Less common malignant esophageal tumors include small-cell carcinoma, melanoma, leiomyosarcoma, lymphoma, and esophageal involvement by metastatic cancer.
Most patients with early-stage disease are asymptomatic or may have symptoms of reflux. Patients with esophageal cancer may complain of dysphagia, odynophagia, and weight loss. Symptoms that are suggestive of unresectability include hoarseness, abdominal pain, persistent back or bone pain, hiccups, and respiratory symptoms (cough or aspiration pneumonia suggesting possible esophagorespiratory fistula). Approximately 50% of presenting patients have unresectable lesions or distant metastasis, which is largely responsible for the generally poor prognosis.
The diagnosis is suggested by a barium swallow and confirmed with esophagoscopy and biopsy or brush cytology.
Staging. A system for staging esophageal cancer allows assignment of patients to groups with similar prognosis, helps to determine if local or systemic therapy is needed, and allows comparison of response to different types of therapy (Table 8-1). Evaluation for lymph node and distant-organ metastatic disease is performed by CT scanning, which can be combined with positron emission tomographic (PET) scanning to improve diagnostic accuracy. Endoscopic ultrasonography is more accurate than radiographic studies for determining the depth of wall invasion and the involvement of peritumoral lymph nodes. Upper esophageal and midesophageal lesions require bronchoscopy to evaluate the airway for involvement by tumor.
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TNM (Tumor, Node, Metastasis) Staging System for Esophageal Cancer
Definition of TNM T: Primary tumor Tx Primary tumor cannot be assessed (cytologically positive tumor not evident radiographically or endoscopically) T0 No evidence of primary tumor (e.g., after treatment with radiation and chemotherapy) Tis Carcinoma in situ T1 Tumor invades the submucosa T2 Tumor invades the muscularis propria T3 Tumor invades periesophageal tissue T4 Tumor invades contiguous structures N: Regional lymph nodes Nx Regional nodes cannot be assessed N0 No regional node metastasis N1 Regional node metastasis M: Distant metastasis Mx Distant metastasis cannot be assessed M0 No distant metastasis M1a Upper thoracic esophagus metastatic to cervical lymph nodes Lower thoracic esophagus metastatic to celiac lymph nodes M1b Metastases to nonregional lymph nodes or other distant sites Stage grouping Stage 0 Tis N0 M0 Stage I T1 N0 M0 Stage IIA T2 N0 M0 T3 N0 M0 Stage IIB T1 N1 M0 T2 N1 M0 Stage III T3 N1 M0 T4 Any N M0 Stage IVA Any T Any N M1a Stage IVB Any T Any N M1b
Treatment
Surgical resection remains a mainstay of curative treatment for patients with localized disease. It offers the best opportunity for cure and provides substantial palliation when cure is not possible. The overall 5-year survival rate is 20% to 30%, with higher rates for patients with lower stages of disease (J Thorac Cardiovasc Surg 1993;105:265).
Options for resection include a standard transthoracic esophagectomy, a transhiatal esophagectomy, or an en bloc esophagectomy. Total esophagectomy with a cervical esophagogastric anastomosis and subtotal resection with a high intrathoracic anastomosis have become the most common resections and produce the best long-term functional results as well as the best chance for cure. Esophagogastrectomy with anastomosis to the distal half of the esophagus is seldom used because troublesome postoperative reflux is common.
Options for esophageal replacement include the stomach, colon, and jejunum.Neoadjuvant therapy with preoperative chemotherapy or chemoradiotherapy has been evaluated in a number of trials. Although it may enhance local control and resectability, it does not appear to reduce the risk of systemic spread, and the survival benefit is unclear. However, a more recent prospective, randomized, controlled study evaluating preoperative treatment with epirubicin, cisplatin, and 5-fluorouracil and then surgery versus surgery alone showed an improved progression-free and overall survival rate. This study compared 250 patients in each arm and had a median follow-up of 4 years. It found an improved 5-year survival from 23% in the surgery-alone arm to 36% in the preoperative-chemotherapy-and-surgery arm (N Engl J Med 2006; 355:1).
Radiotherapy is used worldwide for attempted cure and palliation of patients with squamous cell esophageal cancer deemed unsuitable for resection. The 5-year survival rate is 5% to 10%. Palliation of dysphagia is successful temporarily in 80% of patients but rarely provides complete long-term relief. Combination therapy involving radiation and concurrent administration of 5-fluorouracil with mitomycin C or cisplatin has been suggested to improve results and has replaced radiation alone in most protocols.
The goal of palliative treatment is the relief of obstruction and dysphagia.
Radiotherapy and chemotherapy work best in patients with squamous cell carcinoma, particularly when it is located above the carina. Adenocarcinoma is less responsive to radiation, and the acute morbidity (nausea and vomiting) of external-beam irradiation of the epigastric area is substantial.
Esophageal bypass procedures have been largely abandoned due to excessive complication rates.
Intraluminal prostheses have been developed to intubate the esophagus and stent the obstruction. Self-expanding wire-mesh stents, often with a soft silicone (Silastic) coating, have been used with greater ease of insertion and satisfactory results. None of these prostheses allows normal swallowing, and in most cases no more than a pureed diet can be tolerated. The two methods for insertion are (1) peroral (push technique) and (2) via a laparotomy (pull technique). Of these, the peroral route is associated with fewer complications. Potential complications include perforation, erosion or migration of the stent, and obstruction of the tube by food or proximal tumor growth.
Endoscopic laser techniques can restore an esophageal lumen successfully 90% of the time, with only a 4% to 5% perforation rate.