Note: Separate PDQ summaries on Prevention of Esophageal Cancer and Screening for Esophageal Cancer are also available.

Note: Estimated new cases and deaths from esophageal cancer in the United States in 2008:[1]

• New cases: 16,470.
• Deaths: 14,280.

The incidence of esophageal cancer has risen in recent decades, coinciding with a shift in histologic type and primary tumor location.[2,3] Adenocarcinoma of the esophagus is now more prevalent than squamous cell carcinoma in the United States and western Europe, with most tumors located in the distal esophagus. The cause for the rising incidence and demographic alterations is unknown.

While risk factors for squamous cell carcinoma of the esophagus have been identified (e.g., tobacco, alcohol, diet), the risk factors associated with esophageal adenocarcinoma are less clear.[3] The presence of Barrett esophagus is associated with an increased risk of developing adenocarcinoma of the esophagus, and chronic reflux is considered the predominant cause of Barrett metaplasia. The results of a population-based, case-controlled study from Sweden strongly suggest that symptomatic gastroesophageal reflux is a risk factor for esophageal adenocarcinoma. The frequency, severity, and duration of reflux symptoms were positively correlated with increased risk of esophageal adenocarcinoma.[4]

Esophageal cancer is a treatable disease, but it is rarely curable. The overall 5-year survival rate in patients amenable to definitive treatment ranges from 5% to 30%. The occasional patient with very early disease has a better chance of survival. Patients with severe dysplasia in distal esophageal Barrett mucosa often have in situ or even invasive cancer within the dysplastic area. Following resection, these patients usually have excellent prognoses.

Primary treatment modalities include surgery alone or chemotherapy with radiation therapy. Combined modality therapy (i.e., chemotherapy plus surgery, or chemotherapy and radiation therapy plus surgery) is under clinical evaluation. Effective palliation may be obtained in individual cases with various combinations of surgery, chemotherapy, radiation therapy, stents,[5] photodynamic therapy,[6-8] and endoscopic therapy with Nd:YAG laser.[9]

One of the major difficulties in allocating and comparing treatment modalities for patients with esophageal cancer is the lack of precise preoperative staging. Standard noninvasive staging modalities include computed tomography (CT) of the chest and abdomen, and endoscopic ultrasound (EUS). The overall tumor depth staging accuracy of EUS is 85% to 90%, as compared with 50% to 80% for CT; the accuracy of regional nodal staging is 70% to 80% for EUS and 50% to 70% for CT.[10,11] EUS-guided fine-needle aspiration (FNA) for lymph node staging is under prospective evaluation; one retrospective series reported a 93% sensitivity and 100% specificity of regional nodal staging with EUS-FNA.[12] Thoracoscopy and laparoscopy have been used in esophageal cancer staging at some surgical centers.[13-15] An intergroup trial (CALGB-9380) reported an increase in positive lymph node detection to 56% of 107 evaluable patients using thoracoscopy/laparoscopy, from 41% (using noninvasive staging tests, e.g., CT, magnetic resonance imaging, EUS) with no major complications or deaths.[16] Noninvasive positron emission tomography using the radiolabeled glucose analog 18-F-fluorodeoxy-D-glucose for preoperative staging of esophageal cancer is under clinical evaluation and may be useful in detecting stage IV disease.[17-20]

Gastrointestinal stromal tumors can occur in the esophagus and are usually benign. (Refer to the PDQ summary on Adult Soft Tissue Sarcoma Treatment for more information.)

References

1. American Cancer Society.: Cancer Facts and Figures 2008. Atlanta, Ga: American Cancer Society, 2008. Also available online. Last accessed October 1, 2008. 
   
