Agenesis of the dorsal pancreas associated with mucinous adenocarcinoma and cystic teratoma: a case report and literature review

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Key Clinical Message

Dorsal agenesis of the pancreas is a rare congenital anomaly. Fifty‐eight cases were reported from 1913 till 2015, nine of which were associated with tumors. We present the 10th case, the first to be associated with pancreatic mucinous adenocarcinoma and cystic teratoma, successfully managed by Whipple procedure and total pancreatectomy.

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Dorsal pancreas agenesis in retinoic acid-deficient Raldh2 mutant mice.

Karen Niederreither, Jabier Gallego-Llamas, Pascal Dollé … (2005)

During embryogenesis, the pancreas arises from dorsal and ventral pancreatic protrusions from the primitive gut endoderm upon induction by different stimuli from neighboring mesodermal tissues. Recent studies have shown that Retinoic Acid (RA) signaling is essential for the development of the pancreas in non-mammalian vertebrates. To investigate whether RA regulates mouse pancreas development, we have studied the phenotype of mice with a targeted deletion in the retinaldehyde dehydrogenase 2 (Raldh2) gene, encoding the enzyme required to synthesize RA in the embryo. We show that Raldh2 is expressed in the dorsal pancreatic mesenchyme at the early stage of pancreas specification. RA-responding cells have been detected in pancreatic endodermal and mesenchymal cells. Raldh2-deficient mice do not develop a dorsal pancreatic bud. Mutant embryos lack Pdx 1 expression, an essential regulator of early pancreas development, in the dorsal but not the ventral endoderm. In contrast to Pdx 1-deficient mice, the early glucagon-expressing cells do not develop in Raldh2 knockout embryos. Shh expression is, as in the wild-type embryo, excluded from the dorsal endodermal region at the site where the dorsal bud is expected to form, indicating that the dorsal bud defect is not related to a mis-expression of Shh. Mesenchymal expression of the LIM homeodomain protein Isl 1, required for the formation of the dorsal mesenchyme, is altered in Raldh2--/-- embryos. The homeobox gene Hlxb9, which is essential for the initiation of the pancreatic program in the dorsal foregut endoderm, is still expressed in Raldh2--/-- dorsal epithelium but the number of HB9-expressing cells is severely reduced. Maternal supplementation of RA rescues early dorsal pancreas development and restores endodermal Pdx 1 and mesenchymal Isl 1 expression as well as endocrine cell differentiation. These findings suggest that RA signaling is important for the proper differentiation of the dorsal mesenchyme and development of the dorsal endoderm. We conclude that RA synthesized in the mesenchyme is specifically required for the normal development of the dorsal pancreatic endoderm at a stage preceding Pdx 1 function.

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Selective agenesis of the dorsal pancreas in mice lacking homeobox gene Hlxb9.

H. Li, S Arber, T M Jessell … (1999)

The initial stages of pancreatic development occur early during mammalian embryogenesis, but the genes governing this process remain largely unknown. The homeodomain protein Pdx1 is expressed in the developing pancreatic anlagen from the approximately 10-somite stage, and mutations in the gene Pdx1 prevent the development of the pancreas. The initial stages of pancreatic development, however, still occur in Pdx1-deficient mice. Hlxb9 (encoding Hb9; ref. 6) is a homeobox gene that in humans has been linked to dominant inherited sacral agenesis and we show here that Hb9 is expressed at early stages of mouse pancreatic development and later in differentiated beta-cells. Hlxb9 has an essential function in the initial stages of pancreatic development. In absence of Hlxb9 expression, the dorsal region of the gut epithelium fails to initiate a pancreatic differentiation program. In contrast, the ventral pancreatic endoderm develops but exhibits a later and more subtle perturbation in beta-cell differentiation and in islet cell organization. Thus, dorsally Hlxb9 is required for specifying the gut epithelium to a pancreatic fate and ventrally for ensuring proper endocrine cell differentiation.

