Surgical management of intraductal papillary mucinous neoplasm with main duct involvement: an international expert survey and case-vignette study



      The risk of invasive cancer in resected intraductal papillary mucinous neoplasm with main pancreatic duct involvement is 33%–60%. Most guidelines, therefore, advise resection of main duct intraductal papillary mucinous neoplasm and mixed type intraductal papillary mucinous neoplasm in surgically fit patients, although advice on the surgical strategy (partial or total pancreatectomy) differs. We performed a survey amongst international experts to guide the design of future studies and help to prepare for a single international set of guidelines.


      An online survey including case vignettes was sent to 221 international experts who had published on main duct/mixed type intraductal papillary mucinous neoplasm in the previous decade and to all surgeon and gastroenterologist members of the pancreatic cyst guideline committees of the European Study Group and the International Association of Pancreatology.


      Overall, 97 experts (67 surgeons, 30 gastroenterologists) from 19 countries replied (44% response rate). Most (93%) worked in an academic hospital, with a median of 15 years’ experience with intraductal papillary mucinous neoplasm treatment. In main duct/mixed type intraductal papillary mucinous neoplasm patients with pancreatic duct dilation (>5 mm) in the entire pancreas, 41% (n = 37) advised nonoperative surveillance every 36 months, whereas 59% (n = 54) advised operative intervention. Of those who advised operative intervention, 46% (n = 25) would perform a total pancreatectomy and 31% (n = 17) pancreatoduodenectomy with follow-up. No structural differences in advice were seen between surgeons and gastroenterologists, between continents where the respondents lived, and based on years of experience.


      This international survey identified a clinically relevant lack of consensus in the treatment strategy in main duct/mixed type intraductal papillary mucinous neoplasm among experts. Studies with long-term follow-up including quality of life after partial and total pancreatectomy for main duct/mixed type intraductal papillary mucinous neoplasm are required.
      To read this article in full you will need to make a payment

      Purchase one-time access:

      Academic & Personal: 24 hour online accessCorporate R&D Professionals: 24 hour online access
      One-time access price info
      • For academic or personal research use, select 'Academic and Personal'
      • For corporate R&D use, select 'Corporate R&D Professionals'


      Subscribe to Surgery
      Already a print subscriber? Claim online access
      Already an online subscriber? Sign in
      Institutional Access: Sign in to ScienceDirect


        • Hruban RH
        • Takaori K
        • Klimstra DS
        • Adsay NV
        • Albores-Saavedra J
        • Biankin AV
        • et al.
        An illustrated consensus on the classification of pancreatic intraepithelial neoplasia and intraductal papillary mucinous neoplasms.
        Am J Surg Pathol. 2004; 28: 977-987
        • Tanaka M
        • Chari S
        • Adsay V
        • Fernandez-del Castillo C
        • Falconi M
        • Shimizu M
        • et al.
        International consensus guidelines for management of intraductal papillary mucinous neoplasms and mucinous cystic neoplasms of the pancreas.
        Pancreatology. 2006; 6: 17-32
        • Crippa S
        • Fernandez-Del Castillo C
        • Salvia R
        • Finkelstein D
        • Bassi C
        • Dominguez I
        • et al.
        Mucin-producing neoplasms of the pancreas: an analysis of distinguishing clinical and epidemiologic characteristics.
        Clin Gastroenterol Hepatol. 2010; 8: 213-219
        • Schmidt CM
        • White PB
        • Waters JA
        • Yiannoutsos CT
        • Cummings OW
        • Baker M
        • et al.
        Intraductal papillary mucinous neoplasms: predictors of malignant and invasive pathology.
        Ann Surg. 2007; 246 (discussion 651-4): 644-651
        • Salvia R
        • Fernandez-del Castillo C
        • Bassi C
        • Thayer SP
        • Falconi M
        • Mantovani W
        • et al.
        Main-duct intraductal papillary mucinous neoplasms of the pancreas: clinical predictors of malignancy and long-term survival following resection.
        Ann Surg. 2004; 239 (discussion 685-7): 678-685
        • Kim SC
        • Park KT
        • Lee YJ
        • Lee SS
        • Seo DW
        • Lee SK
        • et al.
        Intraductal papillary mucinous neoplasm of the pancreas: clinical characteristics and treatment outcomes of 118 consecutive patients from a single center.
        J Hepatobiliary Pancreat Surg. 2008; 15: 183-188
        • European Study Group on Cystic Tumours of the Pancreas
        European evidence-based guidelines on pancreatic cystic neoplasms.
        Gut. 2018; 67: 789-804
        • Couvelard A
        • Sauvanet A
        • Kianmanesh R
        • Hammel P
        • Colnot N
        • Levy P
        • et al.
        Frozen sectioning of the pancreatic cut surface during resection of intraductal papillary mucinous neoplasms of the pancreas is useful and reliable: a prospective evaluation.
        Ann Surg. 2005; 242 (discussion 778-80): 774-778
        • Tanaka M
        • Fernández-Del Castillo C
        • Kamisawa T
        • Jang JY
        • Levy P
        • Ohtsuka T
        • Salvia R
        • Shimizu Y
        • Tada M
        • Wolfgang CL
        Revisions of international consensus Fukuoka guidelines for the management of IPMN of the pancreas.
        Pancreatology. 2017; 17: 738-753
        • Ohtsuka T
        • Kono H
        • Tanabe R
        • Nagayoshi Y
        • Mori Y
        • Sadakari Y
        • et al.
        Follow-up study after resection of intraductal papillary mucinous neoplasm of the pancreas; special references to the multifocal lesions and development of ductal carcinoma in the remnant pancreas.
        Am J Surg. 2012; 204: 44-48
        • Kang MJ
        • Jang JY
        • Lee KB
        • Chang YR
        • Kwon W
        • Kim SW
        Long-term prospective cohort study of patients undergoing pancreatectomy for intraductal papillary mucinous neoplasm of the pancreas: implications for postoperative surveillance.
        Ann Surg. 2014; 260: 356-363
        • Marchegiani G
        • Mino-Kenudson M
        • Ferrone CR
        • Warshaw AL
        • Lillemoe KD
        • Fernandez-del Castillo C
        Oncocytic-type intraductal papillary mucinous neoplasms: a unique malignant pancreatic tumor with good long-term prognosis.
        J Am Coll Surg. 2015; 220: 839-844
        • Paini M
        • Crippa S
        • Scopelliti F
        • Baldoni A
        • Manzoni A
        • Belfiori G
        • et al.
        Extent of Surgery and Implications of Transection Margin Status after Resection of IPMNs.
        Gastroenterol Res Pract. 2014; 2014:269803
        • Vege SS
        • Ziring B
        • Jain R
        • Moayyedi P
        Clinical Guidelines Committee; American Gastroenterology Association. American gastroenterological association institute guideline on the diagnosis and management of asymptomatic neoplastic pancreatic cysts.
        Gastroenterology. 2015; 148 (quiz e12-3): 819-822
        • Daude M
        • Muscari F
        • Buscail C
        • Carrere N
        • Otal P
        • Selves J
        • et al.
        Outcomes of nonresected main-duct intraductal papillary mucinous neoplasms of the pancreas.
        World J Gastroenterol. 2015; 21: 2658-2667
        • Takuma K
        • Kamisawa T
        • Anjiki H
        • Egawa N
        • Kurata M
        • Honda G
        • et al.
        Predictors of malignancy and natural history of main-duct intraductal papillary mucinous neoplasms of the pancreas.
        Pancreas. 2011; 40: 371-375
        • Roch AM
        • DeWitt JM
        • Al-Haddad MA
        • Schmidt CM
        • 2nd CeppaEP
        • House MG
        • et al.
        Nonoperative management of main pancreatic duct-involved intraductal papillary mucinous neoplasm might be indicated in select patients.
        J Am Coll Surg. 2014; 219: 122-129
        • Uehara H
        • Ishikawa O
        • Ikezawa K
        • Kawada N
        • Inoue T
        • Takakura R
        • et al.
        A natural course of main duct intraductal papillary mucinous neoplasm of the pancreas with lower likelihood of malignancy.
        Pancreas. 2010; 39: 653-657
        • Lekkerkerker SJ
        • Besselink MG
        • Busch OR
        • Verheij J
        • Engelbrecht MR
        • Rauws EA
        • et al.
        Comparing 3 guidelines on the management of surgically removed pancreatic cysts with regard to pathological outcome.
        Gastrointest Endosc. 2017; 85: 1025-1031
        • Yamada S
        • Fujii T
        • Murotani K
        • Kanda M
        • Sugimoto H
        • Nakayama G
        • et al.
        Comparison of the international consensus guidelines for predicting malignancy in intraductal papillary mucinous neoplasms.
        Surgery. 2016; 159: 878-884
        • Kaimakliotis P
        • Riff B
        • Pourmand K
        • Chandrasekhara V
        • Furth EE
        • Siegelman ES
        • et al.
        Sendai and Fukuoka Consensus Guidelines Identify Advanced Neoplasia in Patients With Suspected Mucinous Cystic Neoplasms of the Pancreas.
        Clin Gastroenterol Hepatol. 2015; 13: 1808-1815
        • Singhi AD
        • Zeh HJ
        • Brand RE
        • Nikiforova MN
        • Chennat JS
        • Fasanella KE
        • et al.
        American Gastroenterological Association guidelines are inaccurate in detecting pancreatic cysts with advanced neoplasia: a clinicopathologic study of 225 patients with supporting molecular data.
        Gastrointest Endosc. 2016; 83 (e2): 1107-1117
        • Billings BJ
        • Christein JD
        • Harmsen WS
        • Harrington JR
        • Chari ST
        • Que FG
        • et al.
        Quality-of-life after total pancreatectomy: is it really that bad on long-term follow-up?.
        J Gastrointest Surg. 2005; 9 (discussion 1066-7): 1059-1066
        • Watanabe Y
        • Ohtsuka T
        • Matsunaga T
        • Kimura H
        • Tamura K
        • Ideno N
        • et al.
        Long-term outcomes after total pancreatectomy: special reference to survivors' living conditions and quality of life.
        World J Surg. 2015; 39: 1231-1239
        • Epelboym I
        • Winner M
        • DiNorcia J
        • Lee MK
        • Lee JA
        • Schrope B
        • et al.
        Quality of life in patients after total pancreatectomy is comparable with quality of life in patients who undergo a partial pancreatic resection.
        J Surg Res. 2014; 187: 189-196
        • Muller MW
        • Friess H
        • Kleeff J
        • Dahmen R
        • Wagner M
        • Hinz U
        • et al.
        Is there still a role for total pancreatectomy?.
        Ann Surg. 2007; 246 (discussion 974-5): 966-974
        • Roberts KJ
        • Blanco G
        • Webber J
        • Marudanayagam R
        • Sutcliffe RP
        • Muiesan P
        • et al.
        How severe is diabetes after total pancreatectomy? A case-matched analysis.
        HPB (Oxford). 2014; 16: 814-821