Revisiting metastatic adult pancreatoblastoma. A case and review of the literature.

UNLABELLED: CONTEX: 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.