Malignant pancreatic tumors in childhood and adolescence: The Memorial Sloan-Kettering experience, 1967 to present.
BACKGROUND
Malignant tumors of the pancreas are uncommon in children and adolescents and only recently have the most common tumor types been well characterized. As a result, the treatment approach to these patients has yet to be standardized, and much of the information available in the literature, particularly with regard to the role of chemotherapy and radiation, is anecdotal.


METHODS
A retrospective review was undertaken of all patients less than 21 years of age with malignant pancreatic tumors who were cared for at Memorial Sloan-Kettering since 1967.


RESULTS
Seventeen patients were identified. The pathologic types were pancreatoblastoma, 5; solid pseudopapillary tumor, 7; acinar cell carcinoma, 1; nonfunctioning pancreatic endocrine neoplasm, 1; malignant VIPoma, 1; and PNET, 2. A complete resection of the primary tumor was achieved in 82%, and 12 of 15 are alive, 10 with no evidence of disease. Chemotherapy or radiation were used in selected cases.


CONCLUSIONS
Unlike malignant pancreatic tumors in adults, tumors in children and adolescents usually are resectable, and long-term survival is likely. However, the risk of recurrence for pancreatoblastoma is high. The roles of chemotherapy and radiation remain undefined.
Shorter
Nicholas A
NA
Department of Surgery and Pathology, Memorial Sloan-Kettering Cancer Center, New York, NY, USA.
Glick
Richard D
RD
Klimstra
David S
DS
Brennan
Murray F
MF
Laquaglia
Michael P
MP
D000293:Adolescent; D000328:Adult; D018267:Carcinoma, Acinar Cell / Q000473:pathology / Q000628:therapy; D017024:Chemotherapy, Adjuvant; D002648:Child; D002675:Child, Preschool; D003131:Combined Modality Therapy; D018572:Disease-Free Survival; D005260:Female; D006801:Humans; D008113:Liver Neoplasms / Q000556:secondary; D008175:Lung Neoplasms / Q000556:secondary; D008297:Male; D009364:Neoplasm Recurrence, Local; D010180:Pancreatectomy; D010190:Pancreatic Neoplasms / Q000175:diagnosis* / Q000628:therapy*; D018714:Radiotherapy, Adjuvant; D012189:Retrospective Studies; D018570:Risk Assessment; D016896:Treatment Outcome; D003969:Vipoma / Q000473:pathology / Q000628:therapy