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
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)
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.
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
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