Peutz-Jeghers syndrome with mesenteric fibromatosis: A case report and review of literature

Table 1 Malignancy risk of Peutz-Jeghers syndrome and main mode of monitoring[11]

Cancer site	General population risk, %	Syndrome risk, %	Average age of diagnosis in yr	Age at the start of monitoring	Monitoring interval in yr	Monitoring mode	Ref.
Pancreas	1.5	11-36	41-52	30	1-2	MRCP or US	[11,14-17]
Stomach	< 1	29	30-40	8.181	3	Gastroscopy	[11,14,15,18]
Small bowel	< 1	13	37-42	8.181	3	Capsule endoscopy	[11,14,15]
Colon	4.5	39	42-46	8.181	3	Colonoscopy	[11]
Testicle (Sertoli cell tumor)	< 1	9	6-9	14	1	Testicular examination or US	[11,14,15]
Breast	12.4	32-54	37-59	25	1	MRI or mammography	[11,14-16,19]
Uterus	2.7	9	43	25	1	Pelvic examination or vaginal US	[14,15]
Cervix (adenoma malignum)	< 1	10	34-40	25	1	Cervical smear	[11,14,15,20]
Ovary (mostly SCTAT (sex cord tumor with annular tubules))	1.6	21	28	25	1	Pelvic examination or vaginal US	[11,15]
Lung	6.9	7-17	47	–	–	Quit smoking	[14-16]

¹Began from the age of 8; if polyps were found, followed every 3 years; if no polyps were found, examined again at age 18, then every 3 years, or earlier if symptoms occurred. MRCP: Magnetic resonance cholangiopancreatography; MRI: Magnetic resonance imaging; US: Ultrasonography; MRI: Magnetic resonance imaging.