This is a case report of a two years old young boy whose abdominal tumor was discovered by a routine examination. The mass was a huge, solid and painless one, located anteriorly in the right upper quadrant of the abdomen. Abdominal X-ray, IVP and ultrasonography showed a calcified mass with a normal right kidney. Urinary catecholamine excretion was significantly increased and the diagnosis of extrasurrenal prerenal sympathoblastoma was preoperatively suspected. Transverse laparotomy founded an encapsulated solid tumor, widening the duodenal loop and developing in the head of the pancreas. A cephalic duodenopancreatectomy was performed with an un eventful post-operative course. Histopathological examination confirmed it was a ganglioneuroblastoma of the head of the pancreas with lymphatic metastasis and local invasion of the neighbouring pancreas with immature tissues. The child was then treated with radiotherapy (35 Grays) and chemotherapy (Oncovin-Endoxan alternated with Oncovin-Adriamycine). The boy is now in good health with a two years follow up. Malignant pancreatic tumors are exceedingly rare in the pediatric age group (about 40 cases in the literature). Most of them are carcinoma and sympathoblastoma is quite exceptional (Research has yielded only one case : Bienaymé, 1976). The presence of a nervous tumor in such an unusual position explains the lack of precision in the preoperative location and brings up the difficult question of prognosis. It leads to pathogenic hypothesis.
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