pancreatic solid pseudopapillary tumor
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Cases - Digital slides
Case 45 (HPC:45)
Case 67 (HPC:67)
Case 68 (HPC:68) (In multiple endocrine neoplasia type 1 - MEN 1)
Case 69 (HPC:69)
Case 75 (HPC:75)
Case 96 (HPC:96)
Case 100 (HPC:100)
Case 199 (HPC:199)
Definition: Solid pseudopapillary neoplasms of the pancreas are rare pancreatic tumors with mostly benign behavior, affecting mainly women. Their histogenetic origin is still unsolved. It usually associated with a good prognosis.
Pancreatic solid pseudopapillary tumor is a usually benign neoplasm with predominant manifestation in young women, composed of monomorphic cells forming solid and pseudopapillary structures, frequently showing haemorrhagic-cystic changes and variably expressing epithelial, mesenchymal and endocrine markers.
In contrast to other pancreatic tumours, aberrant activation of the Wnt-beta-catenin pathway appears to be a constant feature in SPN.
Definition: Pancreatic epithelial tumor of low malignant potential mostly of young women with solid, cystic and papillary architecture and acinar, ductal and endocrine differentiation.
Clinical Features
Usually young women
most commonly a palpable abdominal mass
Low malignant potential
- metastasizing tumors have exhibited a greater incidence of venous invasion, high nuclear grade, and necrosis
Macroscopy
Usually large
areas of hemorrhage and necrosis
predominantly cystic
surrounded by a well-developed capsule
+/- edges a solid infiltrative neoplasm
Multicentricity exceptionally rare
A few cases adjacent to but anatomically separate from the pancreas
Microscopy
Very cellular
Simulates the appearance of a pancreatic endocrine neoplasm
Most distinctive feature:
- pseudopapillae covered by several layers of epithelial cells
Nuclei: ovoid, folded, indistinct nucleoli, few mitoses
May be hyaline globules and collections of foamy cells
Fibrovascular core:
- thick
- often shows prominent mucinous changes (of diagnostic importance)
accumulation of myxoid material around the vessels.
Ultrastructure
+/- evidence of acinar, ductal, and (sometimes) endocrine cell differentiation
Immunochemistry
alpha-1-antitrypsin + (100%)
Vimentin + (100%)
Calretinin + (100%)
AE1/AE3 +/- (50%)
CAM5.2 - (10%)
Synaptophysin +/- (40%)
Chromogranin - (0%)
ER- (20%)
PR+ (90%)
+/-
- neuroendocrine markers
- CD10 (#11023097#)
- keratin
- desmoplakin
- trypsin
- chymotrypsin
- amylase
- vimentin
- CD10 (diagnostically useful)
deregulated expression of cell cycle-associated proteins (#11235905#)
Focal positivity:
- neuron-specific enolase and other neuroendocrine markers
- various islet cell hormones, such as insulin and glucagon
# Progesterone receptors have been detected
Cytogenetics
t(11;22)(q24;q12) translocation (#11000339#)
t(13;17)(q14;p11)
Molecular biology
No EWSR1 rearrangements in 30 cases (#16941013#)
EWSR1/FLI-1 fusion transcript (not confirmed) (#11000339#)
beta-catenin gene mutation (CTNNB1 mutation) (83%) (#11943721#, #11731417#)
Differential diagnosis
adrenal pheochromocytoma (#15182415#)
Expression profiling
SPN display a complex expression profile, distinct from that observed in PET and DAC and involving both the beta-catenin and Notch pathways, together with expression of neural differentiation markers. (#19235837#)
Over-expression of AXIN2, TBX3, SP5 and NOTUM demonstrate activation of the beta-catenin pathway. (#19235837#)
Members of the Notch pathway (HEY1, HEY2, NOTCH2) are also up-regulated, relative to their expression in ductal adenocarcinomas (DAC) or pancreatic endocrine tumours (PET). (#19235837#)
Expression of other genes, such as EDN3, HAND2, netrin-G2 and the receptor netrin-G1 ligand, involved in neural crest differentiation is also identified as altered. (#19235837#)
Increased levels of SOX10 and TuJ-1 proteins are indicative of neural-like differentiation. (#19235837#)
Pathogenesis
primitive pancreatic epithelial cells
predominance of exocrine features
capacity for dual (exocrine and endocrine) differentiation
presence of progesterone receptors
- consistent with its predilection for females
- suggests hormone dependence (therefore potentially susceptible to hormonal manipulation)
- suggested that the tumor might be derived from genital ridge/ovarian anlage-related cells attached to the pancreatic tissue during early embryogenesis
Links
See also
pancreatic cystic tumors
Open access references
Array comparative genomic hybridization analysis of solid pseudopapillary neoplasms of the pancreas. Rund CR, Moser AJ, Lee KK, Zeh HJ, Teot LA, Dacic S, Krasinskas AM. Mod Pathol. 2008 May;21(5):559-64. PMID: #18246043# [Free]
Tang WW, Stelter AA, French S, Shen S, Qiu S, Venegas R, Wen J, Wang HQ, Xie J. Loss of cell-adhesion molecule complexes in solid pseudopapillary tumor of pancreas. Mod Pathol. 2007 May;20(5):509-13. PMID: #17334348# (Free)
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# (Free)
References
Gene expression profiling provides insights into the pathways involved in solid pseudopapillary neoplasm of the pancreas. Cavard C, Audebourg A, Letourneur F, Audard V, Beuvon F, Cagnard N, Radenen B, Varlet P, Vacher-Lavenu MC, Perret C, Terris B. J Pathol. 2009 Jan 20;218(2):201-209. PMID: #19235837#
Transcription factors involved in pancreas development are expressed in paediatric solid pseudopapillary tumours. Galmiche L, Sarnacki S, Verkarre V, Boizet B, Duvillie B, Fabre M, Jaubert F. Histopathology. 2008 Sep;53(3):318-24. Epub 2008 Jul 29. PMID: #18671802#
Min Kim S, Sun CD, Park KC, Kim HG, Lee WJ, Choi SH. Accumulation of beta-catenin protein, mutations in exon-3 of the beta-catenin gene and a loss of heterozygosity of 5q22 in solid pseudopapillary tumor of the pancreas. J Surg Oncol. 2006 Oct 1;94(5):418-25. PMID: #16967453#
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Solid-pseudopapillary tumor of the pancreas: a typically cystic carcinoma of low malignant potential. Klimstra DS, Wenig BM, Heffess CS. Semin Diagn Pathol. 2000 Feb;17(1):66-80. PMID: #10721808#
Solid-pseudopapillary tumor of the pancreas: immunohistochemical localization of neuroendocrine markers and CD10. Notohara K, Hamazaki S, Tsukayama C, Nakamoto S, Kawabata K, Mizobuchi K, Sakamoto K, Okada S. Am J Surg Pathol. 2000 Oct;24(10):1361-71. PMID: #11023097#
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