Solid pseudopapillary tumor of the pancreas
Solid pseudopapillary tumors (SPT) of the pancreas are rare (usually benign) pancreatic tumors.
Terminology
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)
Epidemiology
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 2 and 3 decades of life (the 'daughter' tumor) .
Clinical presentation
Most patients are asymptomatic at diagnosis. They may occasionally present with a gradually enlarging abdominal mass or vague abdominal pain.
Pathology
The tumors frequently contain varying amounts of necrosis, hemorrhage, and cystic change. Lesions can be large at time of diagnosis (median size ~8 cm) .
Location
There is a greater predilection to occur at pancreatic tail.
Associations
- pancreatic dorsal agenesis: possible association
Radiographic features
Ultrasound
Large well-defined mass with heterogeneous apperances, due to its solid and cystic composition.
CT
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.
MRI
Typically demonstrates a well-defined lesion. May show a pure solid consistency in ~80% of cases .
Reported signal characteristics include:
- T1: low to heterogeneous signal intensity
- T2: heterogeneous to high signal intensity
- C+ (Gd): can show heterogeneous and slowly progressive enhancement
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) et al. in 1959 .
Siehe auch:
- zystische Pankreasläsionen
- Azinuszellkarzinom des Pankreas
- Pankreastumoren
- Agenesie der dorsalen Pankreasanlage
und weiter:
Assoziationen und Differentialdiagnosen zu solider pseudopapillärer Tumor des Pankreas:
