An extremely rare case who underwent total remnant pancreatectomy due to recurrent pancreatic metastasis of intraductal tubulopapillary neoplasm

  • Akira UmemuraEmail author
  • Kazuyuki Ishida
  • Hiroyuki Nitta
  • Takeshi Takahara
  • Yasushi Hasegawa
  • Kenji Makabe
  • Tamotsu Sugai
  • Akira Sasaki
Case Report


We describe a rare case of recurrent pancreatic metastasis of intraductal tubulopapillary neoplasm (ITPN). A 53-year-old woman diagnosed with an intraductal papillary mucinous neoplasm (IPMN) and a pancreatic ductal adenocarcinoma (PDAC) of the pancreatic body underwent a distal pancreatectomy. The tumor was composed of cuboidal, high-grade dysplastic cells proliferating in a tubulopapillary growth pattern without mucin production; hence, the final diagnosis was ITPN. A follow-up computed tomography scan revealed an enhanced 2 cm mass of the pancreatic head 3 years after the surgery. From workup investigations, the patient was diagnosed with PDAC or a recurrent ITPN of the remnant pancreas. A total remnant pancreatectomy was then performed. Histopathological findings revealed that the new ITPN had the same features as the prior ITPN. In IPMNs, the presence of an invasive component and high-grade dysplasia can lead to progression to a recurring IPMN and the development of PDAC. Because there have been few reports of recurrent ITPN developing into PDAC, the risk factors for ITPN have not been investigated. Because of the uncertain clinicopathological characteristics of ITPN, more data should be gathered to assess the long-term outcome and malignant potential of ITPN.


Intraductal tubulopapillary neoplasm Pancreatic intraductal neoplasm Tubulopapillary growth Recurrence 



We gratefully acknowledge the technical assistance of members of the Department of Molecular Diagnostic Pathology, Iwate Medical University.

Compliance with ethical standards

Conflict of interest

Akira Umemura and all the co-authors have no conflict of interest.

Human/animal rights

All procedures followed were in accordance with the ethical standards of the responsible committee on human experimentation (institutional and national) and with the Helsinki Declaration of 1975, as revised in 2008(5).

Informed consent

Informed consent was obtained from the patient for being included in the study.


  1. 1.
    Adsay NV, Fukushima N, Furukawa T, et al Intraductal neoplasms of the pancreas. In: Bosman FT, Carneiro F, Hruban RH, Theise ND, editors. World Health Organization classification of tumours of the digestive system. 4th ed. Lyon: IARC; 2010. pp. 304–13.Google Scholar
  2. 2.
    Yogi T, Hijioka S, Imaoka H, et al. Risk factors for postoperative recurrence of intraductal papillary mucinous neoplasms of the pancreas based on a long-term follow-up study: proposals for follow-up strategies. J Hepatobiliary Pancreat Sci. 2015;22:757–65.CrossRefGoogle Scholar
  3. 3.
    Yamaguchi H, Shimizu M, Ban S, et al. Intraductal tubulopapillary neoplasms of the pancreas distinct from pancreatic intraepithelial neoplasia and intraductal papillary mucinous neoplasms. Am J Surg Pathol. 2009;33:1164–72.CrossRefGoogle Scholar
  4. 4.
    Tajiri T, Tate G, Matsumoto K, et al. Diagnostic challenge: intraductal neoplasms of the pancreatobiliary system. Pathol Res Pract. 2012;208:691–6.CrossRefGoogle Scholar
  5. 5.
    Kitaguchi K, Kato Y, Kojima M, et al. A resected case of intraductal tubulopapillary neoplasm of the pancreas: report of a case. Int Surg. 2015;100:281–6.CrossRefGoogle Scholar
  6. 6.
    Date K, Okabayashi T, Shima Y, et al. Clinicopathological features and surgical outcomes of intraductal tubulopapillary neoplasm of the pancreas: a systematic review. Langenbecks Arch Surg. 2016;401:1391–6.CrossRefGoogle Scholar
  7. 7.
    Yamaguchi H, Kuboki Y, Hatori T, et al. Somatic mutations in PIK3CA and activation of AKT in intraductal tubulopapillary neoplasms of the pancreas. Am J Pathol. 2011;351:1812–7.CrossRefGoogle Scholar
  8. 8.
    Urata T, Naito Y, Nagamine M, et al. Intraductal tubulopapillary neoplasm of the pancreas with somatic BRAF mutation. Clin J Gastroenterol. 2012;5:413–20.CrossRefGoogle Scholar
  9. 9.
    Basturk O, Adsay V, Askan G, et al. Intraductal tubulopapillary neoplasm of the pancreas: a clinicopathologic and immunohistochemical analysis of 33 cases. Am J Surg Pathol. 2017;41:313–25.CrossRefGoogle Scholar
  10. 10.
    Kuscher S, Steinle H, Soleiman A, et al. Intraductal tubulopapillary neoplasm (ITPN) of the pancreas associated with an invasive component: a case report with review of the literature. World J Surg Oncol. 2017;15:203.CrossRefGoogle Scholar
  11. 11.
    Kölby D, Thilén J, Andersson R, et al. Multifocal intraductal tubulopapillary neoplasm of the pancreas with total pancreatectomy: report of a case and review of literature. Int J Clin Exp Pathol. 2015;8:9672–80.PubMedPubMedCentralGoogle Scholar
  12. 12.
    Pea A, Yu J, Rezaee N, et al. Targeted DNA sequencing reveals patterns of local progression in the pancreatic remnant following resection of intraductal papillary mucinous neoplasm (IPMN) of the pancreas. Ann Surg. 2017;266:133–41.CrossRefGoogle Scholar
  13. 13.
    Fujita Y, Kitago M, Masugi Y, et al. Two cases of pancreatic ductal adenocarcinoma with intrapancreatic metastasis. World J Gastroenterol. 2016;22:9222–8.CrossRefGoogle Scholar

Copyright information

© Japanese Society of Gastroenterology 2018

Authors and Affiliations

  1. 1.Department of SurgeryIwate Medical UniversityMoriokaJapan
  2. 2.Department of Molecular Diagnostic PathologyIwate Medical UniversityMoriokaJapan

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