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Published on: February 9, 2024
Pancreatoblastoma: A Case Report
Turki Alhazmi1, Ahmed Abduljabbar2, Mohammed Alem3
1Division of Radiology, Umm Al-Qura University, Makkah, Saudi Arabia.
Insights
Pancreatoblastoma (PB) is a rare childhood pancreatic cancer. While less aggressive in children, surgical removal offers the best treatment, though adjuvant therapies are still being studied.
Area of Science:
- Oncology
- Pediatric Pathology
Background:
- Pancreatoblastoma (PB) is an exceptionally rare malignant exocrine pancreatic tumor primarily affecting children, though adult cases exist.
- This tumor, also known as infantile pancreatic carcinoma, exhibits distinct acinar and squamoid cell differentiation.
Observation:
- PBs most commonly arise in the pancreatic head and can elevate alpha-fetoprotein levels in up to 68% of cases.
- Preoperative diagnosis using ultrasound and CT scans is challenging.
- PB generally presents with less hostility in infants and children compared to adults.
Findings:
- Surgical resection is the primary treatment modality for pancreatoblastoma.
- The efficacy of adjuvant chemotherapy or radiotherapy remains under investigation due to limited patient numbers.
- Neoadjuvant chemotherapy regimens have shown anecdotal benefits.
Implications:
- Metastasis and unresectable tumors are associated with a poorer prognosis.
- Further research is needed to establish optimal adjuvant treatment strategies for PB.
- Improved diagnostic methods and treatment protocols are crucial for better patient outcomes.
Abstract:
Pancreatoblastoma (PB), also known as infantile pancreatic carcinoma, is an exceedingly rare pancreatic tumor in childhood, which is considered a malignant exocrine pancreatic tumor. Some cases have been reported in Saudi Arabia. Although PB primarily presents during childhood, it may occur in adults too. PB tends to be less hostile in infants and children when compared to adults. Histologically, PB is characterized by distinguished acinar and squamoid cell differentiation. Most of these tumors develop in the head of the pancreas and increase alpha-fetoprotein in up to 68% of patients. Ultrasound and CT scan play a significant role in preoperative diagnosis, which is often quite difficult. The best treatment is surgical removal of the pancreas. The role of adjuvant chemotherapy or radiotherapy is still under consideration due to a minor number of patients treated. Chemotherapy regimens consisting of cyclophosphamide, etoposide, doxorubicin, and cisplatin have been used in the neoadjuvant setting with anecdotal benefit. It was found that a poorer prognosis was associated with patients who had metastasis, and patients who could not be operated on surgically.