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Congenital Variants and Anomalies of the Pancreas and Pancreatic Duct: Imaging by Magnetic Resonance Cholangiopancreaticography and Multidetector Computed Tomography

Aysel Türkvatan, Ayşe Erden, Mehmet Türkoğlu … (2013)

Though congenital anomalies of the pancreas and pancreatic duct are relatively uncommon and they are often discovered as an incidental finding in asymptomatic patients, some of these anomalies may lead to various clinical symptoms such as recurrent abdominal pain, nausea and vomiting. Recognition of these anomalies is important because these anomalies may be a surgically correctable cause of recurrent pancreatitis or the cause of gastric outlet obstruction. An awareness of these anomalies may help in surgical planning and prevent inadvertent ductal injury. The purpose of this article is to review normal pancreatic embryology, the appearance of ductal anatomic variants and developmental anomalies of the pancreas, with emphasis on magnetic resonance cholangiopancreaticography and multidetector computed tomography.

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Author and article information

Contributors

Elias Saikaly:

ORCID: http://orcid.org/0000-0003-3187-4694

dreliassaikaly@gmail.com

Journal

Journal ID (nlm-ta): Clin Case Rep

Journal ID (iso-abbrev): Clin Case Rep

Journal ID (doi): 10.1002/(ISSN)2050-0904

Journal ID (publisher-id): CCR3

Title: Clinical Case Reports

Publisher: John Wiley and Sons Inc. (Hoboken )

ISSN (Electronic): 2050-0904

Publication date (Electronic): 23 January 2017

Publication date Collection: February 2017

Volume: 5

Issue: 2 ( doiID: 10.1002/ccr3.2017.5.issue-2 )

Pages: 175-181

Affiliations

[ ¹ ] Faculty of Medicine Saint Georges Hospital University Medical CenterUniversity of Balamand BeirutLebanon

[ ² ] General and Digestive Surgery Oncologic Surgery Faculty of Medicine Saint Georges Hospital University Medical CenterUniversity of Balamand BeirutLebanon

Author notes

[*] [* ] Correspondence

Elias Saikaly, Faculty of Medicine, Saint Georges Hospital University Medical Center, University of Balamand, Beirut, Lebanon. Tel: 00 961 3 810 899; Fax: 00 961 1 567 171; E‐mail: dreliassaikaly@ 123456gmail.com

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Elias Saikaly http://orcid.org/0000-0003-3187-4694

Article

Publisher ID: CCR3797

DOI: 10.1002/ccr3.797

PMC ID: 5290498

PubMed ID: 28174646

SO-VID: 11a3cd23-7fbc-4fe0-8710-d693ac325231

License:

This is an open access article under the terms of the Creative Commons Attribution‐NonCommercial‐NoDerivs License, which permits use and distribution in any medium, provided the original work is properly cited, the use is non‐commercial and no modifications or adaptations are made.

History

Date received : 06 November 2016

Date revision received : 18 November 2016

Date accepted : 08 December 2016

Page count

Figures: 8, Tables: 2, Pages: 7, Words: 3526

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source-schema-version-number 2.0

component-id ccr3797

cover-date February 2017

details-of-publishers-convertor Converter:WILEY_ML3GV2_TO_NLMPMC version:5.0.4 mode:remove_FC converted:03.02.2017

Keywords: agenesis of the dorsal pancreas,pancreatic cystic teratoma,pancreatic mucinous adenocarcinoma,rare pancreatic anomalies

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Keywords: agenesis of the dorsal pancreas, pancreatic cystic teratoma, pancreatic mucinous adenocarcinoma, rare pancreatic anomalies

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Agenesis of the dorsal pancreas associated with mucinous adenocarcinoma and cystic teratoma: a case report and literature review

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Most cited references 20

Dorsal pancreas agenesis in retinoic acid-deficient Raldh2 mutant mice.

Selective agenesis of the dorsal pancreas in mice lacking homeobox gene Hlxb9.

Congenital Variants and Anomalies of the Pancreas and Pancreatic Duct: Imaging by Magnetic Resonance Cholangiopancreaticography and Multidetector Computed Tomography

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Cited by 6

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