Pancreatic Masses in Children and Young Adults: Multimodality Review with Pathologic Correlation

Abstract

Masses and masslike lesions of the pancreas are uncommon in the pediatric population. However, owing to overlapping clinical and imaging features, it can be challenging to differentiate the various causes of pediatric pancreatic masses at initial patient presentation. Clinical data such as patient age, signs and symptoms at presentation, laboratory test results, and potential underlying cancer predisposition syndrome can be helpful when formulating a differential diagnosis. US may be the first imaging study to depict a pancreatic mass in a child, as this examination is frequently performed in children with nonspecific abdominal signs and symptoms because of its wide availability and relatively low cost and the lack of a need for sedation or anesthesia. CT or MRI is typically required for more thorough characterization of the mass and surgical planning. Complete characterization of pancreatic masses includes assessment of vascular involvement, local invasion, and extrapancreatic spread of tumor. The authors provide an up-to-date comprehensive review of the clinical manifestations, histopathologic features, and imaging findings of primary and secondary tumors of the pancreas in children and young adults. Advances in imaging, current prognostic information, and treatment paradigms also are highlighted. Finally, nontumorous masslike lesions of the pediatric pancreas, including vascular malformations, cystic disorders (eg, von Hippel-Lindau syndrome, cystic fibrosis), intrapancreatic accessory spleen, and autoimmune pancreatitis, are discussed.

Solid pancreatic masses in children: A review of current evidence and clinical challenges

“…Boys are more affected than girls. (3,8,13,14). PBL has been shown to have an association with Beckwith-Wiedemann syndrome, with a prevalence up to 50% in patients diagnosed (15,16).…”

“…Nonetheless, PBLs are rare with a recent systematic review identifying 81 pediatric cases since 1980 (5). PBLs are typically diagnosed in the first decade of life, with the median age being 4-5 years old (3,(6)(7)(8), although there are several cases reported in older children and adults (9)(10)(11)(12). Boys are more affected than girls.…”

“…Squamoid nests or corpuscles are characteristic and may be scattered throughout the tumor, however, these features may not be identified on a small biopsy. Squamous corpuscles are characterized by clusters of polygonal cells or whorled spindle cells, with or without central keratinization and expression of epithelial membrane antigen and LEF1 on immunohistochemical staining (3,8,15,(21)(22)(23) (Figures 1A,B). PBL demonstrates positive immunostaining for markers of acinar differentiation such as trypsin, chymotrypsin, and Bcl10, though positivity for neuroendocrine markers, such as chromogranin and synaptophysin, and alpha-fetoprotein (AFP)positive cells have also been identified (15,21).…”

“…Beta-catenin staining may also be noted, predominantly within squamoid corpuscles and in a subset of background neoplastic cells. If present, stroma is often hypercellular, containing spindle-shaped cells (8,15).…”

Rare presentation in a rare case of pancreatic extraosseous Ewing’s sarcoma: A case report

“…Tumors of the pancreas in young adults vary widely and include pancreatoblastoma, ductal adenocarcinoma, metastatic lesions, lymphoma, or a myriad of benign tumors. [ 5 ] The treatment of these tumors is markedly different.…”