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


METHODS
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.


CONCLUSIONS
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.
Charlton-Ouw
Kristofer M
KM
Department of Surgery, New York-Presbyterian Hospital, Columbia University Medical Center, New York, NY 10032, USA.
Kaiser
Christy L
CL
Tong
Guo Xia
GX
Allendorf
John D
JD
Chabot
John A
JA
D000328:Adult; D018278:Carcinoma, Neuroendocrine / Q000175:diagnosis*; D017024:Chemotherapy, Adjuvant; D003131:Combined Modality Therapy; D003937:Diagnosis, Differential; D006801:Humans; D008113:Liver Neoplasms / Q000175:diagnosis* / Q000556:secondary* / Q000628:therapy; D008297:Male; D010190:Pancreatic Neoplasms / Q000145:classification / Q000175:diagnosis* / Q000628:therapy*; D016577:Pancreaticoduodenectomy; D018714:Radiotherapy, Adjuvant; D035583:Rare Diseases; D012086:Reoperation; D017741:Survivors; D016896:Treatment Outcome