Solid pseudopapillary neoplasm, solid pseudopapillary tumour/neoplasm of the pancreas, Frantz's tumour
Cytopathology of solid pseudopapillary tumour/neoplasm with main findings that distinguish it from pancreatic endocrine neoplasms and acinar cell carcinomas.[1] Diff-Quik stain.
A solid pseudopapillary tumour is a low-grade malignant neoplasm of the pancreas of papillary architecture that typically afflicts young women.[2]
Signs and symptoms
Solid pseudopapillary tumours are often asymptomatic and are identified incidentally on imaging performed for unrelated reasons. Less often, they may cause abdominal pain. Solid pseudopapillary tumours tend to occur in women, and most often present in the third decade of life.[3]
Diagnosis
Papillae vs pseudopapillae: True papillae are outgrowths of epithelium, surrounding fibrovascular cores of stroma and at least one blood vessel. In contrast, pseudopapillae (such as in solid pseudopapillary tumours) are nests of proliferating cells that eventually grow to become almost back-to-back, with cells in the centers of nests disintegrating, leaving rims of cells lining the periphery of each nest. Discohesive cells and some formations lacking central blood vessels are visual clues.
The gold standard for diagnosing solid pseudopapillary tumour of the pancreas is cytopathology by endoscopic ultrasound (EUS) guided fine needle aspiration (FNA) of the lesion.[4] After surgical excision, the tumor can undergo histopathology evaluation for cancer staging.
Gross morphology
Solid pseudopapillary tumours are typically round, well-demarcated, measuring 2–17 cm in diameter (average 8 cm), with solid and cystic areas with hemorrhage on cut sections.[5]
Histomorphology
Solid pseudopapillary tumours consist of solid sheets of cells that are focally dyscohesive. The cells in the lesion usually have uniform nuclei with occasional nuclear grooves, eosinophilic or clear cytoplasm and PAS positive eosinophilic intracytoplasmic globules.[6] Necrosis is usually present and, as cell death preferentially occurs distant from blood vessels, lead to the formation of pseudopapillae.[citation needed]
^Source for mentioned findings: Pitman MB, Centeno BA, Daglilar ES, Brugge WR, Mino-Kenudson M (2014). "Cytological criteria of high-grade epithelial atypia in the cyst fluid of pancreatic intraductal papillary mucinous neoplasms". Cancer Cytopathol. 122 (1): 40–7. doi:10.1002/cncy.21344. PMID23939829. S2CID205677185. Images and annotations: Mikael Häggström, M.D.
^Pettinato G, Manivel JC, Ravetto C, et al. (November 1992). "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". American Journal of Clinical Pathology. 98 (5): 478–88. doi:10.1093/ajcp/98.5.478. PMID1283055.
^Image by Mikael Häggström, MD. Reference for features: Pooja Navale, M.D., Omid Savari, M.D., Joseph F. Tomashefski, Jr., M.D., Monika Vyas, M.D. "Solid pseudopapillary neoplasm".{{cite web}}: CS1 maint: multiple names: authors list (link) Last author update: 4 March 2022
^Wang Y, Miller FH, Chen ZE, Merrick L, Mortele KJ, Hoff FL, et al. (2011). "Diffusion-weighted MR imaging of solid and cystic lesions of the pancreas". Radiographics. 31 (3): E47-64. doi:10.1148/rg.313105174. PMID21721197. Diagram by Mikael Häggström, M.D.
^Pooja Navale, M.D., Omid Savari, M.D., Joseph F. Tomashefski, Jr., M.D., Monika Vyas, M.D. "Solid pseudopapillary neoplasm". Pathology Outlines.{{cite web}}: CS1 maint: multiple names: authors list (link) Last author update: 4 March 2022