Other tumors

Solid pseudopapillary neoplasm

Editorial Board Member: Raul S. Gonzalez, M.D.
Deputy Editor-in-Chief: Debra L. Zynger, M.D.
Omid Savari, M.D.
Joseph F. Tomashefski, Jr., M.D.

Topic Completed: 1 May 2018

Minor changes: 11 August 2021

Copyright: 2002-2021,, Inc.

PubMed Search: Solid pseudopapillary tumor[TI] pancreas[TI] free full text[sb]

Omid Savari, M.D.
Joseph F. Tomashefski, Jr., M.D.
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Cite this page: Savari O, Tomashefski JF. Solid pseudopapillary neoplasm. website. Accessed October 15th, 2021.
Definition / general
Essential features
  • Presents in young women, classically as large body / tail mass
  • Variable amount of solid and cystic formation at gross exam
  • Papillary fronds on myxoid or hyalinized vascular stalk lined by poorly cohesive, uniform cells with nuclear grooves comprising solid and cystic areas
  • β-catenin positive
  • Also known as solid pseudopapillary tumor, papillary epithelial neoplasm, papillary cystic neoplasm, solid and papillary neoplasm, low grade papillary neoplasm and Hamoudi or Frantz tumor
  • Represents 1 - 2% of pancreatic neoplasms
  • Mainly young females are affected - the female to male ratio is 10:1
  • Usually presents in the third to fourth decade of life (mean age 35 years)
  • In men tends to occur at an older age with more aggressive behavior (Surgery 2008;143:29)
Clinical features
  • The most common symptoms are abdominal pain and a palpable, non-tender, upper abdominal mass
  • Also symptoms related to an intra-abdominal mass effect, such as discomfort, nausea, vomiting and early satiety
Radiology description
  • Well circumscribed, encapsulated, heterogeneous pancreatic lesion with cystic degeneration on CT or MRI
Radiology images

Contributed by Omid Savari, M.D.

MR of abdomen

Images hosted on other servers:

Hypodense lesion

Endoscopic ultrasound

Multiple metastases

Prognostic factors
  • Poor prognostic factors include: size > 5 cm, male gender, necrosis, cellular atypia, vascular invasion, perineural invasion and invasion into adjacent structures (Dig Liver Dis 2013;45:703)
Case reports
  • Surgical resection is curative in more than 95% of cases
Gross description
  • Range from 0.5 to 34.5 cm with a mean diameter of 6 cm
  • Well defined, encapsulated with variable amount of solid and cystic patterns
  • Smaller lesions tend to be more solid but less sharply circumscribed
  • Larger tumors demonstrate a fibrous pseudocapsule and have a variegated and friable cut surface
  • Cystic degeneration and hemorrhage are common findings in larger specimens
Gross images

Contributed by
Omid Savari, M.D.

Predominately solid tumor

 Contributed by
 Dr. Andreas Schulz

Partially necrotic tumor

 Contributed by
 Manfred Stolte, M.D.

Invasion of spleen

 Contributed by
 Dr. Helmut Luchtrath

Cystic lesion

 Case #121

Multilocular cystic mass

Microscopic (histologic) description
  • Solid nests of poorly cohesive cells forming a cuff surrounding blood vessels, resulting in a pseudopapillary architecture
  • Stroma usually shows various degrees of hyalinization or evidence of degeneration such as hemorrhage, foamy macrophages, calcification and cholesterol clefts
  • Tumor cells usually have a moderate amount of eosinophilic cytoplasm with large intracytoplasmic hyaline globules and perinuclear vacuoles
  • Relatively uniform nuclei with finely textured chromatin, inconspicuous nucleoli and characteristic longitudinal grooves
  • Oncocytic or clear cell changes may occur
  • Rare mitotic figures
  • Although grossly well circumscribed, microscopic finding of infiltration to the surrounding pancreatic tissue is not uncommon
Microscopic (histologic) images

Contributed by Omid Savari, M.D.

Solid pseudopapillary neoplasm

Solid pattern

Clear cell changes

Cholesterol cleft, scattered giant cells

Tumor invading parenchyma

Beta catenin



Contributed by Raul S. Gonzalez, M.D.

Metastasis to liver

Cytology description
  • Cellular smears with delicate papillary fronds
  • Tumor cells cells are usually bland and uniform with a moderate amount of cytoplasm, which usually contains variable sized clear perinuclear vacuoles or cytoplasmic eosinophilic hyaline globules
  • Nuclei are round to oval with grooves and finely granular chromatin (J Cytol 2010;27:118, Arch Pathol Lab Med 2017;141:990)
Cytology images

Contributed by Omid Savari, M.D.

Diff Quick


Cell block

Positive stains
Negative stains
Electron microscopy description
  • Mitochondria-rich cytoplasm with electron-dense granules and rough endoplasmic reticulum
Electron microscopy images

Contributed by Dr. H.D. John, Mainz

Abundant mitochondria


Molecular / cytogenetics description
  • Point mutation in exon 3 of β-catenin gene (CTNNB1) is present in more than 90%
  • Gene mutation results in the accumulation of β-catenin in the cytoplasm and formation of a β-catenin–Tcf/Lef complex, through which the Wnt signaling pathway activates several oncogenic genes such as MYC and Cyclin D1 (Am J Clin Pathol 2017;149:67)
Differential diagnosis
  • Pancreatic neuroendocrine tumor: small to medium sized cells with uniform nuclei and finely granular chromatin (salt and pepper appearance), arranged in organoid, trabecular or nested patterns; positive for neuroendocrine markers and negative for β-catenin
  • Acinar cell carcinoma: prominent acinar formation and cells with finely granular cytoplasm; positive for pancreatic enzymes and variable staining for β-catenin
  • Pancreatoblastoma: primitive pancreatic elements with acinar, ductal and neuroendocrine differentiation; also squamoid corpuscles; more common in children younger than 10 years; positive for β-catenin (80%), neuroendocrine markers and pancreatic enzymes (trypsin, chymotrypsin and lipase)
Board review style question #1
What is the most common gene mutation in pancreatic solid pseudopapillary neoplasm?

B. Kit
Board review style answer #1
A. Somatic point mutation of β-catenin gene (CTNNB1) is present in more than 90% of pancreatic solid pseudopapillary neoplasms

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Reference: Solid pseudopapillary neoplasm
Board review style question #2
What are the lab findings in pancreatic solid pseudopapillary neoplasm cyst fluid?

A. High amylase and high CEA
B. High amylase and low CEA
C. Low amylase and high CEA
D. Low amylase and low CEA
Board review style answer #2
D. Although non-specific, cyst fluid analysis of pancreatic solid pseudopapillary neoplasm shows low amylase and low CEA. The same results also can be detected in serous cystic tumors.

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Reference: Solid pseudopapillary neoplasm
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