Definition
Carcinoma of the pancreas exhibiting multiple lines of differentiation including acinar and squamous
Alternate/Historical Names
Infantile pancreatic carcinoma
Pancreatic carcinoma of childhood
Pancreaticoblastoma
Diagnostic Criteria
2/3 of cases under age 5 Usually well circumscribed
May infiltrate
Cellular lobules separated by fibrous bands
Some congenital cases may be cystic
Predominant acinar component
Sheets of cells merge with acinar formations
Sharp cell borders
Nuclei central to basal
Prominent nucleoli
Cytoplasm may be amphophilic to granular eosinophilic
Positive for trypsin, chymotrypsin and lipase (variable)
Squamoid nests required for diagnosis
Abundant eosinophilic cytoplasm
May be vague or with overt keratinization
Frequently with biotin rich optically clear nuclei
Endocrine component in 2/3 of cases
May be detectable only with stains
Ductal / glandular component occasionally present
Usually focal
May be mucin positive
Variably cellular stroma
More cellular in pediatric cases
Rarely forms bone or cartilage
Rarely appears neoplastic
Primitive round cell component rarely present
Uniform immature appearing small cells
Robert V Rouse MD
Original posting/updates: 1/6//08, 1/2/12
Supplemental studies
Immunohistology
Acinar component
Trypsin, chymotrypsin positive
Lipase variable
Alpha fetoprotein variable
Squamoid nests
CK 8, 18, 19 positive
CK5/6 occasionally focal
Endocrine differentiation
Chromogranin, synaptophysin positive scattered cells in 2/3 of cases
Islet polypeptides usually negative
Differential diagnosis
Pancreatoblastoma Solid Pseudopapillary Neoplasm of the Pancreas
Both male and female
Overwhelmingly female
2/3 <5 years of age
Very rare <5
Trypsin, chymotrypsin positive
Trypsin, chymotrypsin negative
Keratin positive
Keratin variable
Lacks pseudopapillary architecture
Frequent pseudopapillary architecture
May have true lumens
No true lumens
Squamoid nests always present
Lacks squamous differentiation
Frequently increased mitotic figures
Infrequent mitotic figures
Pancreatoblastoma Well Differentiated Pancreatic Neuroendocrine (Islet Cell) Tumor
Trypsin, chymotrypsin positive
Trypsin, chymotrypsin negative
Squamoid nests always present
Lacks squamous differentiation
Usually prominent nucleoli
Salt and pepper chromatin
Chromogranin, synaptophysin scattered positive
Chromogranin or synaptophysin widespread staining
Islet polypeptide markers negative or very focal
Islet polypeptide markers frequently positive
Cellular stroma frequent
Lacks cellular stroma
Clinical
Most cases under age 5
Congenital cases reported with Beckwith-Wiedemann syndrome
1/3 of cases in adults
Pediatric cases curable if resectable, otherwise aggressive
Adult cases aggressive
Lipase hypersecretion syndrome not reported
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Lists
Pancreatic Carcinomas
Pancreatic Acinar Cell Lesions
Bibliography
Solcia E, Capella C, Kloppel G . Tumors of the Pancreas, Atlas of Tumor Pathology, AFIP Third Series, Fascicle 20, 1997.
Bosman FT, Carneiro F, Hruban RH, Thiese ND (Eds). WHO Classifiication of Tumors of the Digestive System, IARC, Lyon 2010.
Klimstra DS, Wenig BM, Adair CF, Heffess CS. Pancreatoblastoma. A clinicopathologic study and review of the literature. Am J Surg Pathol. 1995 Dec;19(12):1371-89.
Abraham SC, Wu TT, Klimstra DS, Finn LS, Lee JH, Yeo CJ, Cameron JL, Hruban RH. Distinctive molecular genetic alterations in sporadic and familial adenomatous polyposis-associated pancreatoblastomas : frequent alterations in the APC/beta-catenin pathway and chromosome 11p. Am J Pathol. 2001 Nov;159(5):1619-27.
Klimstra DS. Nonductal neoplasms of the pancreas. Mod Pathol. 2007 Feb;20 Suppl 1:S94-112.
