Skip to main content
Top
Published in: Journal of Gastrointestinal Surgery 9/2011

01-09-2011 | Case Report

The Novel Triad of Dorsal Agenesis of the Pancreas with Concurrent Pancreatic Ductal Adenocarcinoma and Nonalcoholic Chronic Calcific Pancreatitis: A Case Series and Review of the Literature

Authors: David W. Rittenhouse, Eugene P. Kennedy, Andres A. Mascaro, Jennifer L. Brumbaugh, Louis H. Stein, Laura H. Rosenberger, Patricia K. Sauter, Charles J. Yeo, Harish Lavu

Published in: Journal of Gastrointestinal Surgery | Issue 9/2011

Login to get access

Abstract

Introduction

Dorsal agenesis of the pancreas (DAP) is a rare congenital anomaly, with only 44 cases having been reported in the English literature since 1966.

Materials and Methods

A retrospective review of our IRB-approved pancreatic surgery database was performed from November 2005 to November 2010 searching for cases of DAP.

Discussion

Disorders in the retinoic acid (Raldh) and hedgehog (Hh) signaling pathways, which appear to play a role in the development of DAP, have been implicated in other diseases of the pancreas such as pancreatic ductal adenocarcinoma (PDA) and nonalcoholic chronic calcific pancreatitis (NCCP).

