world cancer report - iarc
world cancer report - iarc
world cancer report - iarc
Create successful ePaper yourself
Turn your PDF publications into a flip-book with our unique Google optimized e-Paper software.
Hereditary condition Mode of inheritance Gene (chromosomal location) Lifetime risk of pancreatic <strong>cancer</strong><br />
Early onset familial pancreatic Autosomal dominant Unknown About 30%; 100-fold increased risk<br />
adenocarcinoma associated of pancreatic <strong>cancer</strong>; high risk<br />
with diabetes (Seattle family) of diabetes and pancreatitis<br />
Hereditary pancreatitis Autosomal dominant Cationic trypsinogen (7q35) 30%; 50-fold increased risk of<br />
pancreatic <strong>cancer</strong><br />
FAMMM: familial atypical multiple Autosomal dominant p16 INK4A /CMM2 (9p21) 10%<br />
mole melanoma<br />
Familial breast <strong>cancer</strong> Autosomal dominant BRCA2 (13q12-q13) 5-10%; 6174delT in Ashkenazi Jews,<br />
999del5 in Iceland<br />
Ataxia telangiectasia Autosomal recessive ATM, ATB, others (11q22-q23) Unknown; somewhat increased<br />
(heterozygote state)<br />
Peutz-Jeghers syndrome Autosomal dominant STK11/LKB1 (19p) Unknown; somewhat increased<br />
HNPCC: hereditary non-polyposis Autosomal dominant MSH2 (2p), MLH1 (3p), others Unknown; somewhat increased<br />
colorectal <strong>cancer</strong><br />
Familial pancreatic <strong>cancer</strong> Possibly autosomal Unknown Unknown; 5-10 fold increased risk<br />
dominant if a first-degree relative has<br />
pancreatic <strong>cancer</strong><br />
Table 5.13 Hereditary conditions predisposing to the development of pancreatic <strong>cancer</strong>.<br />
Fig. 5.126 Five-year relative survival rates after<br />
diagnosis of pancreatic <strong>cancer</strong>. Less than 5% of<br />
patients survive more than five years.<br />
However, morbidity remains high at 30-<br />
40%, and complications are common. In<br />
a total pancreatectomy, the entire pancreas,<br />
as well as the duodenum, common<br />
bile duct, gallbladder, spleen, and nearby<br />
lymph nodes are removed. Symptoms of<br />
unresectable tumours may also be<br />
relieved by surgery.<br />
In Western countries and Japan, different<br />
classification systems for staging of pancreatic<br />
<strong>cancer</strong> have evolved, resulting in<br />
difficulties in assessing the efficacy of different<br />
therapies. Both to overcome the barriers<br />
inherent in international classification<br />
systems and to achieve a universal<br />
prospective data acquisition, a uniform<br />
International Documentation System for<br />
Exocrine Pancreatic Cancer has been<br />
developed by an international group of pancreatologists<br />
[13].<br />
Palliative treatment is required for the<br />
treatment of jaundice, gastric outlet<br />
obstruction and pain. Adjuvant<br />
chemotherapy (5-fluorouracil and folinic<br />
acid), but not adjuvant radiotherapy,<br />
appears to confer a slight survival benefit.<br />
Confirmatory trials with newer agents<br />
are ongoing. Despite substantial evidence<br />
for hormone-dependence of pancreatic<br />
<strong>cancer</strong>, there are no data currently<br />
confirming a role for estrogens, androgens,<br />
cholecystokinin or their antagonists<br />
in clinical treatment of exocrine<br />
pancreatic <strong>cancer</strong> [2].<br />
Survival is poor and the majority of pancreatic<br />
<strong>cancer</strong> patients die within one<br />
year of diagnosis, although five-year survival<br />
rates can reach >30% for lesions of<br />
less than 2 cm, negative lymph nodes<br />
and clear surgical margins. In American<br />
males, for example, the overall five-year<br />
survival rate is 3.7%, and for females,<br />
4.4% (Fig. 5.126).<br />
Pancreatic <strong>cancer</strong><br />
251