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Published on: April 7, 2018
Exocrine pancreatic tumors in childhood in Germany
Verena Ellerkamp1, Steven W Warmann, Peter Vorwerk
1Department of Pediatric Surgery, Children's University Hospital Tuebingen, Hoppe-Seyler-Strasse3, Tuebingen, Germany. verenaellerkamp@googlemail.com
Insights
Pediatric pancreatic tumors (PT) are rare, with varied outcomes. Solid-pseudopapillary tumors (SPT) had 100% survival, while pancreatic carcinoma (P-CA) and pancreatoblastoma (PBL) had lower survival rates, highlighting the need for standardized care.
Area of Science:
- Pediatric Oncology
- Surgical Pathology
- Radiology
Background:
- Childhood pancreatic tumors (PT) are rare, lacking standard treatment protocols.
- This study analyzes treatment modalities and outcomes for pediatric PT.
Purpose of the Study:
- To investigate treatment approaches and patient outcomes for rare pediatric pancreatic tumors.
- To identify challenges in diagnosis and treatment of pediatric PT.
Main Methods:
- Retrospective analysis of 55 pediatric patients (<16 years) with exocrine PT diagnosed between 1980-2007.
- Data sourced from the German Pediatric Tumour Registry, including pseudonymized medical records.
Main Results:
- Records for 29 patients were analyzed: 18 solid-pseudopapillary tumors (SPT), 7 pancreatic carcinomas (P-CA), and 4 pancreatoblastomas (PBL).
- Diagnostic accuracy was challenged, with incorrect initial radiological diagnoses in 69% and histopathological discrepancies in up to 50%.
- SPT showed 100% survival post-surgery. P-CA survival was 14%, and PBL survival was 25%. Treatment concepts varied widely.
Conclusions:
- Emphasizes the critical need for reference pathology and radiology in diagnosing pediatric PT.
- Advocates for standardized treatment protocols and prospective data collection for pediatric pancreatic tumors.
Background:
Pancreatic tumors (PT) in childhood are rare. Standard therapeutic approaches are lacking. Our aim was to analyze treatment modalities and outcome in children with PT.
Procedure:
Between 1980 and 2007, 55 patients with exocrine PT < 16 years old were registered. Data were obtained from the German Pediatric Tumour Registry. Medical records were evaluated and patient data were pseudonymized.
Results:
Patient records of 29 children were available (9 male, 20 female, median age 11.2 years, range 3.1-16). In 18 patients a solid-pseudopapillary tumor (SPT) was diagnosed, in 7 patients a pancreatic carcinoma (P-CA) (5 acinar cell carcinoma (ACC), 2 ductal adenocarcinoma (DCA)), and in 4 patients a pancreatoblastoma (PBL). In 69% of the patients the initial radiological findings led to an incorrect tentative diagnosis. Initial histopathological diagnoses were differing from the reference pathology in 50% of the SPT and 45% of the P-CA. In the group of SPT survival rate was 100%; all patients underwent surgical resection. There were two cases of tumor relapse and one late secondary malignancy of the pancreas (DCA). In P-CA patients, survival rate was 14%, in the PBL group the survival rate was 25%. Concepts of chemotherapy, radiotherapy, and surgical intervention in P-CA and PBL were varying widely.
Conclusions:
In all cases of pediatric PT reference pathology and reference radiology should be involved. Standardized treatment concepts as well as prospective data registrations need to be entrenched.
