Elliot K. Fishman M.D.
Johns Hopkins Hospital

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“Pediatric pancreatic masses are rarely encountered at imaging, but a variety of tumors should be considered. The most likely diagnostic considerations include solid pseudopapillary tumor and pancreatoblastoma, which have differing peak ages of incidence. Endocrine lesions, including islet cell adenomatosis and islet cell tumors, are another possible group of epithelial neoplasms. Among cystic lesions, pseudocyst is the most common lesion in the pediatric pancreas. True cysts should be considered in the context of a genetic syndrome affecting multiple abdominal organs. Among nonepithelial tumors, primary or secondary pancreatic lymphoma is the most common consideration, though neurogenic tumors such as neuroblastoma may be considered.”
Imaging of Pediatric Pancreatic Neoplasms With Radiologic Histopathologic Correlation
Narendra S. Shet et al.
AJR 2014; 202:1337–1348

“Pediatric pancreatic neoplasms can be divided into epithelial and nonepithelial types. Most pancreatic neoplasms are epithelial lesions, which can be further subdivided into exocrine and endocrine subtypes. Nonepithelial lesions include benign entities such as lymphatic malformations, malignant neoplasms such as lymphoma, and intermediate lesions, including inflammatory myofibroblastic tumors.”
Imaging of Pediatric Pancreatic Neoplasms With Radiologic Histopathologic Correlation
Narendra S. Shet et al.
AJR 2014; 202:1337–1348

Results: We included 23 children in this study. Of these, 12 had solid and papillary epithelial neoplasms (SPEN), 3 had neuroendocrine tumors, 3 had lymphoma, and 1 each had hemangioendothelioma, benign congenital cysts with adipose tissue, dendritic cell sarcoma, metastatic rhabdomyosarcoma, and lipoma. All children with SPEN were teenage girls. SPEN showed characteristic imaging features including well-defined margins with surrounding capsule, solid-cystic components and hemorrhage.
Conclusion: Pancreatic tumors are uncommon in children. SPEN is the most common tumor and is seen predominantly in teenage girls and shows characteristic imaging features.
Imaging features of pancreatic tumors in children: 13-year experience at a pediatric tertiary hospital. 
Ahmed TS et al. 
Pediatr Radiol. 2013 Nov;43(11):1435-43.

“Pancreatoblastoma is reported to be the most common pancreatic neoplasm in young children. The manifestations of pancreatoblastoma are different from those of SPT in many ways. In contradistinction to patients with SPT, boys are affected approximately twice as frequently as girls, and the mean age at presentation of pancreatoblastoma is within the first decade of life. Most cases of pancreatoblastoma are sporadic, but several cases have been described in patients with Beckwith-Wiedemann syndrome.”
Imaging of Pediatric Pancreatic Neoplasms With Radiologic Histopathologic Correlation
Narendra S. Shet et al.
AJR 2014; 202:1337–1348

“At CT, pancreatoblastoma generally manifests itself as a well-marginated lobulated lesion with heterogeneous and septal enhancement. Higher-grade tumors may have progressively less-defined margins. Calcifications are frequently observed and described as rim or clustered. Metastatic lesions are usually hypoattenuating on CT images. Though biliary dilatation is rarely found, owing to the composition of the tumor, arterial encasement and venous invasion have been found, sometimes making the distinction between pancreatoblastoma and neuroblastoma difficult.”
Imaging of Pediatric Pancreatic Neoplasms With Radiologic Histopathologic Correlation
Narendra S. Shet et al.
AJR 2014; 202:1337–1348

“The clinical presentation of islet cell neoplasms differs between functional and nonfunctional tumors. Nonfunctional tumors present with nonspecific symptoms related to mass effect. Functional tumors, by contrast, often present earlier with symptoms related to the hypersecreting hormone. Insulinoma commonly manifests itself with the Whipple triad of fasting hypoglycemia and resultant symptoms followed by resolution with IV glucose administration. Gastrinoma presents with severe peptic ulcer disease, often occurring in atypical locations such as the postbulbar duodenum. VIPoma presents with WDHA syndrome (watery diarrhea, hypokalemia, achlorhydria), though in children VIP-secreting tumors are more often of neurogenic than islet cell origin.”
Imaging of Pediatric Pancreatic Neoplasms With Radiologic Histopathologic Correlation
Narendra S. Shet et al.
AJR 2014; 202:1337–1348

