PANCREATOBLASTOMA IN CHILDREN - CASE-REPORT AND REVIEW OF THE LITERATURE

Pancreatoblastomas are rare embryonal malignancies in childhood. We re port a 3-year-old girl with a tumor of the head of pancreas. Staging b y bone scintigraphy and CT scans of abdomen and chest did not show evi dence of metastatic disease. Tumor markers showed elevated levels of a lpha-1 fetoprotein (64 ng/ml; normal 0-10 ng/ml) and lactate dehydroge nase (423 U/l; normal range below 300 U/l). The tumor was macroscopica lly completely removed by local resection. Postoperative tumor grading was pT1, NO, MO. The child recovered very soon after surgery without severe complications. Tumor markers dropped to normal values, indicati ng complete remission (follow-up time 12 months). According to the bio logical growth characteristics of pancreatoblastomas and to the litera ture, localized and non-metastatic tumors should be completely resecte d without radical pancreatoduodenectomy and without adjuvant chemother apy. This is the most conservative therapy with a good prognosis. Howe ver metastatic disease, primarily inoperable conditions or local relap ses are indications for chemotherapy combined with radiotherapy and fo llowed by resection of the tumor. At present, the prognosis of such ca ses is rather poor.