Esophageal Cancer

Patients with stage 0, I, or IIa disease (see table Staging Esophageal Cancer Staging Esophageal Cancer* ) respond well to surgical resection; preoperative chemotherapy and radiation provide additional benefit. Patients with stages IIb and III have poor survival with surgery alone; response and survival are enhanced by preoperative (neoadjuvant) use of radiation and chemotherapy to reduce tumor volume before surgery. Patients unable or unwilling to undergo surgery may receive some benefit from combined radiation and chemotherapy. Radiation or chemotherapy alone is of little benefit. Patients with stage IV disease require palliation and should not undergo surgery.

After treatment, patients are screened for recurrence by endoscopy and CT of the neck, chest, and abdomen at 6-month intervals for 3 years and annually thereafter.

Patients with Barrett esophagus require intense long-term treatment for gastroesophageal reflux disease Gastroesophageal Reflux Disease (GERD) Incompetence of the lower esophageal sphincter allows reflux of gastric contents into the esophagus, causing burning pain. Prolonged reflux may lead to esophagitis, stricture, and rarely metaplasia... read more and endoscopic surveillance for malignant transformation at 3- to 12-month intervals depending on the degree of metaplasia.

Superficial, early, noninvasive cancers may be treated with endoscopic mucosal resection or endoscopic submucosal dissection (usually by gastroenterologists at tertiary care centers) if the superficial nature of the lesion has been confirmed by endoscopic ultrasound. However, in the large majority of cases, en bloc resection for cure requires removal of the entire tumor, proximal and distal margins of normal tissue, all potentially malignant lymph nodes, and a portion of the proximal stomach sufficient to contain the distal draining lymphatics. The procedure requires gastric pull-up with esophagogastric anastomosis, small-bowel interposition, or colonic interposition. Pyloroplasty (surgical widening of the pylorus) is required to ensure proper gastric drainage because esophagectomy necessarily results in bilateral vagotomy. This extensive surgery may be poorly tolerated by patients > 75 years, particularly those with underlying cardiac or pulmonary disease (ejection fraction < 40%, or forced expiratory volume in 1 second [FEV1] < 1.5 L/minute). Overall, operative mortality is about 5%.

Preoperative chemotherapy combined with radiation therapy can improve survival after surgical resection of thoracic esophageal cancer. Chemotherapy without radiation therapy followed by surgery may also be considered (1 Treatment reference The most common malignant tumor in the proximal two thirds of the esophagus is squamous cell carcinoma; adenocarcinoma is the most common in the distal one third. Symptoms are progressive dysphagia... read more ).

Complications of surgery include anastomotic leaks, fistulas, and strictures; bilious gastroesophageal reflux; and dumping syndrome. The burning chest pain of bile reflux after distal esophagectomy can be more annoying than the original symptom of dysphagia and may require subsequent Roux-en-Y jejunostomy for bile diversion. An interposed segment of small bowel or colon in the chest has a tenuous blood supply, and torsion, ischemia, or gangrene of the interposed bowel may result.

Radiation is usually used in combination with chemotherapy for patients who are poor candidates for curative surgery, including those with advanced disease. Radiation is contraindicated in patients with tracheoesophageal fistula because tumor shrinkage enlarges the fistula. Similarly, patients with vascular encasement by tumor may experience massive hemorrhage with tumor shrinkage.

During the early stages of radiation therapy, edema may worsen esophageal obstruction, dysphagia, and odynophagia. This problem may require preradiation dilation and/or placement of a stent. Some patients may require a temporary percutaneous gastrostomy feeding tube. Other adverse effects of radiation therapy include nausea, vomiting, anorexia, fatigue, esophagitis, excess esophageal mucus production, xerostomia, stricture, radiation pneumonitis, radiation pericarditis, myocarditis, and myelitis (spinal cord inflammation).

Tumors are poorly responsive to chemotherapy alone. Response rates (defined as ≥ 50% reduction in all measurable areas of tumor) vary from 10 to 40%, but responses generally are incomplete (minor shrinkage of tumor) and temporary. No drug is notably more effective than another.

Most commonly, cisplatin and 5-fluorouracil are used in combination. However, several other drugs, including mitomycin, doxorubicin, vindesine, bleomycin, and methotrexate, also are active against squamous cell carcinoma.

Immunotherapy plus chemotherapy is now recommended as first-line therapy for advanced esophageal squamous cell cancer regardless of programmed cell death ligand 1 (PD-L1) status. This treatment modality is offered as first-line therapy for advanced esophageal adenocarcinoma with overexpression of PD-L1 (1 Treatment reference The most common malignant tumor in the proximal two thirds of the esophagus is squamous cell carcinoma; adenocarcinoma is the most common in the distal one third. Symptoms are progressive dysphagia... read more ).

Palliation is directed at reducing esophageal obstruction sufficiently to allow oral intake. Suffering caused by esophageal obstruction can be significant, with salivation and recurrent aspiration. Options include manual dilation procedures (bougienage), orally inserted stents, radiation therapy, laser photocoagulation, and photodynamic therapy. In some cases, cervical esophagostomy with feeding jejunostomy is required.

Relief provided by esophageal dilation rarely lasts more than a few days. Flexible metal mesh stents are more effective at maintaining esophageal patency. Some plastic-coated models can also be used to occlude malignant tracheoesophageal fistulas, and some are available with a valve that prevents reflux when the stent must be placed near the lower esophageal sphincter.

Endoscopic laser therapy can palliate dysphagia by burning a central channel through the tumor and can be repeated if needed. Photodynamic therapy uses an injection of porfimer sodium, a hematoporphyrin derivative that is taken up by tissues and acts as a photosensitizer. When activated by a laser beam directed on the tumor, this substance releases cytotoxic oxygen singlets that destroy tumor cells. Patients receiving this treatment must avoid sun exposure for 6 weeks after treatment because the skin is also sensitized to light.