In 2012 in the United States, 17,460 cases and 15,070 deaths; less frequent in women than men. Highest incidence in focal regions of China, Iran, Afghanistan, Siberia, Mongolia. In the United States, blacks more frequently affected than whites; usually presents sixth decade or later; 5-year survival <5% because most pts present with advanced disease.
60% squamous cell carcinoma, most commonly in upper two-thirds; <40% adenocarcinoma, usually in distal third, arising in region of columnar metaplasia (Barrett's esophagus), glandular tissue, or as direct extension of proximal gastric adenocarcinoma; lymphoma and melanoma rare. 10% of all esophageal cancers occur in the upper third, 35% in the middle third, and 55% in the lower third.
Major risk factors for squamous cell carcinoma: ethanol abuse, smoking (combination is synergistic); other risks: lye ingestion and esophageal stricture, radiation exposure, head and neck cancer, achalasia, smoked opiates, Plummer-Vinson syndrome, tylosis, chronic ingestion of extremely hot tea, deficiency of vitamin A, zinc, molybdenum. Barrett's esophagus is a risk for adenocarcinoma.
Progressive dysphagia (first with solids, then liquids), rapid weight loss common, chest pain (from mediastinal spread), odynophagia, pulmonary aspiration (obstruction, tracheoesophageal fistula), hoarseness (laryngeal nerve palsy), hypercalcemia (parathyroid hormone–related peptide hypersecretion by squamous carcinomas); bleeding infrequent, occasionally severe; examination often unremarkable.
Double-contrast barium swallow useful as initial test in dysphagia; flexible esophagogastroscopy most sensitive and specific test; pathologic confirmation by combining endoscopic biopsy and cytologic examination of mucosal brushings (neither alone sufficiently sensitive); CT and endoscopic ultrasonography valuable to assess local and nodal spread.
TREATMENT Esophageal Carcinoma
Surgical resection feasible in only 40% of pts; associated with high complication rate (fistula, abscess, aspiration). Squamous cell carcinoma: Surgical resection after chemotherapy [5-fluorouracil (5FU), cisplatin] plus radiation therapy prolongs survival and may provide improved cure rate. Adenocarcinoma: Curative resection rarely possible; <20% of pts with resectable tumors survive 5 years. Palliative measures include laser ablation, mechanical dilatation, radiotherapy, and a luminal prosthesis to bypass the tumor. Gastrostomy or jejunostomy are frequently required for nutritional support. Preoperative chemotherapy with concurrent radiation therapy is somewhat more effective but more toxic therapy.
Highest incidence in Japan, China, Chile, Ireland; incidence decreasing worldwide, eightfold in the United States over past 60 years; in 2012, 21,320 new cases and 10,540 deaths. Male:female = 2:1; peak incidence sixth and seventh decades; overall 5-year survival <15%.
Increased incidence in lower socioeconomic groups; environmental component is suggested by studies of migrants and their offspring. Several dietary factors correlated with increased incidence: nitrates, smoked foods, heavily salted foods; genetic component suggested by increased incidence in first-degree relatives of affected pts; other risk factors: atrophic gastritis, Helicobacter pylori infection, Billroth II gastrectomy, gastrojejunostomy, adenomatous gastric polyps, pernicious anemia, hyperplastic gastric polyps (latter two associated with atrophic gastritis), Ménétrier's disease, slight increased risk with blood group A.
Adenocarcinoma in 85%; usually focal (polypoid, ulcerative), two-thirds arising in antrum or lesser curvature, frequently ulcerative (“intestinal type”); less commonly diffuse infiltrative (linitis plastica) or superficial spreading (diffuse lesions more prevalent in younger pts; exhibit less geographic variation; have extremely poor prognosis); spreads primarily to local nodes, liver, peritoneum; systemic spread uncommon; lymphoma accounts for 15% (most frequent extranodal site in immunocompetent pts), either low-grade tumor of mucosa-associated lymphoid tissue (MALT) or aggressive diffuse large ...