Diagnosis and treatment of pancreatoblastoma in China.
OBJECTIVE
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.
Cao
Liping
L
Department of Surgery, Second Affiliated Hospital, School of Medicine, Zhejiang University, Zhejiang, People's Republic of China. cao@zju.edu.cn
Liu
Daren
D
D000971:Antineoplastic Combined Chemotherapy Protocols / Q000008:administration & dosage*; D002675:Child, Preschool; D002681:China; D003131:Combined Modality Therapy; D005260:Female; D006801:Humans; D008297:Male; D010190:Pancreatic Neoplasms / Q000175:diagnosis / Q000188:drug therapy / Q000601:surgery; D016577:Pancreaticoduodenectomy; D015996:Survival Rate; D016896:Treatment Outcome