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Medline ® Abstract for Reference 43

of 'Pathology of exocrine pancreatic neoplasms'

43
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Management of pancreatoblastoma in children and young adults.
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Glick RD, Pashankar FD, Pappo A, Laquaglia MP
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J Pediatr Hematol Oncol. 2012;34 Suppl 2:S47.
 
Pancreatoblastoma is a very rare childhood tumor originating from the epithelial exocrine cells of the pancreas. It is the most common malignant pancreatic tumor in young children and has a mean age of diagnosis of 5 years. It is slow growing and its presentation is varied and often non-specific. Tumors tend to be quite large and appropriate cross sectional imaging is very important to assess for extent, metastatic disease, and resectability. Biopsy for tissue diagnosis is essential. Complete surgical resection is the goal of therapy although many patients are unresectable at initial diagnosis and require neoadjuvant chemotherapy. Adjuvant chemotherapy is also recommended and chemotherapeutic regimens involve cisplatin and doxorubicin. Even with curative resections, these lesions have a high recurrence rate and patients must be followed closely. Knowledge of this rare tumor is important for the clinician confronted with a large retroperitoneal mass in a young child.
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Steven and Alexandra Cohen Children's Medical Center, Hofstra North Shore-LIJ School of Medicine, New Hyde Park, NY 11040, USA. rglick@nshs.edu
PMID