In 2019 in the United States, 17,060 cases and 16,080 deaths; less frequent in women than men. Highest incidence in focal regions of China, Iran, Afghanistan, Siberia, and Mongolia. In the United States, blacks more frequently affected than whites; usually presents sixth decade or later; 5-year survival <10% because most pts present with advanced disease.
Twenty percent squamous cell carcinoma, most commonly in upper two-thirds; 75% 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. Five percent of all esophageal cancers occur in the upper third, 20% in the middle third, and 75% in the lower third. Fifteen percent of tumors express HER2/neu.
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, selenium. Barrett's esophagus, chronic gastroesophageal reflux, obesity, and smoking are risk factors 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. PET scanning can also assess mediastinal nodes and distant sites.
TREATMENT | ||
Esophageal CarcinomaSurgical 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. Addition of bevacizumab provides minimal benefit. |
Section 6. Hematology and Oncology