Table 1. Genes Associated with Increased Risk for
Pancreatic Cancer
Gene Syndrome
Pancreatic
Cancer Risk
Other Associated
Cancers
b
APC Familial
Adenomatous
Polyposis
1–5% Colorectal
Upper GI
yroid
Brain
ATM Ataxia Telangiectasia
(biallelic)
a
1–5% Breast
Prostate
Gastric
BRCA2 Hereditary Breast
Ovarian Cancer
Syndrome
5–10% Breast
Ovary
Prostate
Melanoma
BRCA1 Hereditary Breast
Ovarian Cancer
Syndrome
2% Breast
Ovary
Prostate
Melanoma
CDKN2A Familial Atypical
Multiple Mole
Melanoma
(FAMMM)
10–30% Melanoma
MLH1, MSH2,
MSH6, PMS2,
EPCAM
Lynch Syndrome 5–10% Colorectal
Uterine
Upper GI
Ovary
Urinary Tract
Brain
Sebaceous neoplasms
PALB2 5–10% Breast
Prostate
STK11 Peutz Jeghers
Syndrome
10–30% Breast
Colorectal
Upper GI
Lung
Reproductive Tract
TP53 Li Fraumeni
Syndrome
N/D Breast
Brain
Sarcoma
Adrenocortical
Carcinoma
a
Biallelic ATM mutation carriers have Ataxia Telangiectasia, but a single ATM mutation is associated
with increased risk for pancreatic cancer.
b
Most commonly associated cancers.
