I. What every physician needs to know.
Esophageal cancer is a relatively rare cancer. It is most often diagnosed in people over the age of 65. The majority of cancers are either adenocarcinoma or squamous cell carcinoma. Five-year survival for patients with esophageal cancer has improved from 5% to 17% over the past 30 years. Unfortunately almost one third of cases are identified when the cancer has already metastasized and for this subset of patients survival is less than 5%.
II. Diagnostic Confirmation: Are you sure your patient has Esophageal Cancer?
Endoscopic evaluation must be done in order to confirm the diagnosis. Endoscopic examination may reveal a mass, nodule or an ulcer. In order to confirm the diagnosis multiple biopsies (at least 7) need to be taken of the suspicious lesion.
A. History Part I: Pattern Recognition:
Early esophageal cancers are often asymptomatic. Cancers may be discovered incidentally when patients are being evaluated for occult bleeding or iron deficiency anemia. For those patients with symptoms, dysphagia is the most common (74%) followed by weight loss (57%), heart burn (20%) and odynophagia (16%). The dysphagia will often present slowly with patients first noting difficulty swallowing solids and then eventually liquids.
B. History Part 2: Prevalence:
The majority of patients with esophageal cancer (over 90%) have either adenocarcinoma or squamous cell carcinoma. Over the past 35 years the histology of esophageal cancer has changed. In the 1970’s patients were diagnosed primarily with squamous cell carcinoma; adenocarcinoma is now the predominant histology, particularly in white and hispanic Americans. Squamous cell carcinoma remains the predominant histology in black, asian and pacific island Americans, although adenocarcinoma is increasing in the black population.
Adenocarcinoma of the esophagus affects the distal esophagus and the gastroesophageal junction. Barrett’s esophagus is believed to be the precursor lesion for adenocarcinoma of the esophagus. Factors associated with Barrett’s esophagus include advanced age, male sex, white race, gastroesophageal reflux disease, and hiatal hernia. Increased waist to hip ratio also places people at greater risk for developing Barrett’s esophagus; thus explaining the increasing incidence of adenocarcinoma in western countries challenged by obesity.
Squamous cell carcinoma is found in the upper two thirds of the esophagus. Heavy alcohol use (>35 drinks per week) is the predominate risk factor for squamous cell carcinoma in the United States. Other factors associated with squamous cell carcinoma include tobacco use, achalasia, tylosis (a rare genetic disorder associated with hyperkeratosis of the palms and soles), caustic injury (most commonly lye ingestion), poverty, and nutritional deficiencies (including Plummer-Vinson syndrome).
C. History Part 3: Competing diagnoses that can mimic Esophageal Cancer.
Malignancies of the esophagus most often present as progressive dysphagia (solids first, then liquids) and can therefore be confused with any disease associated with dysphagia. Other diseases that can present with dysphagia include gastroesophageal reflux disease, esophagitis (eosinophilic esophagitis, candidiasis), Schatzki’s ring, achalasia, diffuse esophageal spasm, and muscle skeletal disorders (such as myasthenia gravis and scleroderma). The patient’s past medical history, ethnicity and concurrent symptoms will help to guide management for work-up of dysphagia.
D. Physical Examination Findings.
There are no pathognomonic findings of esophageal cancer on physical exam. The most common finding will be signs of weight loss. Patients with advanced disease may present with pleural effusions, an enlarged liver and/or lymphadenopathy (specifically supraclavicular nodes which depending on the antomic area of the cancer can either be on the left “Virchow’s node” or on the right).
E. What diagnostic tests should be performed?
While barium studies may suggest the presence of esophageal cancer, the diagnosis must be made endoscopically. Patients with red flag symptoms and suspicious for having esophageal cancer may proceed directly to endoscopic evaluation for visualization of the esophagus and tissue sampling.
1. What laboratory studies (if any) should be ordered to help establish the diagnosis? How should the results be interpreted?
There are currently no commercially available tumor markers specific to esophageal cancer. Therefore laboratory tests should be focused on standard of care patient management. Patients who are older or who have risk factors for kidney disease, will need to have a BUN and creatinine prior to administration of IV contrast. Liver function tests may help to determine if there is metastatic disease. Coagulation labs will need to be obtained prior to endoscopic evaluation (for both upper endoscopy and endoscopic ultrasound).
2. What imaging studies (if any) should be ordered to help establish the diagnosis? How should the results be interpreted?
