Pancreas
Cystic and intraductal lesions
Intraductal tubulopapillary neoplasm


Topic Completed: 8 June 2020

Minor changes: 23 October 2020

Copyright: 2002-2020, PathologyOutlines.com, Inc.

PubMed Search: Intraductal tubulopapillary neoplasm[title]

Gokce Askan, M.D.
Olca Basturk, M.D.
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Cite this page: Askan G, Basturk O. Intraductal tubulopapillary neoplasm. PathologyOutlines.com website. http://www.pathologyoutlines.com/topic/pancreasitpn.html. Accessed October 29th, 2020.
Definition / general
  • Intraductal, grossly solid and cystic, tubule forming, epithelial neoplasm with high grade dysplasia and no overt mucin production (Am J Surg Pathol 2017;41:313)
  • First described in 2004 by Tajiri et al. under the name of intraductal tubular carcinoma (Pancreas 2005;30:115)
  • Renamed in 2009 by Jamaguchi et al. as intraductal tubulopapillary neoplasm (Am J Surg Pathol 2009;33:1164)
  • Previous (2010) and current (2019) WHO classify as a distinct intraductal neoplasm of the pancreas
  • Accounts for 3% of intraductal neoplasms of the pancreas (Am J Surg Pathol 2011;35:1812)
Essential features
  • A rare and distinct type of pancreatic intraductal neoplasm
  • Highly cellular, complex tubular or cribriform growth pattern
  • Focal papillary growth may be seen
  • Intracellular mucin is typically not detectable or is very minimal
  • Positive for MUC1 and MUC6; negative for MUC5AC
  • Lacks genetic alterations commonly seen in intraductal papillary mucinous neoplasms (IPMNs)
  • Favorable overall outcome even if associated with invasive carcinoma (Am J Surg Pathol 2017;41:313)
Terminology
  • Older term is intraductal tubular carcinoma (not recommended)
ICD coding
  • ICD-O:
    • 8503/2 - Intraductal tubulopapillary neoplasm
    • 8503/3 - Intraductal tubulopapillary neoplasm with associated invasive carcinoma
Epidemiology
Sites
Etiology
  • No known etiological factors
Clinical features
  • Nonspecific symptoms: abdominal pain, weight loss, nausea, vomiting, steatorrhea
  • Jaundice is not common
Radiology description
Prognostic factors
Case reports
Treatment
  • Treated primarily by surgical resection
Gross description
Gross images

Images hosted on other servers:

Polypoid mass within dilated main pancreatic duct

Microscopic (histologic) description
  • Circumscribed nodules of back to back tubular glands surrounded by fibrotic stroma; however, the intraductal location of at least some of the nodules is evidenced by continuity of the neoplastic epithelium with nonneoplastic ductal epithelium
  • Tubules are lined by cuboidal or columnar epithelium with a modest amount of eosinophilic or amphophilic cytoplasm
  • Nuclei are small, round to oval and moderately to markedly atypical with readily identifiable mitotic figures
  • Intracellular mucin production is typically not detectable or is very minimal
  • Intraluminal secretions and necrosis, often with comedo-like pattern, are present
  • Clear cell changes, cartilaginous or osseous metaplasia may be seen (Diagn Pathol 2014;9:11, Pathol Int 2012;62:339, Am J Surg Pathol 2017;41:313, Pathol Int 2015;65:501)
  • Associated invasive carcinoma is seen in about 70% of cases as individual cells or small angulated nonmucinous glands surrounded by usually desmoplastic stroma (Am J Surg Pathol 2017;41:313)
Microscopic (histologic) images

Contributed by Gokce Askan, M.D. and Olca Basturk, M.D.

Nodules of back to back tubular glands

Intraductal growth pattern

Sharply circumscribed nest formation

Tightly packed tubules lined by atypical cuboidal epithelium

Comedo-like necrosis

Clear cell changes


Osseous metaplasia

Invasion

MUC1 expression

MUC6 expression

Cytology description
Negative stains
Molecular / cytogenetics description
Sample pathology report
  • Pancreas and duodenum, pancreaticoduodenectomy:
    • Intraductal tubulopapillary neoplasm of pancreas (ITPN) (see comment)
    • Comment: The pancreas was submitted entirely for microscopic evaluation and no associated invasive carcinoma is identified. The neoplasm involves the main pancreatic duct and measures 4 cm in greatest dimension. Adjacent pancreas reveals atrophy. Surgical margins are free of neoplasm. 15 benign lymph nodes are identified (0/15).
  • Pancreas and duodenum, pancreaticoduodenectomy:
    • Intraductal tubulopapillary neoplasm of pancreas (ITPN) associated with an invasive ductal carcinoma (see comment)
    • Comment: ITPN involves the main pancreatic duct. The entire tumor measures 5 cm in greatest dimension; the invasive carcinoma component measures 0.7 cm in greatest dimension (suggestion for if multifocal invasion is present: the invasive carcinoma accounts for 25% of entire tumor and is estimated to measure 1.25 cm in greatest dimension). Adjacent pancreas reveals atrophy. Surgical margins are free of ITPN and invasive carcinoma. 14 benign lymph nodes are identified (0/14).
  • Reference: Ann Surg 2016;263:162
Differential diagnosis
Board review style question #1
Which of the following is true about intraductal tubulopapillary neoplasm of the pancreas?



  1. Jaundice is one of the most common symptoms
  2. Papilla and mucin formation are common histologic features
  3. They are positive for MUC5AC
  4. They commonly have KRAS, GNAS and BRAF mutations
  5. They have relatively indolent prognosis even when associated with invasive carcinoma
Board review answer #1
E. They have relatively indolent prognosis even when associated with invasive carcinoma

Reference: Intraductal tubulopapillary neoplasm (ITPN)

Comment Here
Board review style question #2
Which one of the following stains is negative in intraductal tubulopapillary neoplasm of the pancreas?

  1. CK7
  2. CK19
  3. MUC5AC
  4. MUC1
  5. MUC6
Board review answer #2
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