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
Solid pseudopapillary tumor of pancreas with degenerative changes
Cases - Digital slides
HPC:68 (HPC:68) (In multiple endocrine neoplasia type 1 - MEN 1)
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.
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
areas of hemorrhage and necrosis
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
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
- 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%)
- neuroendocrine markers
- CD10 (11023097)
- CD10 (diagnostically useful)
deregulated expression of cell cycle-associated proteins (11235905)
- 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.
- 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
- No EWSR1 rearrangements in 30 cases (16941013)
- EWSR1/FLI-1 fusion transcript (not confirmed) (11000339)
- 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.
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
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
Solid and papillary epithelial neoplasm of the pancreas. An ultrastructural and immunocytochemical study of six cases. Lieber MR, Lack EE, Roberts JR Jr, Merino MJ, Patterson K, Restrepo C, Solomon D, Chandra R, Triche TJ. Am J Surg Pathol. 1987 Feb;11(2):85-93. PMID: 3812876
Jeng LB, Chen MF, Tang RP. Solid and papillary neoplasm of the pancreas. Emphasis on surgical treatment. Arch Surg. 1993;128:433–436.
Matsunou H, Konishi F. Papillary-cystic neoplasm of the pancreas. A clinicopathologic study concerning the tumor aging and malignancy of nine cases. Cancer. 1990;65:283–291.
Klimstra DS, Wenig BM, Heffess CS. Solid-pseudopapillary tumor of the pancreas: a typically cystic carcinoma of low malignant potential. Semin Diagn Pathol. 2000;17:66–80.
Nishihara K, Nagoshi M, Tsuneyoshi M, Yamaguchi K, Hayashi I. Papillary cystic tumors of the pancreas. Assessment of their malignant potential. Cancer. 1993;71:82–92.
Miettinen M, Partanen S, Fräki O, Kivilaakso E. Papillary cystic tumor of the pancreas. An analysis of cellular differentiation by electron microscopy and immunohistochemistry. Am J Surg Pathol. 1987;11:885865.
Pettinato G, Manivel JC, Ravetto C, Terracciano LM, Gould EW, di Tuoro A, et al. Papillary cystic tumor of the pancreas. A clinicopathologic study of 20 cases with cytologic, immunohistochemical, ultrastructural, and flow cytometric observations, and a review of the literature. Am J Clin Pathol. 1992;98:478–488.
Nishihara K, Tsuneyoshi M, Ohshima A, Yamaguchi K. Papillary cystic tumor of the pancreas. Is it a hormone-dependent neoplasm?. Pathol Res Pract. 1993;189:521–526.
Zamboni G, Bonetti F, Scarpa A, Pelos G, Doglioni C, Iannucci A, et al. Expression of progesterone receptors in solid-cystic tumour of the pancreas. A clinicopathological and immunohistochemical study of ten cases. Virchows Arch [A]. 1993;423:425–431.
Kosmahl M, Seada LS, Janig U, Harms D, Kloppel G. Solid-pseudopapillary tumor of the pancreas: its origin revisited. Virchows Arch. 2000;436:473–480.
Matsunou H, Konishi F, Yamamichi N, Takayanagi N, Mukai M. Solid, infiltrating variety of papillary cystic neoplasm of the pancreas. Cancer. 1990;65:2747–2757.
Orlando CA, Bowman RL, Loose JH. Multicentric papillary-cystic neoplasm of the pancreas. Arch Pathol Lab Med. 1991;115:958–960.
Klöppel G, Maurer R, Hofmann E, Luthold K, Oscarson J, Forsby N, et al. Solid-cystic (papillary-cystic) tumours within and outside the pancreas in men. Report of two patients. Virchows Arch [A]. 1991;418:179–183.
Lieber MR, Lack EE, Roberts JR, Merino MJ, Patterson K, Restrepo C, et al. Solid and papillary epithelial neoplasm of the pancreas. An ultrastructural and immunocytochemical study of six cases. Am J Surg Pathol. 1987;11:85–93.
vde la Roza G, Cleary KR, Ordóñez NG, El-Naggar A, Mackay B, Romsdahl MM. Solid and papillary tumor of the pancreas: Ultrastructural observations on two contrasting cases. Ultrastruct Pathol. 1997;21:439–447.
Jorgensen LJ, Hansen AB, Burcharth F, Philipsen E, Horn T. Solid and papillary neoplasm of the pancreas. Ultrastruct Pathol. 1992;16:659–666.
Stommer P, Kraus J, Stotlte M, Giedl J. Solid and cystic pancreatic tumors. Clinical, histochemical, and electron microscopic features in ten cases. Cancer. 1991;67:1635–1641.
Notohara K, Hamazaki S, Tsukayama C, Nakamoto S, Kawabata K, Mizobuchi K, et al. Solid-pseudopapillary tumor of the pancreas: immunohistochemical localization of neuroendocrine markers and CD10. Am J Surg Pathol. 2000;24:1361–1371.
Morohoshi T, Kanda M, Horie A, Chott A, Dreyer T, Klöppel G, et al. Immunocytochemical markers of uncommon pancreatic tumors. Acinar cell carcinoma, pancreatoblastoma, and solid cystic (papillary-cystic) tumor. Cancer. 1987;59:739–747.
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.
Cappelari JO, Geisinger KR, Albertson DA, Wolfman NT, Kute TE. Malignant papillary cystic tumor of the pancreas. Cancer. 1990;66:193–198.
Kuo T-t, Su I-J, Chien C-h. Solid and papillary neoplasm of the pancreas. Report of three cases from Taiwan. Cancer. 1984;54:1469–1474.
Sclafani LM, Reuter VE, Coit DG, Brennan MF. The malignant nature of papillary and cystic neoplasm of the pancreas. Cancer. 1991;68:153–158.