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.
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.
SCC oesophageal cancers (ESCC)
mainly proximal-mid oesophagus
lifetime risk is ~ 0.5% for high risk males
risk is higher in Asia/Africa
risk factors include:
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)
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
clinical features
early:
usually asymptomatic or early mild dysphagia
locally advanced
severe dysphagia
weight loss due to reduced food intake
odynophagia
metastatic
hoarse voice
bone pain
fatigue
Dx
gastroscopy or barium swallow
Rx
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