[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.
He
L
L
Department of Pathology, Beijing Children's Hospital, Beijing 100045.
Li
P
P
Liu
S
S
Wang
L
L
Liu
N
N
Du
X
X
D002272:Carcinoembryonic Antigen / Q000032:analysis; D002329:Carmine; D002648:Child; D002675:Child, Preschool; D053379:Chromogranin A; D002864:Chromogranins / Q000032:analysis; D004396:Coloring Agents / Q000032:analysis; D005260:Female; D006801:Humans; D007150:Immunohistochemistry; D007223:Infant; D007633:Keratins / Q000032:analysis; D008113:Liver Neoplasms / Q000556:secondary; D008207:Lymphatic Metastasis; D008297:Male; D018396:Mucin-1 / Q000032:analysis; D010190:Pancreatic Neoplasms / Q000378:metabolism / Q000473:pathology*; D010751:Phosphopyruvate Hydratase / Q000032:analysis; D013160:Splenic Neoplasms / Q000556:secondary; D000515:alpha 1-Antitrypsin / Q000032:analysis
D002272:Carcinoembryonic Antigen; D053379:Chromogranin A; D002864:Chromogranins; D004396:Coloring Agents; D018396:Mucin-1; D000515:alpha 1-Antitrypsin; C029618:mucicarmine; D007633:Keratins; D002329:Carmine; D010751:Phosphopyruvate Hydratase