After biopsies have established a diagnosis of esophageal cancer, an IV contrast computed tomography (CT) scan of the chest, abdomen and pelvis will help to determine if there are any distant metastasis. The identification of metastasis will guide future therapy. Endoscopic ultrasound (EUS) is indicated if no distant metastasis are identified. EUS can evaluate for depth of invasion as well as performing fine needle aspiration of regional lymph nodes. Positron emission tomography may be more sensitive for identifying distant metastasis and can be considered if EUS and CT scans are negative.
If endoscopic mucosal resection is available, then this should be performed for early cancers. This may either be curative and can help further stage the cancer. If this is not available then surgical resection will need to be performed.
For cancer involving the upper two thirds of the esophagus (above the carina), patients should undergo bronchoscopy to identify involvement of the trachea.
F. Over-utilized or “wasted” diagnostic tests associated with this diagnosis.
Upper GI series (“Barium Swallow”) are often ordered but are of limited value. In order to definitively diagnose esophageal cancer, tissue biopsies must be obtained endoscopically.
III. Default Management.
Patients presenting with dysphagia or other symptoms or imaging that indicate a possible esophageal malignancy need to have an upper endoscopy with multiple biopsies of the suspicious lesion. Once esophageal carcinoma has been established patients need to have an IV contrast CT scan of the chest, abdomen, and pelvis. Further work-up will need to be determined with consultation of gastroenterologists, oncologists and surgeons. Depending on the expertise of the specialists various options will need to be evaluated including endoscopic mucosal resection, endoscopic ultrasound, esophageal resection and staging laparotomies. The most accurate measurement of determining depth of invasion at this time is with endoscopic ultrasound.
A. Immediate management.
Determination of surgical candidates versus nonsurgical candidates with endoscopic ultrasound and staging is the first step in immediate management.
B. Physical Examination Tips to Guide Management.
There are no physical exam findings specific for esophagael carcinoma.
C. Laboratory Tests to Monitor Response To, and Adjustments in, Management.
D. Long-term management.
Esophageal cancer is ideally managed with a multidisciplinary team of physicians including an oncologist, thoracic surgeon and a gastroenterologist with advanced endoscopic capabilities.
E. Common Pitfalls and Side-Effects of Management.
IV. Management with Co-Morbidities.
A. Renal Insufficiency.
B. Liver Insufficiency.
C. Systolic and Diastolic Heart Failure.
D. Coronary Artery Disease or Peripheral Vascular Disease.
E. Diabetes or other Endocrine issues.
G. Immunosuppression (HIV, chronic steroids, etc).
H. Primary Lung Disease (COPD, Asthma, ILD).
I. Gastrointestinal or Nutrition Issues.
Depending on the size of the lesion, patients may require stenting of the esophagus for palliative treatment of obstructing lesions. The vast majority of patients who receive stents will have improvement in dysphagia. However, complications are frequent and can range from minor discomfort to life threatening. Stents placed in the upper esophagus may cause a globus sensation or airway compromise. Distally placed stents may cause reflux and patients may require proton pump inhibitors. Distally placed stents are also at risk for food impaction. Stents placed after chemoradiation may have an increased risk of complications including the development of esophagea-aortic fistulas.
Patients often require gastrostomy or jejunostomy tube placement for enteral feeding.
J. Hematologic or Coagulation Issues.
K. Dementia or Psychiatric Illness/Treatment.
V. Transitions of Care.
A. Sign-out considerations While Hospitalized.
Patients who have undergone an endoscopic mucosal resection for early stage esophageal cancer are at risk for perforation. If the patient complains of chest pain, neck pain, shortness of breath or appears compromised in anyway (low blood pressure, rapid heart rate, fever) then a perforation needs to be ruled out. Physical exam may reveal crepitus along the anterior chest wall. An upright chest x-ray may reveal free air, a collapsed lung or fluid around the lung. If the chest x-ray is negative, but suspicion for perforation is high, then a non-contrast CT scan of the chest should be ordered. If a perforation is identified then surgery should be contacted for further management.
B. Anticipated Length of Stay.
Length of stay for patients with esophageal cancer is highly variable. Patients may only be hospitalized for one night if admitted for an endoscopic mucosal resection or admission can last several days because of nutrition issues, pain management and long term goals of care.
C. When is the Patient Ready for Discharge.
For all stages of esophageal cancer, patient’s pain will need to be adequately controlled, and mode of nutrition established (total parental nutrition versus esophageal stent placement versus oral intake).