Conclusion

In this report, we describe three cases of DAP in the setting of PDA, two of which include the third component of NCCP. We provide a discussion of the clinical features of this novel triad and address the molecular pathways that relate to these respective diseases.
Literature
1.
go back to reference Wang JT, Lin JT, Chuang CN, Wang SM, Chuang LM, Chen JC, Huang SH, Chen DS, Wang TH. Complete agenesis of the dorsal pancreas--a case report and review of the literature. Pancreas 1990;5:493–7.PubMedCrossRef Wang JT, Lin JT, Chuang CN, Wang SM, Chuang LM, Chen JC, Huang SH, Chen DS, Wang TH. Complete agenesis of the dorsal pancreas--a case report and review of the literature. Pancreas 1990;5:493–7.PubMedCrossRef
2.
go back to reference Sakpal SV, Sexcius L, Babel N, Chamberlain RS. Agenesis of the dorsal pancreas and its association with pancreatic tumors. Pancreas 2009;38:367–73.PubMedCrossRef Sakpal SV, Sexcius L, Babel N, Chamberlain RS. Agenesis of the dorsal pancreas and its association with pancreatic tumors. Pancreas 2009;38:367–73.PubMedCrossRef
3.
4.
go back to reference Chari ST, Mohan V, Pitchumoni CS, Viswanathan M, Madanagopalan N, Lowenfels AB. Risk of pancreatic carcinoma in tropical calcifying pancreatitis: an epidemiologic study. Pancreas 1994;9:62–6.PubMedCrossRef Chari ST, Mohan V, Pitchumoni CS, Viswanathan M, Madanagopalan N, Lowenfels AB. Risk of pancreatic carcinoma in tropical calcifying pancreatitis: an epidemiologic study. Pancreas 1994;9:62–6.PubMedCrossRef
5.
go back to reference Matsusue S, Kashihara S, Koizumi S. Pancreatectomy for carcinoma of the head of the pancreas associated with multiple anomalies including the preduodenal portal vein. Jpn J Surg 1984;14:394–8.PubMedCrossRef Matsusue S, Kashihara S, Koizumi S. Pancreatectomy for carcinoma of the head of the pancreas associated with multiple anomalies including the preduodenal portal vein. Jpn J Surg 1984;14:394–8.PubMedCrossRef
6.
go back to reference Ulusan S, Yakar T, Koc Z, Kayaselcuk F, Torer N. Adenocarcinoma of the pancreas associated with dorsal agenesis. Pancreas 2006;33:437–9.PubMedCrossRef Ulusan S, Yakar T, Koc Z, Kayaselcuk F, Torer N. Adenocarcinoma of the pancreas associated with dorsal agenesis. Pancreas 2006;33:437–9.PubMedCrossRef
7.
go back to reference Balakrishnan V, Narayanan VA, Siyad I, Radhakrishnan L, Nair P. Agenesis of the dorsal pancreas with chronic calcific pancreatitis. case report, review of the literature and genetic basis. Jop 2006;7:651–9PubMed Balakrishnan V, Narayanan VA, Siyad I, Radhakrishnan L, Nair P. Agenesis of the dorsal pancreas with chronic calcific pancreatitis. case report, review of the literature and genetic basis. Jop 2006;7:651–9PubMed
8.
go back to reference Skandalakis LJ, Rowe JS, Jr., Gray SW, Skandalakis JE. Surgical embryology and anatomy of the pancreas. Surg Clin North Am 1993;73:661–97.PubMed Skandalakis LJ, Rowe JS, Jr., Gray SW, Skandalakis JE. Surgical embryology and anatomy of the pancreas. Surg Clin North Am 1993;73:661–97.PubMed
9.
go back to reference Sadler TW. Susceptible periods during embryogenesis of the heart and endocrine glands. Environ Health Perspect 2000;108 Suppl 3:555–61.PubMedCrossRef Sadler TW. Susceptible periods during embryogenesis of the heart and endocrine glands. Environ Health Perspect 2000;108 Suppl 3:555–61.PubMedCrossRef
10.
go back to reference Schnedl WJ, Piswanger-Soelkner C, Wallner SJ, Reittner P, Krause R, Lipp RW, Hohmeier HE. Agenesis of the dorsal pancreas and associated diseases. Dig Dis Sci 2009;54:481–7.PubMedCrossRef Schnedl WJ, Piswanger-Soelkner C, Wallner SJ, Reittner P, Krause R, Lipp RW, Hohmeier HE. Agenesis of the dorsal pancreas and associated diseases. Dig Dis Sci 2009;54:481–7.PubMedCrossRef
11.
go back to reference Li H, Arber S, Jessell TM, Edlund H. Selective agenesis of the dorsal pancreas in mice lacking homeobox gene Hlxb9. Nat Genet 1999;23:67–70.PubMed Li H, Arber S, Jessell TM, Edlund H. Selective agenesis of the dorsal pancreas in mice lacking homeobox gene Hlxb9. Nat Genet 1999;23:67–70.PubMed
12.
