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
- Poorly cohesive monomorphic epithelial cells forming solid and pseudopapillary structures
- Pseudopapillae with fibrovascular cores
- Several layers of bland fragile epithelial cells with clear to eosinophilic cytoplasm
- Cysts formed due to solid nests minus cells degenerating away from the small vessels
Molecular abnormality
- Gain-of-function mutation of beta-catenin
Treatment
- Surgery
Back to contents
Comments
Post a Comment