dehiscence at the stomach-esophagus anastomosis. This complication leads to sepsis, mediastinitis, empyema, and multiple organ failure and increases mortality exponentially. Incidence of leaks varies widely; a 2011 study showed an incidence of 10% to 20%. (My husband died from an anastomotic leak 9 months after robotic surgery.)
Research shows anastomotic leaks relate directly to surgical technique. About 78% of technical complications included a leak directly attributable to surgical technique. One study of cancer patients who’d undergone esophagogastric resection found 147 complications related to surgical technique in 138 patients; 21% (106) of patients experienced anastomotic leaks. They had poorer outcomes and a substantially lower survival rate.
Preoperatively, teach patients who will undergo robotic surgery about the postoperative course, including expected pain level, permissible activity level, possible complications, and surgical-site care. To the extent possible, answer their questions about the robotic procedure and surgical equipment. However, be aware that little patient-education material is available other than generic resources from manufacturers.
Explain that the robotic system is not a preprogrammed medical device that performs on its own. Reinforce the surgeon’s explanation of why he or she believes the robotic technique will benefit the patient—for example, promoting a shorter hospital stay, reducing pain, and allowing a faster return to normal activities. Tailor your teaching to each patient.
Postoperatively, the same standards of care apply whether the patient had a robotic procedure or a traditional open procedure. Patients who’ve had the robotic procedure typically stay in the hospital for approximately 6 days; those who’ve had a traditional open procedure usually stay 10 to 14 days.
Inform the patient that before discharge, a gastrografin swallow test is done to rule out an anastomotic leak. If no leak is found, the patient will be started on a clear liquid and advanced to soft foods as tolerated. If a leak is suspected, jejunostomy tube feedings may be started.
Activity levels are based on how the patient feels. Caution patients against heavy lifting or driving until the first postoperative visit (usually a week after discharge). Advise patients and family members to monitor the incision site and report drainage, and to keep the dressing dry and intact.
Healthcare professionals need to raise public awareness of AEG, especially among men, to encourage early diagnosis and treatment of upper GI symptoms and help guide selection of treatment options. Early diagnosis is crucial. Equally important, if surgery is warranted, it should be done in a high-volume facility to optimize the chance for recovery and quality of life.
Valera A. Hascup is an assistant professor of nursing at Kean University in Union, New Jersey and a nurse researcher at Somerset Medical Center in Somerville, New Jersey.
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I am the author of the article that was written in 2011 after my husband’s death for to this terrible disease. As a professor and RN, I wrote the article out of a sense of frustration with the poor medical care, and as one who devoted her entire career caring for patients, I as an PhD educated RN could not navigate the system. I felt powerless, and in a hospital in which I spent the majority of my nursing career. I was surprised to learn there are recent comments to the article. Thank you so much for sharing your comments and stories.
What I learned in retrospect, was that you must be in a specific cancer medical center to get the best care. In my area it would be Sloan Kettering in NYC. You need a high volume center that has cutting edge techniques and care modalities, care with outstanding surgeons and the entire healthcare team. That is the key to the best chance for positive outcomes and that is well documented in the research literature.
One of the comments questioned on what can
be done to prevent this disease. There are no guarantees for prevention. Barrett’s esophagus is thought to be a precursor to EG carcinoma, so being diligent in treating Barrett’s, or acid reflux, if one has that diagnosis, is of critical importance. The rate of EG adenocarcinoma has continued to rise over the last several decades, especially in Caucasian males. Alcohol, smoking, Barrett’s esophagus are all thought to be contributing factors, but the specific etiology is not known. There are so many variables. What is known that adjuvant therapy of chemo & radiation plus surgery appears to provide the best chance of a positive outcome. However survival rate percentages vary and are generally low.
Thank you again for your comments.
My 38 yo son has adenocarcinoma of the esophaegal junction. His doctor did not order EUS, though he did order CT and PET. As such, we do not know the specific stage. Based on test results, his cancer is “locally invasive”, well differentiated and has not spread to lymph nodes or any areas or organs outside the esophagus. Before he was told he had cancer, his gastroenterologist, after one failed attempt, successfully inserted a metal stint to allow food to pass through.
After 6 weeks of radiation therapy, the oncologist plans to do long term chemotherapy rather than refer for surgery. But for his weakened physical state from weight loss and side effects of radiation and chemo, all indications are that surgery is the next step in his treatment. However I have been unsuccessful in locating a high volume esophageal resection facility or surgeon. Apparently this I formation is not readily available to the public. We live in southern WV and understand that we would have to travel to somewhere like Pittsburgh or Cleveland for surgery. Do you have more recent information about success and side effects of esophageal juncture resection and any information about where this surgery could be performed? At 38 years old, my son should be provided the opportunity to be evaluated for surgery and informed of the outcomes should he decide to go that route.
I know I sound like I’m grasping at straws. However the only resource for him is an elderly oncologist in a declining coal mining community. Surely there is more recent and modern treatment or improvements in treatment. Information I find online is dated 2005 or earlier. Any information from the author or others would be greatly appreciated. You have my approval to publish my email address..trishcgm at gmail dot com.
Interesting, but how do we prevent the disease rather then find it at a later stage? I’ve just had an endoscopy, and have muiltiiple grade A to Grade IV compatible with gastroesophageal reflux..but no real symptoms of reflux, occasional pain in my left should after dinner and occasional difficulty swallowing.
My father was diagnosed with Stage 4 Adenocarcinoma of the GE Junction on Feb 8th, 2019. He is 75. He also did not have any of the typical risk factors. He has never smoked, used alcohol, and has never been overweight. He did have acid reflex, which is pretty common. Starting Chemo and hoping for the best.
Thank you for writing this article. This is a terrible disease. In 10/2017 we lost my brother to Adenocarcinoma of the GE Junction. He was diagnosed late, even though he had already been seeing a gastroenterologist for a few years. After multiple biopsies and inaccurate results, it then got to the point where he went 14 days without food and was choking on his own saliva before he went to another facility for a second opinion. They handed him the diagnosis within 24 hours….he passed away 8 months later at the age of 38. He had none of the typical risk factors, and there were no genetic markers for him to be at increased risk…it was just the luck of the draw.
I believe there is a growing incidence in the under 50 age group, which is concerning because this disease typically affects those in the 60+ age group.