Congenital pancreatoblastoma. A case report

The literature describes 15 cases of congenital pancreatoblastoma (PB): 5 had prenatal diagnosis, none had metastases at diagnosis, 7 were associated with BeckwitheWiedemann syndrome (BWS). In 13 cases resection was radical, while in 2 there were macroscopic residues. Only one patient underwent chemotherapy after distant recurrence. All children are alive except one who died because of problems related to BWS. Our goal is to describe the approach adopted in an infant with congenital PB treated in our center. After a prenatal third semester diagnosis of abdominal anechoic lesion, the radiological investigations (ultrasound, MRI) performed at birth described a cystic lesion of unclear nature. We proceeded to laparoscopic exploration, transformed into open approach after the detection of a lesion located in the body of the pancreas; this lesion was resected, preserving the head and tail of pancreas. The histological diagnosis showed a completely excised PB. After excluding metastatic lesions, we decided to perform only careful follow-up without chemotherapy. The follow-up at 12 months is negative. Although PB is a malignant tumor that requires a multidisciplinary treatment, the congenital cases seem to have a less aggressive biological behavior. The treatment, therefore, in case of complete resection, could be only surgical, followed by a careful follow-up. These forms are often associated with congenital BWS, but in our case the patient did not have the typical characteristics of the syndrome.