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    Mucinous Cystic Neoplasm of the Pancreas

    Definition

    • Cystic mucinous neoplasm with ovarian type stroma and lacking communication with the pancreatic ducts

    Alternate/Historical Names

    • Mucinous cystic tumor

    Diagnostic Criteria

    • Grossly or radiographically visible lesion
      • 95% in body or tail of pancreas
    • Usually multilocular
      • Cysts variably sized
        • Usually 1 to several cm
      • Frequently one dominant thick walled cystic structure
        • Frequently contains multiple smaller daughter cysts in wall
    • Lined by tall columnar cells
      • Basal nuclei
      • Abundant intracellular mucin
        • Mucin may be depleted in high grade lesions
      • May be partially denuded or lined by atrophic cells
      • Goblet cells frequently admixed
      • Scattered neuroendocrine cells in half of cases
      • Focal gastric, intestinal or squamous differentiation may be seen
      • Varying degrees of cytologic atypia and architectural complexity
    • Ovarian type stroma
      • Inner layer moderately to densely cellular
        • Spindled cells with bland round to oval nuclei
        • May be luteinized
        • May be sparse or absent in patients >60
        • May be sparse in large lesions
      • Outer layer densely collagenous
      • May calcify
    • Lacks communication to pancreatic ducts
    • Virtually all cases in females
    • Separated into adenoma grade, borderline grade, carcinoma in situ grade
      • See Grading for specific features
    • Up to 1/3 have associated carcinoma
      • Usually ductal
      • Not colloid (mucinous)
      • Entire lesion should be submitted to evaluate for associated carcinoma
    • Rare cases with sarcomatous stroma
      • With or without heterologous elements

    Reetesh Pai MD
    Robert V Rouse MD
    Department of Pathology
    Stanford University School of Medicine
    Stanford CA 94305-5342

    Original posting/updates: 2/1/07, 6/16/10, 1/2/12

    Supplemental studies

    Immunohistology

    Ovarian type stroma
    Inhibin 90%
    Smooth muscle actin 90%
    Progesterone receptor 80%
    Estrogen receptor 30-50%

    Differential Diagnosis

    Intraductal Papillary Mucinous Neoplasm Mucinous Cystic Neoplasm of the Pancreas
    Usually over age 50 Usually under age 50
    Male>female Nearly all female
    Involves segments of ducts Does not communicate with ducts
    Multiple adjacent cystic spaces If multilocular, usually many smaller cysts within a circumscribed larger cyst
    Extensive papillae formation Minimal papillae
    No ovarian type stroma Ovarian type stroma
    May be associated with colloid carcinoma No association with colloid carcinoma

    Mucinous Cystic Neoplasm of the Pancreas Intraductal Oncocytic Papillary Neoplasm
    Lacks oncocytic epithelium Oncocytic epithelium
    Lacks intracytoplasmic lumens and cribriform pattern Prominent cribriform pattern formed by intracytoplasmic lumens
    Complex papillae not seen Complex arborizing papillae
    Cellular ovarian type stroma Paucicellular stroma
    No communication with ducts Communicates with duct system

    Intraductal Tubular Adenoma Mucinous Cystic Neoplasm of the Pancreas
    Nodular intraductal growth Mucin filled cyst or cysts
    Low grade atypia Atypia may vary from low to high
    Males and females affected Nearly all female
    Involves segments of ducts Does not communicate with ducts
    Extensive tubule formation No tubules
    No ovarian type stroma Ovarian type stroma

    Intraductal Tubular Carcinoma Mucinous Cystic Neoplasm of the Pancreas
    Nodular intraductal growth Mucin filled cyst or cysts
    Males and females affected Nearly all female
    Involves segments of ducts Does not communicate with ducts
    Extensive tubule formation No tubules
    No ovarian type stroma Ovarian type stroma

    Serous Macrocystic / Oligocystic Adenoma Mucinous Cystic Neoplasm of the Pancreas
    Cuboidal clear epithelial lining Mucinous lining
    Occurs in males and females Nearly all female
    Lack ovarian type stroma Ovarian type stroma

