The incidence of SCC varies widely across the globe, with countries in central Asia and the far-east (India and China) having a much higher disease burden compared to the Western world [1]. This may be directly related to increased tobacco and alcohol consumption, although genetic, familial, and environmental factors have been invoked. Achalasia and a prior history of caustic injury to the esophagus have been associated with an increased incidence of esophageal SCC. The overall incidence of SCC in the USA is on the decline.

Esophageal adenocarcinoma is much more common in the USA and the Western world, compared to SCC, and is almost always found in the setting of Barrett’s esophagus (BE). The overall incidence of esophageal adenocarcinoma is on the rise [2]. The presence of long-standing acid reflux, genetic factors, race (Caucasians), and high BMI has all been implicated as risk factors. Cigarette smoking increases the risk further, especially in patients with BE.

Dysphagia, weight loss, retrosternal burning or discomfort, and regurgitation of food are the usual symptoms in patients with malignant esophageal strictures. Patients may also present with gastrointestinal bleeding (melena or hematemesis) and anemia. Hoarseness and respiratory symptoms (pneumonia) may suggest laryngeal nerve involvement and/or the development of a fistula, respectively, due to locally advanced, infiltrating disease.

Apart from intrinsic luminal disease, esophageal strictures can also be encountered due to malignant processes that arise in the mediastinum (lymphoma) and thorax (lung cancer). These will typically cause “extrinsic” compression of the esophageal lumen leading primarily to symptoms of dysphagia, regurgitation, and chest pain as well as weight loss over a period of time due to poor nutrition (Fig. 7.2). In these patients, additional clinical signs and symptoms may coexist related to the primary pathology (e.g., superior vena cava syndrome due to lung cancer). Primary mediastinal tumors (germ cell, mesenchymal, neurogenic, and thymic origin), lymphoma, and thyroid malignancies (especially with retrosternal and substernal extension) can cause extrinsic esophageal compression and dysphagia. In addition, some of these patients may have cough, stridor, hemoptysis, and constitutional symptoms such as fever, night sweats, and weight loss. The degree and severity of the esophageal symptoms depend on the proximity of the tumor to the esophagus, tumor size, and rate of growth. Some patients can “adapt” remarkably well to a slow-growing tumor and report minimal symptoms over time.

Metastatic breast carcinoma, melanoma, and neuroendocrine tumor can also lead to a bulky mediastinal tumor burden and may cause a compressive “mass effect” on the esophagus, causing focal or complex esophageal luminal stenosis, presenting as dysphagia.

Adenocarcinoma of the lung and other primary pulmonary malignancies with associated bulky adenopathy may lead to significant esophageal compression on occasion, often presenting as dysphagia. Other symptoms such as dyspnea, hemoptysis, hoarseness, and chest pain typically also exist in patients with such advanced stage tumors.

Carcinoid tumors and gastrointestinal stromal tumors (GIST) of the esophagus are neoplastic lesions that can create luminal obstruction and stenosis depending on the size, location in the esophagus, and rate of growth. These lesions can grow in both directions (into the mediastinum or toward the esophageal lumen) and create a mass effect, predominantly causing dysphagia and/or chest pain in most patients. Many of these tumors are “pre-malignant,” although malignant features are well described, and they carry metastatic potential [3, 4].

Malignant strictures of the stomach and duodenum can be due to adenocarcinoma of either organ, or secondary to obstruction of the pyloro-duodenal channel from locally advanced pancreatic cancer, ampullary cancer, cholangiocarcinoma, lymphoma, or metastatic malignancy (Table 7.1). The most common symptoms due to clinically significant malignant foregut strictures are abdominal pain, abdominal distension, and nausea/vomiting due to GOO (Fig. 7.3a, b). Weight loss in these patients can be both dramatic and rapid. These patients may also present with anemia, gastrointestinal bleeding (melena or occult blood loss), and anorexia. In patients with pancreatic head malignancy, biliary obstruction will usually be present concomitantly with GOO. In this section, we will briefly discuss the common causes of malignant foregut (gastric and duodenal) strictures.

In this section, we describe the current approach to evaluation and management of the various malignant esophageal and foregut strictures discussed above. The importance of a detailed history and review of records cannot be overemphasized. In most cases, the mainstay of investigation involves radiographic (contrast studies and cross-sectional imaging) and endoscopic evaluation.

Tissue sampling using luminal endoscopy and/or endoscopic ultrasound (EUS) is essential to make an accurate diagnosis and to plan appropriate treatment. Depending on the anatomic location of the stricture and the patient’s history and clinical presentation, one or more diagnostic tests may be needed in a given situation. These are listed in Table 7.2.

Depending on the nature of the malignancy, location of pathology, extent of disease and clinical stage, and overall condition of the patient, several different management options can be considered. For loco-regional disease, surgical management (with or without neoadjuvant chemo-radiation) may be an option in patients who are deemed fit to undergo surgery. For non-surgical candidates (either due to comorbidity or advanced malignancy), endoscopic palliative therapy has become the first-line therapy and provides symptomatic relief [6, 7]. Patients with advanced disease may still be able to receive some benefits from chemotherapy and radiation treatment concomitantly.