Pancreatoblastoma in childhood:: Clinical course and therapeutic management of seven patients

Background To analyze the clinical course of pancreatoblastoma in children and to propose management and therapy. Procedure. Retrospective review of seven cases of pancreatoblastoma treated in France over a 20-year period and literature review. Results, Median age of patients was 6 years. The tumor was well encapsulated in one patient and had direct extension to adjacent tissues in two others. Three patients had regional lymph node involvement and one had liver metastases. The tumor arose in the head of the pancreas in three children, in the tail and body in two and in the tail and in the body, one each. Elevation of AFP serum levels was noted in four out of 6 cases. Five tumor resections were performed, one initially and 4 after neoadjuvant chemotherapy, and cisplatin plus doxorubicin seemed effective. Two chidren received post-operative irradiation because of incomplete resection. In all, four children are disease free with a median followup of 50 months ( range, 5-120 months) : one had a complete removal of tumor at diagnosis and no further treatment, th ree had un resectable tumor at diagnosis and received neoadjuvant chemotherapy with cisplatin and doxorubicin. One of them also received post-operative irradiation. Conclusions. Pancreatoblastoma is a curable tumor. Examination of serum AFP levels may be useful for diagnosis and to follow the course of the disease. Complete resection is the treatment of choice. However, tumor is often unresectable at diagnosis and preoperative chemotherapy is needed to reduce tumor volume. We suggest a regimen that include cisplatin and doxorubicin. In patients with incompletely resected disease, postoperative radiation may be indicated. (C) 2001 Wiley-Liss, Inc.