solid pseudopapillary tumor of the pancreas
Solid pseudopapillary neoplasms of the pancreas are rare pancreatic tumors with mostly benign behavior, affecting almost exclusively women. Their histogenetic origin is still unsolved.
Immunochemistry
deregulated expression of cell cycle-associated proteins (11235905)
Cytogenetics
t(11;22)(q24;q12) translocation (11000339)
Molecular biology
No EWSR1 rearrangements in 30 cases (16941013)
EWSR1/FLI-1 fusion transcript (not confirmed) (11000339)
beta-catenin gene (CTNNB1) mutation (83%) (11943721, 11731417)
Differential diagnosis
adrenal pheochromocytoma (15182415)
References
Tiemann K, Kosmahl M, Ohlendorf J, Krams M, Klöppel G. Solid pseudopapillary neoplasms of the pancreas are associated with FLI-1 expression, but not with EWS/FLI-1 translocation. Mod Pathol. 2006 Nov;19(11):1409-13. PMID: 16941013
Tang LH, Aydin H, Brennan MF, Klimstra DS. Clinically aggressive solid pseudopapillary tumors of the pancreas: a report of two cases with components of undifferentiated carcinoma and a comparative clinicopathologic analysis of 34 conventional cases. Am J Surg Pathol. 2005 Apr;29(4):512-9. PMID: 15767807
Klimstra DS, Wenig BM, Heffess CS. Solid-pseudopapillary tumor of the pancreas: a typically cystic carcinoma of low malignant potential. Semin Diagn Pathol. 2000 Feb;17(1):66-80. PMID: 10721808
Lieber MR, Lack EE, Roberts JR Jr, Merino MJ, Patterson K, Restrepo C, Solomon D, Chandra R, Triche TJ. Solid and papillary epithelial neoplasm of the pancreas. An ultrastructural and immunocytochemical study of six cases. Am J Surg Pathol. 1987 Feb;11(2):85-93. PMID: 3812876