Pancreatoblastoma

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[edit] Discussion of Pancreatoblastoma

  • Pancreatoblastoma is the most common pancreatic tumor of young children.
  • The age range is fetus to 9 years.
  • More than half of reported cases are in Asians.
  • Patients most commonly present with an asymptomatic, large abdominal mass.
  • Elevated {alpha}-fetoprotein levels are seen in up to one-third of patients, as seen in other embryonal tumors such as hepatoblastoma and embryonal carcinoma.
  • Congenital cases of pancreatoblastoma have been described in association with Beckwith-Wiedemann syndrome, and these are predominantly cystic in nature.
  • Invasion of adjacent organs and distant metastases may occur.
  • Pancreatoblastomas are best treated with complete surgical resection.
  • The benefit of adjuvant chemotherapy has not been fully elucidated, but chemotherapy is commonly used empirically.
  • The long-term prognosis is good in the absence of metastatic disease with complete surgical resection.
  • Recurrence is common, so long-term follow-up is compulsory.


[edit] Differential Diagnosis

  • When the mass is large and not clearly arising from the pancreas, common tumors of adjacent organs occurring in young children must be considered. These include neuroblastoma, Wilm's tumor, hepatoblastoma, and other primary liver tumors.
  • Predominantly cystic pancreatoblastomas can appear radiologically similar to solid and pseudopapillary epithelial neoplasm


[edit] Imaging Findings for Pancreatoblastoma

  • Often the mass is so large at presentation as to make determination of the organ of origin quite difficult.
  • These large tumors typically compress surrounding structures without appearing to invade them, although local invasion may be evident at surgical resection.
  • At sonography, the majority of pancreatoblastomas appear as well-circumscribed heterogeneous masses with solid and cystic components.
  • At CT, the mass is most commonly well or partially circumscribed, although an infiltrative margin is uncommonly encountered. The tumor is often smooth and may be multilobulated. Small punctate, clustered, or curvilinear calcifications may be identified.
  • On MRI, a pancreatoblastomas are well marginated with low to intermediate signal intensity on T1-weighted images and heterogeneous high signal intensity on T2-weighted images.

[edit] Images

Patient #1


[edit] External Links

[edit] References for Pancreatoblastoma

  • Ellen M. Chung, Mark D. Travis, and Richard M. Conran. [From the Archives of the AFIP: Pancreatic Tumors in Children: Radiologic-Pathologic Correlation.] RadioGraphics 2006 26: 1211-1238.
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