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Updated: Jul 17, 2026
Application of Mid-Pancreatectomy with End-to-End Anastomosis in Pancreatic Benign Tumors
Published on: February 9, 2024
Pancreatoblastoma
1Yale University School of Medicine, New Haven, CT 06520, USA. wasif.saif@yale.edu
Insights
Pancreatoblastoma (PB) is a rare childhood pancreatic tumor. Complete surgical resection offers a good prognosis, emphasizing early detection and management.
Area of Science:
- Pediatric Oncology
- Gastrointestinal Pathology
Background:
- Pancreatoblastoma (PB) is an extremely rare pancreatic neoplasm, primarily affecting children but also occurring in adults.
- While less aggressive in younger patients, PB often presents late with abdominal pain and palpable masses.
Purpose of the Study:
- To review the clinical presentation, etiology, diagnosis, treatment, and prognosis of Pancreatoblastoma.
- To highlight the importance of early detection and proper management of this rare pancreatic tumor.
Main Methods:
- Literature review of Pancreatoblastoma cases.
- Analysis of clinical presentation, histological findings, and diagnostic modalities.
- Evaluation of treatment strategies and patient outcomes.
Main Results:
- PB is characterized by acinar and squamoid cell differentiation, often associated with Wnt signaling pathway alterations and Beckwith-Wiedemann syndrome.
- Diagnosis can be challenging, with elevated alpha-fetoprotein in some cases; imaging like ultrasound and CT are useful.
- Complete surgical resection is the primary curative treatment, with adjuvant therapies under investigation.
Conclusions:
- Pancreatoblastoma, though rare, is considered a curable tumor when detected and treated early through complete resection.
- Prognosis is favorable with complete resection but poorer in cases of metastasis or inoperability.
- Increased awareness is crucial for timely diagnosis and effective management of pediatric pancreatic cancer.
Abstract:
Pancreatoblastoma (PB), or infantile pancreatic carcinoma, is an extremely rare pancreatic tumor in childhood, comprising 0.5% of pancreatic non-endocrine tumors. Although PB mainly presents during childhood but can also occur in adults. PB tend to be less aggressive in infants and children compared to adults. Children with PB usually present late with upper abdominal pain and many have a palpable mass in the epigastrium. Mechanical obstruction of the upper duodenum and gastric outlet by tumor in the head of the pancreas may be associated with vomiting, jaundice and gastrointestinal bleeding. Histologically, PB is characterized with distinct acinar and squamoid cell differentiation. PB has been associated with alterations in the Wnt signaling pathway and chromosome 11p loss of heterozygosity (LOH), Beckwith-Wiedemann syndrome and familial adenomatous polyposis. The majority of these tumors arise in the head of the pancreas. Alpha-fetoprotein may be elevated in up to 68% of patients with PB. Ultrasound and CT scan may be useful but preoperative diagnosis is often quite difficult. The treatment of choice is complete resection, that may often be curative. The role of adjuvant chemotherapy or radiotherapy is still under discussion due to small number of patients treated as yet. Chemotherapy regimens consisting of cyclophosphamide, etoposide, doxorubicin, and cisplatin have been used in neoadjuvant setting with anecdotal benefit. Prognosis of this rare tumor is good, when resected completely. Prognosis is poorer, when there is metastasis or when it is inoperable. On the whole, PB is regarded to be a curable tumor; hence the clinical diagnosis should be made early. Awareness of this rare tumor of pancreas is essential for early detection and proper management. The author review the clinical presentation, etiology, diagnosis, treatment and prognosis of PB in this presentation.
