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[A clinicopathological study of pancreatoblastoma]
Objective:
To investigate the clinicopathological, immunohistochemical features and the histogenesis of pancreatoblastoma (PB) in 14 pediatric cases, including 9 male and 5 female patients, their age ranged from 1.5 to 8 years with a mean of 4.4 years.
Methods:
Routine pathological, histochemical, and immunohistochemical methods were utilized to analyse the PB specimens.
Results:
Tumors of 6 patients had metastasized to the liver, spleen and lymph nodes, 8 patients were alive 10 months to 105 months after diagnosis. Histologically, the tumors were composed of dense epithelial elements separated by fibro-stromal tissue, resulting in a nesting or organoid pattern. Immunohistochemically and histochemically, the tumors exhibited acinar, endocrine and ductal differentiation. The positive rates for were: CK 100%, EMA 86%, NSE 79%, CEA 71%, SYN 64%, CgA 43%, alpha1-AT 57%, PAS 100% and mucicarmine 57%.
Conclusions:
PB is the most common malignant pancreatic neoplasm of childhood. PB arises from primitive pluripotential cells capable of differentiating into 3 pancreatic cell types: acinar, endocrine and ductal. The prognosis is better than pancreatic carcinoma in adults.