“Lymphoma can involve the pancreas, either primarily or secondarily, and is the most common nonepithelial pancreatic tumor. Primary pancreatic lymphoma is far less common than secondary lymphomatous involvement, which when present is suggestive of diffusely disseminated disease. Although fewer than 2% of all extranodal nonHodgkin lymphomas are primary pancreatic lymphoma, secondary involvement of the pancreas by non-Hodgkin lymphoma was found in one third of pediatric patients in autopsy studies. Among the histologic variants of non-Hodgkin lymphoma, large-cell lymphoma and Burkitt lymphoma most commonly involve the pancreas in children.”
Imaging of Pediatric Pancreatic Neoplasms With Radiologic Histopathologic Correlation
Narendra S. Shet et al.
AJR 2014; 202:1337–1348

“Solid pseudopapillary tumor (SPT), also known as solid papillary epithelial neoplasm or Frantz tumor, is a mixed solid and cystic mass of low malignant potential. This tumor accounts for 2–3% of pancreatic neoplasms in all age groups and 6% of all exocrine pancreatic tumors. The lesion is most frequently encountered in girls and women in the second to third decades of life, the female to-male ratio ranging between 2:1 and 10:1. Although the tumor has been described as more common in patients of African and Asian ancestry, studies encompassing multiple ethnicities have been lacking.”
Imaging of Pediatric Pancreatic Neoplasms With Radiologic Histopathologic Correlation
Narendra S. Shet et al.
AJR 2014; 202:1337–1348

“Patients typically present with abdominal pain; less frequent initial symptoms and signs include nausea, emesis, anorexia, and a palpable mass. SPT generally presents as a large (mean tumor size exceeding 5 cm) wellencapsulated mass. The most frequent site of involvement is the pancreatic head [14]. Metastatic spread at presentation is found in fewer than 10% of patients.”
Imaging of Pediatric Pancreatic Neoplasms With Radiologic Histopathologic Correlation
Narendra S. Shet et al.
AJR 2014; 202:1337–1348

“The CT manifestation of SPT is a heterogeneous mass with clearly defined margins. The tumor may have areas of low-attenuation necrosis that correlate with the ultrasound findings and areas of high attenuation representing hemorrhage. Calcifications have been noted in approximately one third of cases. Peripheral enhancement of the fibrous capsule has been described, though enhancement characteristics have not been consistent in the literature.”
Imaging of Pediatric Pancreatic Neoplasms With Radiologic Histopathologic Correlation
Narendra S. Shet et al.
AJR 2014; 202:1337–1348

“Solid pseudopapillary neoplasms of the pancreas are rare tumors accounting for 1–2% of pancreatic exocrine neoplasms. This entity was first described by Dr. Frantz in 1959 and was defined by the World Health Organization in 1996 as “solid pseudopapillary tumor.” It is most often a benign neoplasm, but 10–15% of the cases are malignant. Over the past decades, the incidence of this tumor is increasing. However, many surgeons are still unfamiliar with this neoplasm and its unique characteristics, which can lead to pitfalls in the diagnosis and treatment. The correct diagnosis of SPNP is of utmost importance since it has a low malignant potential and with the appropriate treatment, patients have a long life expectancy.”
Solid Pseudopapillary Neoplasms of the Pancreas: A Surgical and Genetic Enigma
Leon Naar et al.
World J Surg (2017). doi:10.1007/s00268-017-3921-y

Cystic Pancreatic Masses

• Serous Cystadenoma (SC)
• Mucinous Cystic Neoplasm (MCN)
• Intraductal Papillary Mucinous Neoplasm (IPMN)
• Cystic Neuroendocrine Neoplasms

Presentation:

• 43 year old woman, abdominal mass palpated on exam

Coronal volume rendered images demonstrate a 7.4 cm complex mass with extensive peripheral calcification. 3D mapping demonstrates both solid and cystic components. No ductal dilatation was noted. Based on the age, a solid pseudopapillary neoplasm was suspected. Surgical resection was performed and pathology revealed solid pseudopapillary neoplasm.