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pancreatic solid pseudopapillary tumor

Wednesday 12 November 2003

PSPPT; solid-pseudopapillary tumor of the pancreas, Frantz tumor, Frantz’s tumor; solid pseudopapillary tumor of the pancreas; ICD-O codes Solid pseudopapillary neoplasm 8452/1, Solid pseudopapillary carcinoma 8452/3; pancreatic solid pseudopapillary tumor; pancreatic solid and pseudopapillary tumor; Solid-pseudopapillary neoplasm of the pancreas


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.


- Solid pseudopapillary tumor of pancreas - Macroscopy

- Solid pseudopapillary tumor of pancreas - Microscopy

- Solid pseudopapillary tumor of pancreas - Imprint

- Solid pseudopapillary tumor of pancreas with degenerative changes

- Solid pseudopapilary tumor with some clear cell areas and giant cells

- Solid pseudopapillary tumor of pancreas : cytology EUS FNA: cellular branching papillary clusters, myxoid fibrovascular cores, nuclear grooves.

- CD99 + in pancreatic solid pseudopapillary neoplasm

Cases - Digital slides

- HPC:45 (HPC:45)
- HPC:67 (HPC:67)
- HPC:68 (HPC:68) (In multiple endocrine neoplasia type 1 - MEN 1)
- HPC:69 (HPC:69)
- HPC:75 (HPC:75)
- HPC:96 (HPC:96)
- HPC:100 (HPC:100)
- HPC:199 (HPC:199)
- JRC:14920 : Pancreas solid and pseudopapillary tumor
- JRC:14615 : Pancreas solid and pseudopapillary tumor (with neuroendocrine differentiation)

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.

Solid-pseudopapillary tumor (SPT) of the pancreas is characterized by a discohesive appearance of the neoplastic cells.

This has been linked to the displacement of E-cadherin and beta-catenin from their normal membrane location, which prevents adherens junctions to form.

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


- 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


- 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.


- solid pseudopapillary tumor of the pancreas with atypical multinucleated giant tumour cells (23134473)


- +/- evidence of acinar, ductal, and (sometimes) endocrine cell differentiation


- 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

- nuclear localization of beta-catenin

  • The nuclear localization of beta-catenin is a feature of SPT that helps in differential diagnosis.
  • This latter includes pancreatic endocrine tumor (PET) as SPT may show neuroendocrine differentiation, and pancreatic acinar cell carcinoma (ACC) and pancreatoblastoma (PB) that may often show nuclear beta-catenin staining.

- Expression pattern of claudins 5 (CLDN5) and 7 (CLDN7) distinguishes solid-pseudopapillary from pancreatoblastoma, acinar cell and endocrine tumors of the pancreas. (19194274)

  • All SPT showed intense membrane claudin 5 and cytoplasmic claudin 2 staining, lack of claudins 3 and 4, and positive cytoplasmic claudins 1 and 7 in few cases.
  • Conversely, PET, ACC, and PB showed strong membrane expression of claudin-7 and lack of claudin-5, whereas claudins 1, 2, 3, and 4 showed variable expression among samples.
  • All SPT showed nuclear beta-catenin and lack of E-cadherin membrane staining, whereas PET, ACC, and PB only showed nuclear beta-catenin in 1, 2, and 2 cases, respectively.
  • SPT shows a peculiar claudin expression profile and the highly specific pattern of claudins 5 and 7 differentiates SPT from PET, ACC, and PB.

- Progesterone receptors have been detected


- 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)

- LEF1

  • Overexpression of lymphoid enhancer-binding factor 1 (LEF1) in solid-pseudopapillary neoplasms of the pancreas. (24658583)
  • LEF1 can be a useful ancillary stain in the diagnosis of solid-pseudopapillary neoplasms.

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)


- 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


- PathConsult

See also

- pancreatic cystic tumors

Open access references

- Overexpression of lymphoid enhancer-binding factor 1 (LEF1) in solid-pseudopapillary neoplasms of the pancreas. Singhi AD, Lilo M, Hruban RH, Cressman KL, Fuhrer K, Seethala RR. Mod Pathol. 2014 Oct;27(10):1355-63. doi : 10.1038/modpathol.2014.40 PMID: 24658583 - Free PMC Article

- 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)


- 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

- 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

- 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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- Abraham SC, Klimstra DS, Wilentz RE, Yeo CJ, Conlon K, Brennan M, et al. Solid-pseudopapillary tumors of the pancreas are genetically distinct from pancreatic ductal adenocarcinomas and almost always harbor beta-catenin mutations. Am J Pathol. 2002;160:1361–1369.

- Grant LD, Lauwers GY, Meloni AM, Stone JF, Betz JL, Vogel S, et al. Unbalanced chromosomal translocation, der(17)t(13;17)(q14;p11) in a solid and cystic papillary epithelial neoplasm of the pancreas. Am J Surg Pathol. 1996;20:339–345.

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