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Table 1 Differential diagnosis of malignant pancreatic neoplasms. Overview of pancreatic neoplasms with their relative prevalence, direction of differentiation, macroscopic and microscopic appearance, and immunohistochemical markers

From: Surgical and molecular pathology of pancreatic neoplasms

 

Prevalence (% of all malignant pancreatic tumors)

Mean Age (SD) in years

Sex predominance

Direction of differentiation Ductal/acinar/endocrine

Gross: Solid/solid and cystic/cystic

Microscopic

Immunohistochemical

Pancreatic ductal adenocarcinoma

90 %

66 (11)

Male (3:2)

Ductal

Solid

• Glandular and ductal structures

• Abundant desmoplastic stroma

• Eosinophilic to clear cytoplasm and enlarged pleomorphic nuclei

• Perineural, lymphatic and blood vessel invasion

Aberrant TP53 expression, SMAD4 loss, expression of MUC1, MUC3, MUC4, MUC5AC, CA19-9

Pancreatic neuroendocrine tumor/carcinoma

5 %

58 (15)

Male (3:2)

Endocrine

Solid, sometimes cystic degeneration

• Nested or trabecular growth pattern

• Granular amphophilic to eosinophilic cytoplasm

• “Salt and pepper” chromatin

Expression of synaptophysin and chromogranin, peptide hormones (e.g. insulin and glucagon), aberrant nuclear TP53 expression in PanNECs

Solid-pseudopapillary neoplasm

1–2 %

29 (14)

Female (9:1)

Uncertain

Solid and cystic

• Poorly cohesive uniform cells

• Extensive degenerative changes.

• Eosinophilic or clear vacuolated cytoplasm Round to oval nuclei, often grooved or indented.

• Eosinophilic globules and foamy macrophages

Abnormal nuclear labeling for β-catenin, expression of CD10, paranuclear dot-like CD99 labeling or lymphoid enhancer-binding factor 1 (LEF1). Loss of membranous E-cadherin

Acinar cell Carcinoma

1–2 %

56 (15) 6 % between 8 and 15

Male (2:1)

Acinar

Solid, sometimes cystic degeneration

• Enlarged uniform nuclei with prominent nucleoli

• Finely granular eosinophilic cytoplasm.

• Small acinar units or sheets

BCL10, expression of pancreatic exocrine enzymes: trypsin, chymotrypsin, lipase

Pancreatoblastoma

<1 %

5 (2), second peak around 40

Slightly male

Acinar

Solid, cystic in BWS a

• Similar to ACC

• Squamoid nests required for diagnosis

• Neuroendocrine or ductal component.

Expression of pancreatic exocrine enzymes, BCL10, SMAD4 loss, Abnormal nuclear labeling for β-catenin

  1. a BWS Beckwith-Wiedemann syndrome