go back to reference Martin M, Gallego-Llamas J, Ribes V, Kedinger M, Niederreither K, Chambon P, Dolle P, Gradwohl G. Dorsal pancreas agenesis in retinoic acid-deficient Raldh2 mutant mice. Dev Biol 2005;284:399–411.PubMedCrossRef Martin M, Gallego-Llamas J, Ribes V, Kedinger M, Niederreither K, Chambon P, Dolle P, Gradwohl G. Dorsal pancreas agenesis in retinoic acid-deficient Raldh2 mutant mice. Dev Biol 2005;284:399–411.PubMedCrossRef
13.
go back to reference Kayed H, Kleeff J, Osman T, Keleg S, Buchler MW, Friess H. Hedgehog signaling in the normal and diseased pancreas. Pancreas 2006;32:119–29.PubMedCrossRef Kayed H, Kleeff J, Osman T, Keleg S, Buchler MW, Friess H. Hedgehog signaling in the normal and diseased pancreas. Pancreas 2006;32:119–29.PubMedCrossRef
14.
go back to reference Singh B, Murphy RF, Ding XZ, Roginsky AB, Bell RH, Jr., Adrian TE. On the role of transforming growth factor-beta in the growth inhibitory effects of retinoic acid in human pancreatic cancer cells. Mol Cancer 2007;6:82.PubMedCrossRef Singh B, Murphy RF, Ding XZ, Roginsky AB, Bell RH, Jr., Adrian TE. On the role of transforming growth factor-beta in the growth inhibitory effects of retinoic acid in human pancreatic cancer cells. Mol Cancer 2007;6:82.PubMedCrossRef
15.
go back to reference Bhatia E, Choudhuri G, Sikora SS, Landt O, Kage A, Becker M, Witt H. Tropical calcific pancreatitis: strong association with SPINK1 trypsin inhibitor mutations. Gastroenterology 2002;123:1020–5.PubMedCrossRef Bhatia E, Choudhuri G, Sikora SS, Landt O, Kage A, Becker M, Witt H. Tropical calcific pancreatitis: strong association with SPINK1 trypsin inhibitor mutations. Gastroenterology 2002;123:1020–5.PubMedCrossRef
16.
go back to reference Parsaik AK, Murad MH, Sathananthan A, Moorthy V, Erwin PJ, Chari S, Carter RE, Farnell MB, Vege SS, Sarr MG, Kudva YC. Metabolic and target organ outcomes after total pancreatectomy: Mayo Clinic experience and meta-analysis of the literature. Clin Endocrinol (Oxf) 2010;73(6):723–31CrossRef Parsaik AK, Murad MH, Sathananthan A, Moorthy V, Erwin PJ, Chari S, Carter RE, Farnell MB, Vege SS, Sarr MG, Kudva YC. Metabolic and target organ outcomes after total pancreatectomy: Mayo Clinic experience and meta-analysis of the literature. Clin Endocrinol (Oxf) 2010;73(6):723–31CrossRef
17.
go back to reference Maeda H. [Ductal and vascular factors in the etiology of experimentally induced pancreatic fibrosis and fat replacement in dogs]. Nippon Shokakibyo Gakkai Zasshi 1986;83:2580–7PubMed Maeda H. [Ductal and vascular factors in the etiology of experimentally induced pancreatic fibrosis and fat replacement in dogs]. Nippon Shokakibyo Gakkai Zasshi 1986;83:2580–7PubMed
18.
go back to reference Watanabe S, Abe K, Anbo Y, Katoh H. Changes in the mouse exocrine pancreas after pancreatic duct ligation: a qualitative and quantitative histological study. Arch Histol Cytol 1995;58:365–74.PubMedCrossRef Watanabe S, Abe K, Anbo Y, Katoh H. Changes in the mouse exocrine pancreas after pancreatic duct ligation: a qualitative and quantitative histological study. Arch Histol Cytol 1995;58:365–74.PubMedCrossRef
19.
go back to reference Toyama N, Kamiyama H, Suminaga Y, Namai K, Ota M, Konishi F. Pancreas head carcinoma with total fat replacement of the dorsal exocrine pancreas. J Gastroenterol 2004;39:76–80.PubMedCrossRef Toyama N, Kamiyama H, Suminaga Y, Namai K, Ota M, Konishi F. Pancreas head carcinoma with total fat replacement of the dorsal exocrine pancreas. J Gastroenterol 2004;39:76–80.PubMedCrossRef
Metadata
Title
The Novel Triad of Dorsal Agenesis of the Pancreas with Concurrent Pancreatic Ductal Adenocarcinoma and Nonalcoholic Chronic Calcific Pancreatitis: A Case Series and Review of the Literature
Authors
David W. Rittenhouse
Eugene P. Kennedy
Andres A. Mascaro
Jennifer L. Brumbaugh
Louis H. Stein
Laura H. Rosenberger
Patricia K. Sauter
Charles J. Yeo
Harish Lavu
Publication date
01-09-2011
Publisher
Springer-Verlag
Published in
Journal of Gastrointestinal Surgery / Issue 9/2011
Print ISSN: 1091-255X
Electronic ISSN: 1873-4626
DOI
https://doi.org/10.1007/s11605-011-1542-6

Other articles of this Issue 9/2011

Journal of Gastrointestinal Surgery 9/2011 Go to the issue