    Pancreatic Pseudocyst Mucinous Cystic Neoplasm of the Pancreas
    Lack epithelial lining Epithelial lining (may be focal in degenerate cases)
    Male>female Nearly all female
    Lack ovarian type stroma Ovarian type stroma

    Clinical

    • Virtually all cases in females
      • Nearly all cases reported in males lack ovarian type stroma
        • Many may be better classified as intraductal papillary mucinous neoplasm
    • Most cases in age range 40-50
    • Up to 1/3 associated with invasive ductal carcinoma
    • Entire lesion should be examined microscopically
      • If entirely submitted, noninvasive mucinous cystic neoplasms have no risk of metastasis
      • If not entirely submitted, risk is about 5% for recurrence or metastasis

    Grading / Staging / Report

    • Grading
      • Adenoma grade
        • Single layer of mucinous columnar cells
        • Flat or irregular undulations
          • No well formed papillae
        • No cytologic atypia
      • Borderline grade
        • Papillary projections
        • Pseudostratification
        • Nuclear crowding
        • Moderate cytologic atypia
          • Irregular hyperchromatic nuclei
          • One or more nucleoli
        • May be focal
      • Carcinoma in situ grade
        • Multilayered epithelium
        • Cribriform architecture
        • Marked nuclear pleomorphism
        • Frequent mitotic figures
        • Loss of intracytoplasmic mucin
        • May be focal
    • Staging is not relevant unless invasive carcinoma is present
      • If present, follow TNM staging for carcinoma
    • Report
      • Grade
      • Presence or absence of invasive carcinoma
        • Give type and size
      • For noninvasive lesions
        • Note that there appears to be about a 5% risk of developing carcinoma even if margins negative
        • Suggest clinical followup

    Lists

    Cystic Pancreatic Lesions

    Mucinous Pancreatic Lesions

    Bibliography

    • Hruban RA, Pitman MB, Klimstra DS. Tumors of the Pancreas, Atlas of Tumor Pathology, AFIP Fourth Series, Fascicle 6, 2007.
    • Bosman FT, Carneiro F, Hruban RH, Thiese ND (Eds). WHO Classifiication of Tumors of the Digestive System, IARC, Lyon 2010.
    • Basturk O, Coban I, Adsay NV. Pancreatic cysts: pathologic classification, differential diagnosis, and clinical implications. Arch Pathol Lab Med. 2009 Mar;133(3):423-38.
    • Crippa S, Salvia R, Warshaw AL, Domínguez I, Bassi C, Falconi M, Thayer SP, Zamboni G, Lauwers GY, Mino-Kenudson M, Capelli P, Pederzoli P, Castillo CF. Mucinous cystic neoplasm of the pancreas is not an aggressive entity: lessons from 163 resected patients. Ann Surg. 2008 Apr;247(4):571-9.
    • Volkan Adsay N. Cystic lesions of the pancreas. Mod Pathol. 2007 Feb;20 Suppl 1:S71-93.
    • Zamboni G, Scarpa A, Bogina G, Iacono C, Bassi C, Talamini G, Sessa F, Capella C, Solcia E, Rickaert F, Mariuzzi GM, Klöppel G. Mucinous cystic tumors of the pancreas: clinicopathological features, prognosis, and relationship to other mucinous cystic tumors. Am J Surg Pathol. 1999 Apr;23(4):410-22.
    • Wilentz RE, Albores-Saavedra J, Zahurak M, Talamini MA, Yeo CJ, Cameron JL, Hruban RH. Pathologic examination accurately predicts prognosis in mucinous cystic neoplasms of the pancreas. Am J Surg Pathol. 1999 Nov;23(11):1320-7.
    • Thompson LD, Becker RC, Przygodzki RM, Adair CF, Heffess CS. Mucinous cystic neoplasm (mucinous cystadenocarcinoma of low-grade malignant potential) of the pancreas: a clinicopathologic study of 130 cases. Am J Surg Pathol. 1999 Jan;23(1):1-16.
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