Familial pancreatic cancer: Concept, management and issues

Table 4 Outcomes of pancreatic cancer surveillance of high risk individual

Ref.	Year	Country/registry	Entry period	Subjects conditions	Age (range), yr	n	Duration (mo)	Modality	Ratio of surgical cases (n)	Pathology of the pancreatic lesion: n			Ratio of unresectable advanced PC (n)
								(surveillance→examination)		Benign1	Border/CIS2	PC
Brentnall et al[42]	1999	United States	NA	FPC kindred	41 (28-65)	14	15	EUS, CT → ERCP	50.0% (7)	0	75	0	0% (0)
Canto et al[101]	2004	United States	1998-2001	FPC kindred, PJS	58 (NA)	38	22	EUS → CT, EUS-FNA, ERCP	18.4% (7)	4	2	1	0% (0)
Canto et al[97]	2006	United States	2001-2004	FPC kindred, PJS	52 (32-77)	78	12	EUS, CT → EUS-FNA, ERCP	9.0% (7)	4	3	0	1.3% (1)
Langer et al[50]	2009	FaPaCa	1999-2007	FPC kindred, BRCA2 (+)4, CDKN2A (+) FAMMM family	60 (35-85)	76	NA	EUS, MRI → EUS-FNA	9.2% (7)3	6	0	0	0% (0)
Poley et al[95]	2009	Netherlands	2005-2007	FPC kindred, HP, PJS, FAMMM, BRCA1/2 (+), TP53 (+)	50 (32-75)	44	Initial6	EUS → CT, MRI	6.8% (3)	0	0	3	0% (0)
Verna et al[124]	2010	United States	2005-2008	FPC kindred, BRCA1/2 (+), LS, FAMMM	52 (29-77)	51	Initial	EUS, MRI → EUS-FNA, ERCP	9.8% (5)	4	0	1	2.0% (1)
Ludwig et al[114]	2011	United States	2002-2009	FPC kindred, BRCA1/2(+)	54 (33-86)	109	Initial	MRI → EUS, EUS-FNA	5.5% (6)	3	2	1	0% (0)
Vasen et al[125]	2011	Netherlands	2000-2010	CDKN2A-Leiden (+)	56 (39-72)	79	48	MRI	6.3% (5)	0	0	5	2.5% (2)
Zubarik et al[126]	2011	United States	2006-2009	FDR of PC with sCA19-9↑	59 (NA)	26	NA	EUS → EUS-FNA	11.5% (3)	2	0	1	0% (0)
Al-Sukhni et al[127]	2012	Canada	2003-2011	FPC kindred, PJS, HP, CDKN2A (+), BRCA1/2 (+), STK11 (+)	54 (22-89)	262	50	MRI → MRI, EUS, EUS-FNA, ERCP	1.5% (4)	3	0	1	0.8% (2)
Sud et al[91]	2014	United States	2008-2011	FPC kindred, HP, CDKN2A (+), BRCA1/2 (+), PJS, LS	51 (20-75)	16	NA	EUS → EUS-FNA	18.8% (3)	1	0	2	0% (0)
Del Chiaro et al[92]	2015	Sweden	2010-2013	FPC kindred, individuals with increased genetic risk	50 (23-76)	40	13	MRI → EUS, EUS-FNA	12.5% (5)	2	0	3	0% (0)
Vasen et al[94]	2016	FaPaCa	2000-2015	FPC kindred, CDKN2A (+), BRCA1/2 (+), PALB2 (+)	46-56 (25-81)	411	16-53	MRI ± EUS → EUS, CT → EUS-FNA	7.3% (30)	15	4	11	1.0% (4)

¹Benign lesions included low-moderate grade of intraductal papillary mucinous neoplasm (IPMN), grade 1-2 of pancreatic intraepithelial neoplasm (PanIN), serous cystadenoma, and neuroendocrine tumor;

²High-grade precursors and PanIN3;

³No lesion detected in one case of resected pancreas;

⁴(+): mutation carrier;

⁵Wide spread dysplasia;

⁶Evaluated only by the initial surveillance, one resectable pancreatic cancer case (T1N0M0) not resected because of metastatic melanoma. PC: Pancreatic cancer; FPC: Familial pancreatic cancer; PJS: Peutz-Jeghers syndrome; HP: Hereditary pancreatitis; FAMMM: Familial atypical multiple mole melanoma; LS: Lynch syndrome; FDR: First degree relative; FaPaCa: German national case collection for familial pancreatic cancer; NA: Not available, EUS: Endoscopic ultrasonography; EUS-FNA: EUS-guided fine needle aspiration; CT: Computed tomography; ERCP: Endoscopic retrograde cholangiopancreatography; MRI: Magnetic resonance imaging.