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Pancreatoblastoma
1Department of Pediatric Surgery, Seoul National University Children's Hospital, Korea.
Insights
Pancreatoblastoma, a rare childhood pancreatic tumor, presents as an abdominal mass. Complete surgical excision offers the best chance for cure, while unresectable tumors have a poor prognosis.
Area of Science:
- Pediatric Oncology
- Surgical Pathology
Background:
- Pancreatoblastoma is an extremely rare pancreatic neoplasm affecting young children, characterized by distinct acinar and squamoid cell differentiation.
- Over 50 cases reported globally highlight its rarity and the need for increased awareness.
Observation:
- A review of five pediatric cases treated between 1984-1994 revealed abdominal masses as the primary presentation.
- Symptoms included abdominal pain, anorexia, vomiting, and weight loss in some patients.
- Elevated serum alpha-fetoprotein was noted in a case with liver metastases.
Findings:
- Complete tumor excision was achieved in two cases; one had successful long-term survival after recurrence, while the other developed liver metastases and died.
- Partial excision or biopsy was performed for unresectable tumors, with all three patients succumbing to the disease despite adjuvant therapies.
- Electron microscopy revealed zymogen granules, and immunocytochemistry was negative for common pancreatic hormones.
Implications:
- Diagnosis of pancreatoblastoma should be considered in children presenting with an abdominal mass.
- Complete surgical resection is critical for achieving a potential cure.
- The prognosis for unresectable pancreatoblastoma remains poor, underscoring the importance of early detection and complete surgical removal.
Background:
Pancreatoblastoma is a rare pancreatic tumor with distinct acinar and squamoid cell differentiation that generally affects infants and young children. Just over 50 cases have been reported in the literature.
Methods:
Five cases of pathologically proven pancreatoblastoma treated at Seoul National University Hospital from 1984 to 1994 were reviewed. There were three girls and two boys who were 2 years to 5 years of age. All cases came to medical attention because of an abdominal mass.
Results:
Abdominal pain was observed in one case and anorexia, vomiting, and weight loss in one case. There was marked elevation of serum alpha-fetoprotein (27,000 ng/mL) in one case of liver metastases. Complete excision was performed in two cases in which the tumors were located in the tail of the pancreas. Partial excision was performed in two patients who had unresectable tumors of the head of the pancreas. One patient had an unresectable tumor at diagnosis and needle aspiration biopsy was carried out under ultrasound guidance. Electron microscopy was performed on pathological specimens of three cases and showed zymogen granules but not neuroendocrine granules. Immunocytochemical studies for alpha-fetoprotein, insulin, glucagon and somatostatin were performed in one patient, and results were all negative. Of two patients who underwent complete excision, one patient presented with liver metastases 4 months after operation and received chemotherapy, but died of tumor 6 months after operation. The other patient had local recurrence 1 year after operation. Reoperation and chemotherapy were performed, and the child is now alive without evidence of disease for 32 months. All three patients who had unresectable tumor died of tumor despite adjuvant radiotherapy and chemotherapy.
Conclusions:
The authors emphasize that the diagnosis of pancreatoblastoma in childhood should be suspected with palpation of an abdominal mass, and the chance for cure may be determined by complete excision of the tumor.