Agenesis of dorsal pancreas associated with pancreatic neuroendocrine tumor: a case report and review of the literature

BACKGROUND: Agenesis of the dorsal pancreas is very rare. Less than 70 cases have been reported to date. Some of these cases had an association with a tumor. The literature of agenesis of the dorsal pancreas and agenesis of the dorsal pancreas-associated pancreatic neoplasia is limited. Here we report the second case of a pancreatic neuroendocrine tumor in a setting of agenesis of the dorsal pancreas. CASE PRESENTATION: A 71-year-old man, originally from North Africa, with a history of insulin-dependent diabetes mellitus, presented with a 2-month history of nonspecific abdominal symptoms. Contrast-enhanced computed tomography demonstrated an almost 3 cm round, quite well-defined and homogeneous tumor formation in the area between the neck and absent body and tail of his pancreas. The mass was confirmed by endoscopic ultrasound. Our patient underwent computed tomography-guided biopsy of the mass which provided proof of a neuroendocrine tumor. He underwent pancreas resection because of the presence of a neuroendocrine tumor. Seven months later his glycated hemoglobin increased from 6.9 to 8.7%. CONCLUSIONS: Diagnosis of agenesis of the dorsal pancreas is based on imaging techniques like computed tomography, magnetic resonance cholangiopancreatography, or endoscopic ultrasound. Endoscopic ultrasound-guided fine-needle aspiration can be helpful for the histological diagnosis of the tumor. The hypothesis of the association between pancreatic neoplasia and agenesis of the dorsal pancreas leads us to the suggestion that every patient with diagnosed agenesis of the dorsal pancreas should be observed with a focus on the early detection of potential malignancy.