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Solid and Papillary Epithelial Neoplasm (SPEN), also called Solid Pseudopapillary Neoplasm (SPN), is a rare low-grade malignant epithelial neoplasm of the pancreas characterized by solid and cystic components, with hemorrhagic degeneration and pseudopapillary architecture.

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• Accounts for <5% of exocrine pancreatic tumors.

Etiopathology

• Cell of origin: Unclear; likely from multipotent pancreatic progenitor cells with aberrant differentiation.
• Genetics:
  • Associated with CTNNB1 gene mutations (β-catenin pathway activation).
  • Nuclear β-catenin and loss of membranous E-cadherin expression are characteristic.
• Histology:
  • Poorly cohesive epithelial cells → degeneration → pseudopapillary structures.
  • Hyaline globules, hemorrhage, cholesterol clefts common.
• Immunohistochemistry: Positive for β-catenin (nuclear), vimentin, CD10, PR; variable CK.

Clinical features

Demographics:

• Strong female predominance (90%).
• Young patients: 20–30 years (“tumor of young women”).

Location:

• Pancreatic body and tail > head.

Clinical features:

• Often large mass at presentation (mean 8–10 cm).
• Abdominal pain, palpable mass.
• Rarely jaundice (if in head).
• Sometimes incidental finding.