Hamartomatous polyps: Diagnosis, surveillance, and management

Table 1 Studies investigating the malignancy risk associated with Peutz-Jeghers syndrome

Ref.	N	Location	Outcome
Chen et al[80]	336	China	Patients with PJS possess a 50% cumulative malignancy risk by the age of 60, with the most common malignancy being colorectal cancer at a median age of 41
Hearle et al[81]	419	United Kingdom	The risk of developing a malignancy is 85% at the age of 70 in patients with PJS, most common being gastrointestinal in origin
Ishida et al[82]	583	Japan	Patients with PJS possess a cumulative malignancy risk of 83% by the age of 70, with an increased rate of gynecologic malignancy in comparison to previously reported data
Korsse et al[83]	144	Netherlands	The cumulative risk of pancreatico-biliary malignancy is 32% by the age of 70 in patients with PJS
Mehenni et al[84]	149	Switzerland	Patients with PJS have a cumulate malignancy risk of 67% at age 70, particularly with STK11/LKB1 mutations in exon 6. Malignancies most commonly occur in the GI tract
Resta et al[11]	119	Italy	The STK11/LKB1 mutation is associated with a relative overall cancer risk of 15.1, with pancreatic and cervical malignancies being the most common; median age of diagnosis noted to be 41 yr
Van Lier et al[8]	133	Netherlands	Patients with PJS possess a cumulative malignancy rate of 76% by the age of 70; malignancies most commonly occur in the GI tract and in women

GI: Gastrointestinal; PJS: Peutz-Jeghers syndrome.