Solid Pseudopapillary Neoplasm of

Pancreas
Rawa Muhsin
Etiology
Low-grade malignant neoplasm of uncertain cellular
differentiation
Electron microscopy shows evidence of epithelial
differentiation
90-100% harbor mutations in CTNNB1 gene
Clinical
Most patients in 20s and 30s
Female predominance (10-20:1)
Evenly distributed throughout pancreas
Vague abdominal symptoms
Indolent
10-15% metastasize (liver, peritoneum, lymph nodes)
Prognosis excellent
Gross
Large solitary mass
Well circumscribed
Solid to cystic, usually mixed
White-gray to yellow cut surface
Evenly distributed throughout pancreas
Microscopic
Solid monomorphic sheets
Delicate vessels with hyalinized or myxoid stroma
Low mitotic rate
Perineural and true vascular invasion quite rare
Marked degenerative changes (pseudopapillae, foamy
macrophages, cholesterol clefts, hemorrhage,
lipofuscin or melanin pigment, calcification, areas of
infarction)
Infiltration of adjacent parenchyma is common
"Blood lakes" common at periphery of neoplasm
Cytologic
Nuclei oriented away from vessels with zone of
cytoplasm separating nuclei from capillaries
Uniform and round to oval with finely dispersed
nuclear chromatin
Often with longitudinal nuclear grooves
Moderate amount of eosinophilic cytoplasm but can be
clear with vacuoles
Intracytoplasmic eosinophilic hyaline globules
 Immunohistochemistry

Antibody Pattern % Cases

Beta-catenin Nuclear 99% 524
CD10 Membranous 93% 181
CD56 Membranous 98% 152
CD99 Membranous 98% 110
Progesterone Nuclear 100% 96
Cyclin D1 Nuclear 78% 162
E-cadherin Membranous 8% 319
Immunohistochemistry
Antibody % Cases
CD56 98% 152
NSE 92% 76
Synaptophysin 68% 288
Chromogranin 12% 161
INSM1 9% 11
Molecular
Missense mutations in exon 3 of CTNNB1 in nearly all
cases
Prevents destruction of beta-catenin
Differential diagnoses
Pancreatic neuroendocrine tumor
Nuclear features
IHC panel
Acinic cell carcinoma
Pattern
IHC panel
Pseudocyst
Serous neoplasms