Most often solid, but can have variable internal cystic components and intratumoral hemorrhage
•
Capsule enhances on CECT and T1 C+ MR and appears as rim of low T2 signal intensity
•
Frequent peripheral or central calcification (45-50%)
•
Presence of internal hemorrhage highly characteristic feature, and may result in fluid-fluid or hematocrit levels
Internal hemorrhage usually easier to perceive on MR
•
Usually no biliary or pancreatic ductal obstruction
•
Metastatic disease is very uncommon, but most often metastasizes to liver and locoregional lymph nodes
PATHOLOGY
•
Rare: < 3% of all pancreatic tumors
•
Previously thought to have separate benign and malignant subtypes, but recent WHO classification defines all SPEN as low-grade malignancies
Low malignant potential (< 10% metastasize or recur)
CLINICAL ISSUES
•
Almost always arises in patients < 35 years (rarely reported in older adults)
Accounts for 8-16% of pancreatic tumors in children
•
Possible predilection for African Americans and Asians
•
Most patients are symptomatic, with abdominal pain most common presenting symptom
•
Treatment: Complete surgical resection
DIAGNOSTIC CHECKLIST
•
Consider SPEN when confronted by an encapsulated solid pancreatic mass in a young woman, particularly when there is evidence of internal hemorrhage
TERMINOLOGY
Abbreviations
•
Solid and pseudopapillary neoplasm (SPEN)
Synonyms
•
Hamoudi tumor, Franz tumor
•
Papillary epithelial neoplasm, papillary cystic carcinoma, solid and cystic tumor of pancreas, Franz tumor, solid and papillary epithelial neoplasm,
IMAGING
General Features
•
Best diagnostic clue
Encapsulated solid mass with cystic components and internal hemorrhage in a young woman
•
Location
Can occur anywhere in pancreas without predisposition for any location
•
Size
Average: 5 cm, range: 2.5-20 cm
CT Findings
•
Well-defined, heterogeneous, encapsulated mass with thick, enhancing capsule
Usually quite large at presentation (mean > 5 cm)
•
Frequent peripheral or central calcification (45-50%)
•
Most often solid, but can have variable cystic components and intratumoral hemorrhage
Usually very little enhancement, with “solid” components often representing intratumoral blood products
•
Metastatic disease is very uncommon, but most often metastasizes to liver and locoregional lymph nodes
•
Usually no biliary or pancreatic ductal obstruction
•
Gross vascular invasion or occlusion on imaging is rare
MR Findings
•
Large, well-demarcated mass with central areas of low and high T1 signal intensity (hemorrhage)
Presence of internal hemorrhage highly characteristic feature, and may result in fluid-fluid or hematocrit levels
•
Solid or cystic with minimal enhancement on T1WI C+
•
Capsule appears as rim of low T2 signal intensity and enhances on post-gadolinium images
Ultrasonographic Findings
•
Fluid-debris levels; posterior acoustic enhancement
•
Very little internal color flow vascularity
Angiographic Findings
•
Hypovascular; depends on degree of necrosis
Nuclear Medicine Findings
•
PET/CT
Variable, but can demonstrate increased FDG uptake
Imaging Recommendations
•
Protocol advice
CECT or MR
DIFFERENTIAL DIAGNOSIS
Mucinous Cystic Pancreatic Tumor