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Published on: November 20, 2021
Pancreatoblastoma in children: Clinical management and literature review
Tingting Liu1, Tong Zhao2, Cuicui Shi3
1Department of Pediatric Hematology/Oncology, Xinhua Hospital, Shanghai Jiaotong University School of Medicine, Shanghai, China.
Insights
This study analyzed pancreatoblastoma (PB) clinical features and identified risk factors for better management. Combination therapy showed favorable outcomes in relapsed or metastatic PB cases.
Area of Science:
- Oncology
- Pathology
Background:
- Pancreatoblastoma (PB) is a rare pancreatic neoplasm.
- Understanding its clinical and pathological features is crucial for effective patient management.
Purpose of the Study:
- To analyze the clinical and pathological features of pancreatoblastoma.
- To identify optimal management strategies for relapsed or metastatic PB.
Main Methods:
- Review of four institutional cases and 59 literature cases (PubMed, 2000-2020).
- Analysis of clinical presentation, pathological findings, and treatment outcomes.
Main Results:
- PB cases presented with abdominal pain and masses (5.2-18 cm).
- Risk factors included invasion of splenic vein, superior mesenteric artery, duodenum, and lymph nodes.
- Combination therapy yielded favorable outcomes; chemotherapy alone was insufficient.
- Squamous corpuscles were a defining histological feature.
Conclusions:
- Multidisciplinary diagnosis is vital for PB clinical management.
- Therapeutic stratification should consider identified risk factors before surgery.
Purpose:
The aim of this study is to analyze the clinical and pathological features of pancreatoblastoma (PB) and to obtain better management for patients with relapsed or metastatic disease.
Methods:
Four cases treated in our institution and 59 cases reported previously in the literature from the PubMed biomedical database (2000-2020) were reviewed and analyzed.
Results:
Four cases with PB presented with abdominal pain and palpable abdominal masses, with the tumor size ranging from 5.2 to 18 cm in diameter. The invasion of the splenic vein and superior mesenteric artery, duodenum, and lymph nodes were risk factors for PB. Three cases were treated with combination therapy and showed favorable outcomes, while one case was treated with chemotherapy alone due to tumor progression and died of the disease. Squamous corpuscles were revealed in the tumor samples and considered a defining component for histological diagnosis.
Conclusions:
Multidisciplinary diagnosis plays an important role in clinical management. The risk factors should be considered in the therapeutic stratification of PB before surgery.
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