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Diagnosis and treatment of pancreatoblastoma in China
1Department of Surgery, Second Affiliated Hospital, School of Medicine, Zhejiang University, Zhejiang, People's Republic of China. cao@zju.edu.cn
Objectives:
Pancreatoblastoma is an extremely rare pancreatic neoplasm. The aims of the current study were to analyze the clinical, pathological, and immunohistochemical features of pancreatoblastoma and to discuss its management.
Methods:
The clinical data of 30 Chinese pancreatoblastoma patients (among them, 1 case was treated by authors, and the other 29 cases were reported in Chinese literature from January 1981 to December 2005) were reviewed and analyzed.
Results:
Patients with pancreatoblastoma had an age range of 5 months to 42 years. There were 24 children and 6 adult cases, with a male-female ratio of 2:1 (20/10). The tumor size ranged from 2 to 20 cm in diameter. Metastases were found during the operation in 8 cases (26.7%) (among them, 66% with liver invasion and 50% with multiple metastases including spleen, kidney, and bone). Pancreatoduodenectomy was performed in 5 cases (16.7%). Nine cases (30%) received chemotherapy with vincristine, cyclophosphamide, and adriamycin. By immunohistochemistry, the tumors exhibited acinar, endocrine, and ductal differentiation. The mean time of survival was 25.5 months.
Conclusions:
These results suggest that the diagnosis of pancreatoblastoma mainly depends on the pathological findings, surgical resection is the most effective way for cure, and adjuvant chemotherapy could be a good palliative method in certain patients.
