Solid pseudopapillary neoplasm
- Uncommon tumour (1 to 2% of pancreatic exocrine neoplasms) of low malignant potential
- Predominantly in adolescent girls and young women
- Slight preference for pancreatic tail
- Histogenesis unclear – possible genital ridge origin
- Somatic mutation in exon 3 of CTNNB1 gene
- A rare subset of cases may be associated with familial adenomatous polyposis
- Well-demarcated tumours with solid areas, pseudocystic spaces and haemorrhagic necrosis
- The presence of hyalinised vascular stalk with clinging neoplastic cells is a key morphologic feature
- Highly divergent immunohistochemical profile
- Positive: Beta-catenin (nuclear), cyclin D1, alpha-1 antitrypsin, CD56, CD10, PR receptor, synaptophysin (focal and a pitfall)
- Recent studies show high rate of diffuse nuclear expression of LEF1, AR and TFE3 (1)
- Kim, E. K., Jang, M., Park, M., & Kim, H. (2017). LEF1, TFE3, and AR are putative diagnostic markers of solid pseudopapillary neoplasms. Oncotarget, 8(55), 93404–93413. https://doi.org/10.18632/oncotarget.21854
Remaining information cited from: Klöppel, G., Klimstra, D. S., Basturk, O., Notohara, K. Solid pseudopapillary neoplasm. (2019). In: WHO Classification of Tumours: Digestive system tumours (5th edn). Lyon; IARC.