Solid-pseudopapillary neoplasm

Solid-pseudopapillary neoplasm
Updated: 03/26/2019
© Jun Wang, MD, PhD

General features
  • AKA papillary and solid epithelial neoplasm, papillary cystic neoplasm, Gruber-Frantz tumor
  • Low grade malignancy
  • Commonly 30-35 year old women, not associated with any clinical syndrome
  • Poor prognostic factors: Vascular invasion, high nuclear grade, “necrobiotic nests”
  • Metastases in 10-15%
Clinical presentations
  • Epigastric pain, nausea, vomiting, weight loss, etc
Key pathological features
Molecular abnormality
  • Gain-of-function mutation of beta-catenin
Treatment
  • Surgery


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