Pancreatoblastoma in childhood: Clinical course and therapeutic managementof seven patients

Background To analyze the clinical course of pancreatoblastoma in children and to propose management and therapy. Procedure. Retrospective review of s even 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 t issues in two others. Three patients had regional lymph node involvement an d 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 neoadjuvan t chemotherapy, and cisplatin plus doxorubicin seemed effective. Two chidre n received post-operative irradiation because of incomplete resection. In a ll, four children are disease free with a median followup of 50 months ( ra nge, 5-120 months) : one had a complete removal of tumor at diagnosis and n o further treatment, th ree had un resectable tumor at diagnosis and receiv ed neoadjuvant chemotherapy with cisplatin and doxorubicin. One of them als o 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 treatmen t of choice. However, tumor is often unresectable at diagnosis and preopera tive chemotherapy is needed to reduce tumor volume. We suggest a regimen th at include cisplatin and doxorubicin. In patients with incompletely resecte d disease, postoperative radiation may be indicated. (C) 2001 Wiley-Liss, I nc.