Therapeutic management of rare malignant pancreatic tumors in children.

Malignant pancreatic tumors in children are rare. The major problem for the clinician is a lack of experience and of accepted therapeutic strategies. Malignant pancreatic tumors in children show a pattern different from that in adults. In infants, especially pancreatoblastomas, solid cystic tumors of females, and endocrine carcinomas of the pancreas must be expected. We report our experience in three patients with malignant pancreatic tumors (one pancreatoblastoma and two malignant endocrine pancreatic carcinomas) and review the present literature with a focus on the typical clinical and biologic features and the presently recommended therapeutic strategies. Pancreatoblastomas and solid cystic tumors are mainly found in the head of the pancreas. Fibrotic capsules with rare, late metastases are characteristics of these tumors, indicating total resection to be an important therapeutic procedure. Pancreatoblastomas should additionally be treated with chemotherapy (ADM, IFO, cis-PL, VP16). Endocrine carcinomas of the pancreas (malignant gastrinomas and malignant insulinomas) should also primarily be treated with radical surgery, including extensive lymph node dissection. In case of distant metastases, local resection (liver) or somatostatin in combination with chemotherapy (streptozocin in the case of malignant insulinomas) may be used.