Pancreas
Exocrine tumors / carcinomas
Mucinous pancreatic tumors

Author: Katrina Krogh, M.D.
Senior Author: Guang-Yu Yan​g, M.D., Ph.D.
Editorial Board Member Reivew: Raul S. Gonzalez, M.D.
Deputy Editor Review: Debra Zynger, M.D.

Revised: 15 October 2018, last major update June 2018

Copyright: (c) 2002-2018, PathologyOutlines.com, Inc.

PubMed Search: Mucinous tumors[title] pancreas

Cite this page: Krogh, K. Mucinous pancreatic tumors. PathologyOutlines.com website. http://www.pathologyoutlines.com/topic/pancreasmucin.html. Accessed November 12th, 2018.
Definition / general
  • Neoplasms within the pancreas characterized by predominant mucin production by the tumor cells
  • Mucus: a substance that is 95 - 98% water and 2 - 5% solids, with > 60% of the solid component consisting of mucin (World J Gastroenterol 2011;17:4757)
  • Mucin: a glycoprotein consisting of a polypeptide backbone with attached oligosaccharide conjugates
Essential features


Comparison of essential features of the four types of pancreatic mucinous tumors
Pancreatic ductal adenocarcinoma MCN IPMN Simple Mucinous Cyst / MNC
Age, decade 7th - 8th 4th - 5th 6th - 7th 6th - 7th
Location Head > body / tail Body / tail > head Head > body / tail Head = body = tail
Sex predilection M > F F >>> M M > F F > M
CEA N / A
Amylase N / A Low Low
Pancreatic duct communication Yes No Yes Rare
Associated with invasive carcinoma N / A Generally no Yes Yes
Cytologic atypia N / A Low grade - high grade Low grade - high grade No
Epithelium type Flat or papillary Flat or papillary Papillary Flat
Ovarian stroma N / A Yes No No
Molecular mutations KRAS, TP53, CDKN2A, SMAD4 KRAS, SMAD4, TP53 (in situ) GNAS, KRAS, RNF43, TP53 KRAS



Epidemiology
Sites
Pathophysiology
  • Pancreatic ductal adenocarcinoma
    • Arises from pancreatic duct epithelia
  • Mucinous cystic neoplasm (MCN)
  • Intraductal papillary mucinous neoplasm (IPMN)
  • Simple mucinous cyst / mucinous nonneoplastic cyst (MNC)
Etiology
Clinical features
  • Pancreatic ductal adenocarcinoma
    • Weight loss, painless jaundice, diabetes, malaise, coexisting pancreatitis
  • Mucinous cystic neoplasm (MCN)
    • Clinical presentation depends on the size of tumor; > 3 cm can cause compression of adjacent structures
    • Associated with diabetes mellitus
  • Intraductal papillary mucinous neoplasm (IPMN)
    • Majority are asymptomatic; often incidental
    • Can give rise to invasive adenocarcinoma
    • Some patients present with epigastric pain, weight loss, diabetes and jaundice
  • Simple mucinous cyst / mucinous nonneoplastic cyst (MNC)
Diagnosis
  • Pancreatic ductal adenocarcinoma
    • Endoscopic ultrasound guided fine needle aspiration (EUS-FNA), histologic analysis upon surgical resection
  • Mucinous cystic neoplasm (MCN)
    • Cytology, fine needle aspiration (FNA), cyst fluid analysis
  • Intraductal papillary mucinous neoplasm (IPMN)
  • Simple mucinous cyst / mucinous nonneoplastic cyst (MNC)
    • FNA, cyst fluid analysis, histologic analysis
  • Laboratory
    • Pancreatic ductal adenocarcinoma
      • Serum tumor markers include CA 19-9, CEA
      • Cyst fluid has high levels of CA 19-9, DUPAN-2, CEA, SPan-1
    • Mucinous cystic neoplasm (MCN)
      • Serum tumor markers include CEA, CA 19-9, TAG-72, CA 15-3 or MCA and low levels of amylase
    • Intraductal papillary mucinous neoplasm (IPMN)
      • Serum amylase and lipase are elevated
    • Simple mucinous cyst / mucinous nonneoplastic cyst (MNC)
    Prognostic factors
    • Pancreatic ductal adenocarcinoma
      • Most ductal adenocarcinomas are fatal (Cancer Res 2014;74:2913)
      • Survival time is longer in patients with tumors confined to the pancreas or < 3 cm in diameter
      • Tumors in the body or tail tend to present at a more advanced stage
    • Mucinous cystic neoplasm (MCN)
      • Classified as benign or low grade (IARC: 8470/0 mucinous cystoma)
      • Head lesions are uncommon but have a predilection to be a mucinous cystadenocarcinoma
    • Intraductal papillary mucinous neoplasm (IPMN)
    • Simple mucinous cyst / mucinous nonneoplastic cyst (MNC)
    Case reports
    Treatment
    • Pancreatic ductal adenocarcinoma
      • Radical resection or chemotherapy
    • Mucinous cystic neoplasm (MCN)
      • Surgical resection
    • Intraductal papillary mucinous neoplasm (IPMN)
      • Surgical resection
    • Simple mucinous cyst / mucinous nonneoplastic cyst (MNC)
      • Conservative treatment
    Gross description
    • Pancreatic ductal adenocarcinoma
      • Firm, poorly defined mass, with yellow to white cut surface
      • Can be difficult to differentiate from background pancreatitis
    • Mucinous cystic neoplasm (MCN)
      • 2 - 35 cm in greatest dimension
      • Unilocular or multilocular, thick walls
      • Round mass with smooth surface and fibrous pseudocapsule
      • Calcifications are frequent
    • Intraductal papillary mucinous neoplasm (IPMN)
      • 1 - 8 cm in greatest dimension
      • Cystic, multiloculated
      • Grape-like clusters in branch type
    • Simple mucinous cyst / mucinous nonneoplastic cyst (MNC)
    Gross images

    Images hosted on PathOut server:

    Contributed by Katrina Krogh, M.D.

    PDA: ill defined white lesion

    MCN: unilocular cyst with thick wall

    IPMN: small cysts with smooth linings

    MNC: unilocular
    simple cyst with
    smooth thin lining

    Microscopic (histologic) description
    Microscopic (histologic) images

    Images hosted on PathOut server:

    Contributed by Katrina Krogh, M.D.

    Pancreatic adenocarcinoma, grade 2

    Pancreatic mucinous adenocarcinoma

    MCN: ovarian type stroma

    IPMN, gastric type

    IPMN, intestinal type

    Simple mucinous
    cyst / mucinous
    nonneoplastic
    cyst (MNC)

    Positive stains
    Negative stains
    Molecular / cytogenetics description
    Board review question #1
    Which of the following statements about this pancreatic lesion is true?

    1. Serum amylase and lipase are typically depressed
    2. This lesion is more common than pancreatic ductal adenocarcinoma
    3. This lesion is more commonly found in males
    4. This lesion typically contains ovarian stroma
    Board review answer #1
    C. This lesion is more commonly found in males (true for pancreatic intraductal papillary mucinous tumor (IPMN) which is shown here)
    Board review question #2
    Which of the following statements about this pancreatic lesion is true?

    1. This lesion can undergo immunophenotypic sex cord stromal differentiation
    2. This lesion does not undergo malignant transformation
    3. This lesion is more common than pancreatic ductal adenocarcinoma
    4. This lesion is seen equally in males and females
    Board review answer #2
    A. This lesion can undergo immunophenotypic sex cord stromal differentiation (true for pancreatic mucinous cystic neoplasm which is shown here)