title: Management of pancreatoblastoma in children and young adults.
abstract: 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.
authors.affiliation: Steven and Alexandra Cohen Children's Medical Center, Hofstra North Shore-LIJ School of Medicine, New Hyde Park, NY 11040, USA. rglick@nshs.edu
authors.forename: Richard D
authors.initials: RD
authors.lastname: Glick
authors.forename: Farzana D
authors.initials: FD
authors.lastname: Pashankar
authors.forename: Alberto
authors.initials: A
authors.lastname: Pappo
authors.forename: Michael P
authors.initials: MP
authors.lastname: Laquaglia
mesh_terms: D000293:Adolescent; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use*; D002648:Child; D002675:Child, Preschool; D002945:Cisplatin / Q000627:therapeutic use; D004317:Doxorubicin / Q000627:therapeutic use; D005260:Female; D006801:Humans; D008297:Male; D009362:Neoplasm Metastasis; D009364:Neoplasm Recurrence, Local / Q000473:pathology* / Q000517:prevention & control*; D010190:Pancreatic Neoplasms / Q000473:pathology* / Q000628:therapy*; D055815:Young Adult
chemical_list: D004317:Doxorubicin; D002945:Cisplatin