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Published on: January 3, 2013
Childhood pancreatoblastoma: clinical features and immunohistochemistry analysis
Wei Zhong Gu1, Chao Chun Zou, Zheng Yan Zhao
1Department of Pathology, The Children's Hospital of Zhejiang University School of Medicine, Hangzhou, China.
Insights
Pancreatoblastoma (PB) is a rare pancreatic tumor in children. Diagnosis relies on pathological findings, with surgery and combination therapy showing good outcomes.
Area of Science:
- Pediatric Oncology
- Gastrointestinal Pathology
Background:
- Pancreatoblastoma (PB) is an exceptionally rare pancreatic neoplasm primarily affecting infants and children.
- Understanding its clinical presentation and diagnostic markers is crucial for early intervention.
Observation:
- Three pediatric cases (two males, one female; aged 3 days to 4.7 years) presented with abdominal pain and diarrhea.
- All cases exhibited elevated serum alpha-fetoprotein (AFP) levels.
- Imaging revealed well-defined, heterogeneous pancreatic or mesenteric masses.
Findings:
- Histopathology confirmed PB diagnosis.
- Immunohistochemistry showed positivity for cytokeratin AE1/AE3 and CEA in all cases.
- Tumor markers like EMA, S-100, AFP, NSE, chromogranin A, synaptophysin, and 5-HT were variably expressed.
Implications:
- Pathological examination is paramount for accurate pancreatoblastoma diagnosis.
- PB should be considered in pediatric differential diagnoses for pancreatic or mesenteric masses, particularly with elevated AFP.
- Treatment approaches including surgery and multimodal therapy yield favorable outcomes.
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.