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lüll Revisiting metastatic adult pancreatoblastoma A case and review of the literature Charlton-Ouw KM; Kaiser CL; Tong GX; Allendorf JD; Chabot JAJOP 2008[Nov]; 9 (6): 733-8CONTEX: Most cases of pancreatoblastoma, a rare tumor of neuroendocrine origin, are seen in the pediatric population. To date, at least sixteen case reports have been described of pancreatoblastoma in patients 19-year old or older. Surgical resection is the mainstay of curative treatment. Even patients with liver metastasis can have long-term disease-free survival. CASE REPORT: One recent example is a 33-year-old male who presented to us for a right hepatic lobectomy for removal of the presumed primary tumor - later discovered to be a metastasis - followed by pancreaticoduodenectomy for resection of the true primary lesion. Five years after resection, this patient is the longest disease-free survivor of metastatic adult pancreatoblastoma. CONCLUSION: We review the literature and propose that resection of pancreatoblastoma can offer long-term disease-free survival even with liver metastasis and microscopically-positive surgical margins.|Adult[MESH]|Carcinoma, Neuroendocrine/*diagnosis[MESH]|Chemotherapy, Adjuvant[MESH]|Combined Modality Therapy[MESH]|Diagnosis, Differential[MESH]|Humans[MESH]|Liver Neoplasms/*diagnosis/*secondary/therapy[MESH]|Male[MESH]|Pancreatic Neoplasms/classification/*diagnosis/*therapy[MESH]|Pancreaticoduodenectomy[MESH]|Radiotherapy, Adjuvant[MESH]|Rare Diseases[MESH]|Reoperation[MESH]|Survivors[MESH]|Treatment Outcome[MESH] |