Solid pseudopapillary tumors of the pancreas are rare (usually benign) pancreatic tumors.
The tumor has been referred to with multiple different names, including:
- solid pseudopapillary tumor (SPT) of the pancreas
- solid pseudopapillary neoplasm (SPN)
- solid pseudopapillary epithelial neoplasm (SPEN)
- papillary cystic neoplasm of the pancreas
- Hamoudi tumor
- Gruber-Frantz tumor (or just Frantz tumor)
They are rare and thought to account for 1-2% of exocrine pancreatic tumors. They tend to present in young non-Caucasian females around the 2nd and 3rd decades of life (the 'daughter' tumor) 10.
- pancreatic dorsal agenesis: possible association 5
Most patients are asymptomatic at diagnosis. They may occasionally present with a gradually enlarging abdominal mass or vague abdominal pain.
The tumors frequently contain varying amounts of necrosis, hemorrhage, and cystic change. Lesions can be large at time of diagnosis (median size ~8 cm) 2.
There is a greater predilection to occur at pancreatic tail.
Large well-defined mass with heterogeneous appearances, due to its solid and cystic composition.
Usually seen as a well-encapsulated lesion with varying solid and cystic components owing to hemorrhagic degeneration. Following IV contrast administration, enhancing solid areas are typically noted peripherally, whereas cystic spaces are usually more centrally located. Calcifications and enhancing solid areas may be present at the periphery of the mass.
Typically demonstrates a well-defined lesion. May show a pure solid consistency in ~80% of cases 6.
Reported signal characteristics include:
- T1: low to heterogeneous signal intensity 1,6
- T2: heterogeneous to high signal intensity 1,6
- C+ (Gd): can show early heterogeneous and slowly progressive enhancement 11
Treatment and prognosis
While most lesions are benign, ~15% can be malignant. Complete resection is associated with long-term survival even in the presence of metastatic disease.
History and etymology
It was first described by renowned American surgical pathologist, Virginia K Frantz (1896-1967) 9 et al. in 1959, hence the historical name "Frantz tumor" 4,5.
- 1. Coleman KM, Doherty MC, Bigler SA. Solid-pseudopapillary tumor of the pancreas. Radiographics. 23 (6): 1644-8. doi:10.1148/rg.236035006 - Pubmed citation
- 2. Martin RC, Klimstra DS, Brennan MF et-al. Solid-pseudopapillary tumor of the pancreas: a surgical enigma? Ann. Surg. Oncol. 9 (1): 35-40. - Pubmed citation
- 3. Choi JY, Kim MJ, Kim JH et-al. Solid pseudopapillary tumor of the pancreas: typical and atypical manifestations. AJR Am J Roentgenol. 2006;187 (2): W178-86. doi:10.2214/AJR.05.0569 - Pubmed citation
- 4. Frantz VK. Tumors of the pancreas. Atlas of tumor pathology: fasc 27–28, ser 7. Washington, DC: Armed Forces Institute of Pathology, 1959; 32-33
- 5. Ulusan S, Bal N, Kizilkilic O et-al. Case report: solid-pseudopapillary tumour of the pancreas associated with dorsal agenesis. Br J Radiol. 2005;78 (929): 441-3. doi:10.1259/bjr/91312352 - Pubmed citation
- 6. Yu MH, Lee JY, Kim MA et-al. MR imaging features of small solid pseudopapillary tumors: retrospective differentiation from other small solid pancreatic tumors. AJR Am J Roentgenol. 2010;195 (6): 1324-32. doi:10.2214/AJR.10.4452 - Pubmed citation
- 7. Baek JH, Lee JM, Kim SH et-al. Small (less than 3 cm) solid pseudopapillary tumors of the pancreas at multiphasic multidetector CT. Radiology. 2010;257 (1): 97-106. doi:10.1148/radiol.10092089 - Pubmed citation
- 8. Liu W, Liang W, Xu S. Prevalence of small solid pseudopapillary tumors. AJR Am J Roentgenol. 2011;197 (5): W964. doi:10.2214/AJR.10.6309 - Pubmed citation
- 9. Humphreys GH. In memoriam. Virginia Kneeland Frantz, M.D. 1896-1967. (1968) American journal of clinical pathology. 49 (3): 429-30. Pubmed
- 10. Galvin A, Sutherland T, Little AF. Part 1: CT characterisation of pancreatic neoplasms: a pictorial essay. (2011) Insights into imaging. 2 (4): 379-388. doi:10.1007/s13244-011-0102-7 - Pubmed
- 11. Cantisani V, Mortele KJ, Levy A, et al. MR imaging features of solid pseudopapillary tumor of the pancreas in adult and pediatric patients. AJR 2003; 181:395-401
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