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Review
. 2018 Jun 30;12(1):185.
doi: 10.1186/s13256-018-1733-9.

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

A Erotokritou  1 , C D Gerharz  2 , A Sagir  3
Affiliations
Review

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

A Erotokritou et al. J Med Case Rep. .

Abstract

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.

Keywords: Agenesis; Neuroendocrine; Pancreas; Tumor.

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Conflict of interest statement

Ethics approval and consent to participate

Not applicable.

Consent for publication

Written informed consent was obtained from the patient for publication of this case report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.

Competing interests

The authors declare that they have no competing interests.

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Figures

Fig. 1
Fig. 1
Contrast-enhanced computed tomography scan with axial image in arterial phase (a) and coronal reconstructed image in venous phase (b) showing a round hypervascular mass, measuring approximately 3 cm at the neck of the pancreas with absence of the body and tail of the pancreas. The arrows show the lesion in the pancreas
Fig. 2
Fig. 2
Well-differentiated neuroendocrine tumor with solid tumor cell aggregates; hematoxylin and eosin (a) exhibiting intensive cytoplasmic staining for synaptophysin (b), and nuclear staining for Ki-67 in less than 2% of the tumor cells (c)
See this image and copyright information in PMC

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