title: Childhood pancreatoblastoma: clinical features and immunohistochemistry analysis.
abstract: Pancreatoblastoma (PB) is an extremely rare pancreatic tumor that is most commonly encountered in infants and young children. In this study, three cases of childhood pancreatoblastoma are reported to emphasize the clinical features, laboratory findings, diagnosis, and management of this rare disease. They were two boys and one girl, aged 3 days, 4.6, and 4.7 years, respectively. The main causes of hospitalization were abdominal pain and diarrhea. Elevated serum alpha-foetoprotein levels were noted in all cases. Imaging findings indicated a well-defined heterogeneous large mass in the pancreas or mesentery. The diagnosis was confirmed by histopathology. Immunohistochemical staining showed cytokeratin AE1/AE3 and carcinoembryonic antigen positive in all samples, epithelial membrane antigen, S-100 and alpha-foetoprotein positive in two, neurone-specific enolase, chromogranin A, synaptophysin, and 5-hydroxytryptamine positive in one. Surgery alone was performed for two cases, and the other case with a huge mass was administrated combination therapy (chemotherapy, tumorectomy, and radiotherapy), with a good outcome in the follow-up. These data suggest the diagnosis of PB depends mainly on the pathological findings. The PB should be included in the differential diagnosis of a mass in the pancreas or mesentery, especially with elevated AFP and a well-defined heterogeneous imaging finding.
authors.affiliation: Department of Pathology, The Children's Hospital of Zhejiang University School of Medicine, Hangzhou, China.
authors.forename: Wei Zhong
authors.initials: WZ
authors.lastname: Gu
authors.forename: Chao Chun
authors.initials: CC
authors.lastname: Zou
authors.forename: Zheng Yan
authors.initials: ZY
authors.lastname: Zhao
authors.forename: Li
authors.initials: L
authors.lastname: Liang
authors.forename: Hong Feng
authors.initials: HF
authors.lastname: Tang
mesh_terms: D014408:Biomarkers, Tumor / Q000096:biosynthesis / Q000097:blood; D002675:Child, Preschool; D005260:Female; D006801:Humans; D007150:Immunohistochemistry; D007231:Infant, Newborn; D008297:Male; D010179:Pancreas / Q000473:pathology / Q000601:surgery; D010190:Pancreatic Neoplasms / Q000175:diagnosis* / Q000378:metabolism / Q000628:therapy; D016577:Pancreaticoduodenectomy; D035583:Rare Diseases / Q000175:diagnosis* / Q000378:metabolism / Q000628:therapy; D000509:alpha-Fetoproteins / Q000032:analysis / Q000378:metabolism
chemical_list: D014408:Biomarkers, Tumor; D000509:alpha-Fetoproteins