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Pancreas: Pediatric Pancreas Tumors Imaging Pearls - Educational Tools | CT Scanning | CT Imaging | CT Scan Protocols - CTisus
Imaging Pearls ❯ Pancreas ❯ Pediatric Pancreas Tumors

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  • “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
  • “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
  •  “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
  •  “In addition to islet cell hyperplasia, pancreatic islet cell or neuroendocrine tumors may arise as either functional or nonfunctional lesions. Functional islet cell tumors include insulinoma, gastrinoma, and vasoactive intestinal peptide tumor (VIPoma). Insulinoma is the most common islet cell tumor in pediatric patients, accounting for nearly one half of such lesions. Somatostatinoma and glucagonoma are other islet cell tumors in adults that have not been described in children.”
    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
  •  “Inflammatory myofibroblastic tumors, previously called inflammatory pseudotumors, occur most commonly in the second decade of life and affect girls more commonly than boys. It is a neoplasm composed of myofibroblastic spindle cells with an inflammatory infiltrate of lymphocytes, plasma cells, and eosinophils in the background. These lesions are usually discovered incidentally during imaging of the abdomen for other purposes but may produce nonspecific symptoms, such as pain, jaundice, and a palpable mass. At imaging, inflammatory myofibroblastic tumor appears as a solid well-defined mass. Ultrasound shows an isoechoic or hypoechoic mass, and CT depicts a lesion that enhances comparably to the remainder of the pancreas, apart from lower-attenuation regions of necrosis.”
    Imaging of Pediatric Pancreatic Neoplasms With Radiologic Histopathologic Correlation
    Narendra S. Shet et al.
    AJR 2014; 202:1337–1348
  • “Incidental pancreatic cysts (PCs) are commonly encountered in radiology practice. The prevalence rate of PCs is estimated at 2.5%.1 There is a 9% reported incidence on computed tomography (CT) and a 27% incidence on MR imaging.2 PCs are a heterogeneous group, including intraductal papillary mucinous neoplasm (IPMN), serous cystic neoplasm (SCN), and mucinous cystic neoplasm (MCN). Non-neoplastic PCs are pancreatic pseudocysts (common), epithelial cysts (uncommon), and lymphoepithelial cysts (rare). The significance in categorizing PCs lies in the potential of the mucinous varieties to develop malignancy.”  
    Incidental Pancreatic Cysts on Cross-Sectional Imaging  
    Shannon M. Navarro et al.
    Radiol Clin N Am 59 (2021) 617–629  
  • “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
    Shet NS et al
    AJR 2014 202:6, 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.”

    Imaging of Pediatric Pancreatic Neoplasms With Radiologic-Histopathologic Correlation
    Shet NS et al
    AJR 2014 202:6, 1337-1348 
  • “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. The primary tumor is often large at presentation, and identifying the pancreas as the source organ can be difficult.”

    Imaging of Pediatric Pancreatic Neoplasms With Radiologic-Histopathologic Correlation
    Shet NS et al
    AJR 2014 202:6, 1337-1348 
  • “At CT, pancreatoblastoma generally manifests itself as a well-marginated lobulated lesion with heterogeneous and septal enhancement.”

    Imaging of Pediatric Pancreatic Neoplasms With Radiologic-Histopathologic Correlation
    Shet NS et al
    AJR 2014 202:6, 1337-1348  
  • “In addition to islet cell hyperplasia, pancreatic islet cell or neuroendocrine tumors may arise as either functional or nonfunctional lesions. Functional islet cell tumors include insulinoma, gastrinoma, and vasoactive intestinal peptide tumor (VIPoma). Insulinoma is the most common islet cell tumor in pediatric patients, accounting for nearly one half of such lesions. Somatostatinoma and glucagonoma are other islet cell tumors in adults that have not been described in children. Neuroendocrine tumors can be further subdivided into malignant and benign entities. Islet cell tumors account for 20% of malignant pancreatic tumors in children.”

    Imaging of Pediatric Pancreatic Neoplasms With Radiologic-Histopathologic Correlation
    Shet NS et al
    AJR 2014 202:6, 1337-1348  
  • “Cysts can be subdivided into false and true cysts. Cystic neoplasms (discussed later) are a rare subset of cystic pancreatic lesions found in children. Pseudocysts, or cysts without a true epithelial lining, are the most common cystic pancreatic lesion in the pediatric population. These lesions can develop as the result of blunt trauma or as a complication of pancreatitis. True congenital cysts can occur either in isolation or as multiple lesions. Solitary true cysts may develop in the absence of a defined syndrome, whereas multiple true cysts can be seen in the setting of systemic disorders such as autosomal dominant polycystic kidney disease, von Hippel–Lindau syndrome, and Beckwith-Wiedemann syndrome.”

    Imaging of Pediatric Pancreatic Neoplasms With Radiologic-Histopathologic Correlation
    Shet NS et al
    AJR 2014 202:6, 1337-1348 
  • “Cystic neoplasms of the pancreas are rare in children. Benign cystic neoplasms include serous and mucinous cystadenoma, each of which has been described fewer than 10 times in the pediatric literature. The malignant counterpart, cystadenocarcinoma, has only been described once in the pediatric literature, to our knowledge, and in that case, there was a presumed predisposition to pancreatic malignancy in a patient with cystic fibrosis.”
     Imaging of Pediatric Pancreatic Neoplasms With Radiologic-Histopathologic Correlation
    Shet NS et al
    AJR 2014 202:6, 1337-1348  
  • “Neurogenic tumors fall into three categories: those arising from the sympathetic chain, such as neuroblastoma and ganglioneuroblastoma; those originating from peripheral nerves, such as schwannoma and neurofibroma; and those that consist of paraganglionic cells, such as pheochromocytoma. Neuroblastoma, the most common solid tumor of infancy, has been reported to involve the pancreas secondarily by contiguous extension rather than arising from the gland itself . However, primary pancreatic neuroblastoma can also occur in infants. Although the number of cases reported in the literature—seven to our knowledge— is not sufficient to draw definitive conclusions, imaging features should be consistent with those of neuroblastoma in other, more conventional locations.”

    Imaging of Pediatric Pancreatic Neoplasms With Radiologic-Histopathologic Correlation
    Shet NS et al
    AJR 2014 202:6, 1337-1348  
  • “Neurogenic tumors fall into three categories: those arising from the sympathetic chain, such as neuroblastoma and ganglioneuroblastoma; those originating from peripheral nerves, such as schwannoma and neurofibroma; and those that consist of paraganglionic cells, such as pheochromocytoma. Neuroblastoma, the most common solid tumor of infancy, has been reported to involve the pancreas secondarily by contiguous extension rather than arising from the gland itself . However, primary pancreatic neuroblastoma can also occur in infants. Although the number of cases reported in the literature—seven to our knowledge— is not sufficient to draw definitive conclusions, imaging features should be consistent with those of neuroblastoma in other, more conventional locations.”

    Imaging of Pediatric Pancreatic Neoplasms With Radiologic-Histopathologic Correlation
    Shet NS et al
    AJR 2014 202:6, 1337-1348 
  • “Lymphoma can involve the pancreas, e ither 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 diseas. Although fewer than 2% of all extranodal non-Hodgkin 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
    Shet NS et al
    AJR 2014 202:6, 1337-1348 

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