D. Arranging for Clinic Follow-up.
1. When should clinic follow up be arranged and with whom.
Patients should have follow-up with an oncologist within 1-2 weeks after discharge. If the patient has localized disease they should be evaluated by either a gastroenterologist capable of endoscopic ultrasound or by a thoracic surgeon. If the patient has metastatic disease then consideration should be given to offering palliative care.
2. What tests should be conducted prior to discharge to enable best clinic first visit.
A CT scan with IV contrast of the chest, abdomen and pelvis to start staging the disease.
3. What tests should be ordered as an outpatient prior to, or on the day of, the clinic visit.
E. Placement Considerations.
Patients with distant metastasis have a poor prognosis and depending on the patient’s wishes, current physical status, and comorbidities, hospice care should be considered.
F. Prognosis and Patient Counseling.
See Table I.
Resection is essentially precluded by any of the following:
Distant metastasis (lung, peritoneum, brain, liver, extraregional lymph nodes)
Of note, celical lymph node disease is now considered regional disease and does not preclude one from resection
Presence of tracheal esophageal fistula
Involvement of aorta, trachea, heart
Local invasion of cervical esophageal carcinoma
For those with unresectable disease, external beam radiation with chemotherapy is standard of care. However, there is no consistent evidence for improved survival with chemoradiation versus radiation alone. Five year survival for unresectable disease varies greatly but is consistently < 50%.
VI. Patient Safety and Quality Measures.
A. Core Indicator Standards and Documentation.
B. Appropriate Prophylaxis and Other Measures to Prevent Readmission.
VII. What's the evidence?
Howlader, N, Noone, AM, Krapcho, M, Neyman, N, Aminou, R, Waldron, W. “SEER Cancer Statistics Review, 1975-2008”.
Longo, D L, Fauci, AS, Kasper, DL, Hauser, SL, Jameson, J, Loscalzo, J, Longo, DL, Fauci, AS, Kasper, DL, Hauser, SL, Jameson, J, Loscalzo, J. “Tumors of the Gastrointestinal Tract”. . 2013.
Westerterp, M, Koppert, LB, Buskens, CJ. “Outcome of surgical treatment for early adenocarcinoma of the esophagus or gastro-esophageal junction”. Virchows Arch. vol. 446. 2005. pp. 497
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- Esophageal Cancer
- I. What every physician needs to know.
- II. Diagnostic Confirmation: Are you sure your patient has Esophageal Cancer?
- A. History Part I: Pattern Recognition:
- B. History Part 2: Prevalence:
- C. History Part 3: Competing diagnoses that can mimic Esophageal Cancer.
- D. Physical Examination Findings.
- E. What diagnostic tests should be performed?
- 1. What laboratory studies (if any) should be ordered to help establish the diagnosis? How should the results be interpreted?
- 2. What imaging studies (if any) should be ordered to help establish the diagnosis? How should the results be interpreted?
- F. Over-utilized or “wasted” diagnostic tests associated with this diagnosis.
- III. Default Management.
- A. Immediate management.
- B. Physical Examination Tips to Guide Management.
- C. Laboratory Tests to Monitor Response To, and Adjustments in, Management.
- D. Long-term management.
- E. Common Pitfalls and Side-Effects of Management.
- IV. Management with Co-Morbidities.
- A. Renal Insufficiency.
- B. Liver Insufficiency.
- C. Systolic and Diastolic Heart Failure.
- D. Coronary Artery Disease or Peripheral Vascular Disease.
- E. Diabetes or other Endocrine issues.
- F. Malignancy.
- G. Immunosuppression (HIV, chronic steroids, etc).
- H. Primary Lung Disease (COPD, Asthma, ILD).
- I. Gastrointestinal or Nutrition Issues.
- J. Hematologic or Coagulation Issues.
- K. Dementia or Psychiatric Illness/Treatment.
- V. Transitions of Care.
- A. Sign-out considerations While Hospitalized.
- B. Anticipated Length of Stay.
- C. When is the Patient Ready for Discharge.
- D. Arranging for Clinic Follow-up.
- 1. When should clinic follow up be arranged and with whom.
- 2. What tests should be conducted prior to discharge to enable best clinic first visit.
- 3. What tests should be ordered as an outpatient prior to, or on the day of, the clinic visit.
- E. Placement Considerations.
- F. Prognosis and Patient Counseling.
- VI. Patient Safety and Quality Measures.
- A. Core Indicator Standards and Documentation.
- B. Appropriate Prophylaxis and Other Measures to Prevent Readmission.