see also:

• neoplasia / cancer / tumours

• the most common tumours of the oesophagus are primary oesophageal tumours - in Australia, over 70% are SCC (mainly proximal), the remainder are adenocarcinoma (mainly distal).
• survival is still poor, particularly if late diagnosis as metastatic disease at diagnosis reducing 12 month survival rates to a third of those with local disease only.
  • 12 month survival rates: local disease ~50%; regional disease 43%; distant 12%¹⁾;
  • 60 month survival rates: local disease 22%; regional disease 12%; distant 2%²⁾;

• ~80% of global cases occur in Asia
• SCC predominates (>90%) in Eastern/Central Asia, Eastern/Southern Africa
• adenocarcinoma is more common in Western countries
• NSW 1972-2005³⁾:
  • 72% were SCC and others, and these affected men and women almost equally although incidence was marginally higher in men
    • age distribution similar to that of adenocarcinoma
    • incidence is slowly falling since 1982 in both men and women
  • 28% were adenocarcinoma and mainly affected men (over 4x incidence of women)
    • most were diagnosed in 60-80 year olds (this age group accounted for 60% of cases, while ~20% were in those older than 80 yrs and only 6% were in those under 50 years).
    • incidence in men has been steadily rising, quadrupling since 1984 from 1 case per 100,000 to 4 cases per 100,000, presumably related to obesity, smoking, alcohol intake, perhaps also due to the falling incidence of Helicobacter pylori infection and increasing GOR.

• mainly proximal-mid oesophagus
• lifetime risk is ~ 0.5% for high risk males
• risk is higher in Asia/Africa

• smoking
  • seems to be the primary driver
  • current smokers have roughly 2-4x elevated ESCC risk versus never-smokers, rising to 5-10x for heavy smokers (>1 pack/day or high pack-years)
  • ex-smokers retain ~2x risk, which declines after 5+ years of cessation (to ~60% of current smoker risk), approaching non-smoker levels after 20+ years
• moderate to heavy alcohol intake
  • in non-smokers risk increases by 1.5-3x
  • in smokers, the combined risk becomes over 10 fold and even over 100 fold increased risk ⁴⁾
  • risk is higher in Asians with ALDH2*2 E487K gene mutation (see below)
• regular drinking hot beverages over 65degC
  • 4-6 cups/day appears to double the risk independent of smoking risk in a dose dependent manner, increasing to 5-7x risk for > 8 very hot drinks/d ⁵⁾
• eating hot foods
  • burning hot soup or porridge in China seems to increase risk ~5-7x⁶⁾
  • hot nsima (corn meal; local staple food and commonly consumed very hot) in Malawi seems to increase risk by ~3x⁷⁾
• human papilloma virus (HPV) may have a role but < 50% of patients have HPV
• poverty, poor oral health, poor nutrition
• achalasia
• other rare causes:
  • caustic ingestion (lye)
  • Plummer-Vinson syndrome (iron deficiency with webs)
  • tylosis (genetic palmoplantar keratoderma)
• genetic
  • mitochondrial ALDH2*2 E487K gene mutation causing impaired aldehyde dehydrogenase activity
    • an ALDH2-deficient drinker who drinks two beers per day has six to ten times the risk of developing eosophageal cancer as a drinker not deficient in the enzyme, especially if they also smoke as the enzyme also metabolizes acrolein which is a highly reactive aldehyde produced by environmental exposure to pollutants such as cigarette smoke.
    • most people with this enzyme deficiency are descendants of the Han dynasty in China (thus extends to the Japanese - 40% of whom have this gene mutation) in whom it may be protective against Entamoeba infections⁸⁾
    • it is rare in Caucasians
    • these people may be at risk of metabolic insults and hepatocyte apoptosis due to “aldehyde storm” following exposures to alcohol and acrolein when glutathion stores are low ⁹⁾
    • is also associated with osteoporosis, Alzheimer's disease, and coronary artery spasm in relation to tobacco smoke exposure

• early:
  • usually asymptomatic or early mild dysphagia
• locally advanced
  • severe dysphagia
  • weight loss
  • odynophagia
• metastatic
  • hoarse voice
  • bone pain
  • fatigue

• gastroscopy or barium swallow

• early ESCC T1N0M0 confined to mucosa (T1a) or superficial submucosa (T1b), is often curable (cure rates may exceed 90% esp. with ESD) with endoscopic or surgical approaches such as endoscopic submucosal dissection (ESD) or mucosal resection (EMR)
• higher risk disease may require definitive chemoradiotherapy (CRT: 50-60 Gy + cisplatin/5-FU) or oesophagectomy with lymphadenectomy

• mainly distal and related to chronic inflammation from gastro-oesophageal reflux

• Barrett's oesophagus metaplasia due to chronic GOR
• chronic gastro-oesophageal reflux is also an independent risk factor
• obesity
• smoking