world cancer report - iarc

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