   
2. Devesa SS, Blot WJ, Fraumeni JF Jr: Changing patterns in the incidence of esophageal and gastric carcinoma in the United States. Cancer 83 (10): 2049-53, 1998.  [PUBMED Abstract]
   
   
3. Blot WJ, McLaughlin JK: The changing epidemiology of esophageal cancer. Semin Oncol 26 (5 Suppl 15): 2-8, 1999.  [PUBMED Abstract]
   
   
4. Lagergren J, Bergström R, Lindgren A, et al.: Symptomatic gastroesophageal reflux as a risk factor for esophageal adenocarcinoma. N Engl J Med 340 (11): 825-31, 1999.  [PUBMED Abstract]
   
   
5. Tietjen TG, Pasricha PJ, Kalloo AN: Management of malignant esophageal stricture with esophageal dilation and esophageal stents. Gastrointest Endosc Clin N Am 4 (4): 851-62, 1994.  [PUBMED Abstract]
   
   
6. Lightdale CJ, Heier SK, Marcon NE, et al.: Photodynamic therapy with porfimer sodium versus thermal ablation therapy with Nd:YAG laser for palliation of esophageal cancer: a multicenter randomized trial. Gastrointest Endosc 42 (6): 507-12, 1995.  [PUBMED Abstract]
   
   
7. Kubba AK: Role of photodynamic therapy in the management of gastrointestinal cancer. Digestion 60 (1): 1-10, 1999 Jan-Feb.  [PUBMED Abstract]
   
   
8. Heier SK, Heier LM: Tissue sensitizers. Gastrointest Endosc Clin N Am 4 (2): 327-52, 1994.  [PUBMED Abstract]
   
   
9. Bourke MJ, Hope RL, Chu G, et al.: Laser palliation of inoperable malignant dysphagia: initial and at death. Gastrointest Endosc 43 (1): 29-32, 1996.  [PUBMED Abstract]
   
   
10. Ziegler K, Sanft C, Zeitz M, et al.: Evaluation of endosonography in TN staging of oesophageal cancer. Gut 32 (1): 16-20, 1991.  [PUBMED Abstract]
    
    
11. Tio TL, Coene PP, den Hartog Jager FC, et al.: Preoperative TNM classification of esophageal carcinoma by endosonography. Hepatogastroenterology 37 (4): 376-81, 1990.  [PUBMED Abstract]
    
    
12. Vazquez-Sequeiros E, Norton ID, Clain JE, et al.: Impact of EUS-guided fine-needle aspiration on lymph node staging in patients with esophageal carcinoma. Gastrointest Endosc 53 (7): 751-7, 2001.  [PUBMED Abstract]
    
    
13. Bonavina L, Incarbone R, Lattuada E, et al.: Preoperative laparoscopy in management of patients with carcinoma of the esophagus and of the esophagogastric junction. J Surg Oncol 65 (3): 171-4, 1997.  [PUBMED Abstract]
    
    
14. Sugarbaker DJ, Jaklitsch MT, Liptay MJ: Thoracoscopic staging and surgical therapy for esophageal cancer. Chest 107 (6 Suppl): 218S-223S, 1995.  [PUBMED Abstract]
    
    
15. Luketich JD, Schauer P, Landreneau R, et al.: Minimally invasive surgical staging is superior to endoscopic ultrasound in detecting lymph node metastases in esophageal cancer. J Thorac Cardiovasc Surg 114 (5): 817-21; discussion 821-3, 1997.  [PUBMED Abstract]
    
    
16. Krasna MJ, Reed CE, Nedzwiecki D, et al.: CALGB 9380: a prospective trial of the feasibility of thoracoscopy/laparoscopy in staging esophageal cancer. Ann Thorac Surg 71 (4): 1073-9, 2001.  [PUBMED Abstract]
    
    
17. Flamen P, Lerut A, Van Cutsem E, et al.: Utility of positron emission tomography for the staging of patients with potentially operable esophageal carcinoma. J Clin Oncol 18 (18): 3202-10, 2000.  [PUBMED Abstract]
    
    
18. Flamen P, Van Cutsem E, Lerut A, et al.: Positron emission tomography for assessment of the response to induction radiochemotherapy in locally advanced oesophageal cancer. Ann Oncol 13 (3): 361-8, 2002.  [PUBMED Abstract]
    
    
19. Weber WA, Ott K, Becker K, et al.: Prediction of response to preoperative chemotherapy in adenocarcinomas of the esophagogastric junction by metabolic imaging. J Clin Oncol 19 (12): 3058-65, 2001.  [PUBMED Abstract]
    
    
20. van Westreenen HL, Westerterp M, Bossuyt PM, et al.: Systematic review of the staging performance of 18F-fluorodeoxyglucose positron emission tomography in esophageal cancer. J Clin Oncol 22 (18): 3805-12, 2004.  [PUBMED Abstract]
    
    

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