Epidemiology, Risk Factors, and Clinical Manifestations of Esophageal Cancer

Incidence

In 2016, an estimated 16,910 people will be diagnosed with esophageal cancer in the United States alone. Worldwide, over 450,000 people are diagnosed annually. Over the past few decades, there has been a major shift in the incidence of esophageal cancer worldwide with trends differing by histologic subtype.

In the United States, the overall incidence of esophageal cancer has been falling an average of 1.4% per year over the past decade ( Fig. 35.1 ). The most recent Surveillance Epidemiology, and End Results (SEER) data estimate 4.3 new cases annually per 100,000 men and women. Across all races, EAC is the most common histologic subtype in the United States and Europe. Since the 1970s, the incidence of EAC has increased at a rate greater than any other malignancy in the United States. The absolute incidence of EAC has increased from 0.4 case per 100,000 in 1975 to 2.58 cases per 100,000 in 2009. During this same period, the incidence of SCC has steadily decreased.

Worldwide, the incidence of esophageal cancer varies by more than 21-fold, with SCC being the predominant subtype. The highest-risk area, referred to as the esophageal cancer belt, extends from the Middle East to northeast China, where the incidence of SCC is more than 100 cases per 100,000 people annually. High rates of esophageal SCC are also seen in Southern and Eastern Africa, whereas the lowest rates are found in Western Africa.

Mortality and Prognosis of Patients With Esophageal Cancer

Esophageal cancer is the 11th leading cause of cancer-related death in the United States. It accounted for an estimated 15,690 deaths in 2016. Over the past decade, the death rate due to esophageal cancer has been declining an average of 0.8% per year (see Fig. 35.1 ). Across all races, the death rate is approximately 5 times higher in men than women. The overall relative 5-year survival rate has increased from 4% in the 1970s to 18.7% currently.

Improved prognosis is seen in patients with localized disease. With complete surgical resection, the relative 5-year survival rate is approximately 90% for pTis tumors, 75% for pT1, 45% for pT2, 30% for pT3, and 10% to 15% for pT4 disease. Unfortunately, more than 30% of patients have metastatic disease at the time of presentation, with a relative 5-year survival of 4.5% ( Table 35.1 ).

Age, Sex, and Race Distribution

Esophageal cancer is more common with increased age. The majority of new cases are diagnosed in people aged 65 to 74 years with a median age at diagnosis of 67. Overall, it has a male preponderance (7 : 1) with a high of 11 : 1 in those aged 50 to 54 and a low of 4 : 1 in those aged 75 to 79. This predilection for males is seen irrespective of race/ethnicity.

Across races, substantial differences are seen in the incidence of esophageal cancer and the distribution of the histologic subtypes. The age-adjusted incidence rate of esophageal cancer is highest in white and black men (7.9 and 7.2 per 100,000 people, respectively) and lowest in men of Asian/Pacific Islander descent (3.4 per 100,000). Over the past three decades, the incidence of esophageal cancer has steadily decreased in black men and women, whereas it has increased in white men ( Fig. 35.2 ). The overall incidence has been relatively stable in white women. Histologically, the rate of SCC is fourfold greater in black versus white men, whereas EAC is fivefold greater in white versus black men. The SEER data from 2009–2013 demonstrated that SCC accounted for 78.7% of esophageal cancer in blacks and 68.5% of Asian/Pacific Islanders, compared with only 25.4% in whites. The opposite was true for EAC, which was the primary histologic subtype in 69.1% of whites, 61.3% of American Indians/Alaskan Natives, and 57.4% of Hispanics, but only 16.6% of blacks.