International Journal of Surgery xxx (2015) 1e3

Contents lists available at ScienceDirect

International Journal of Surgery journal homepage: www.journal-surgery.net

Case report

“Ductal adenocarcinoma in anular pancreas” Giacomo Benassai*, Stefano Perrotta, Ermenegildo Furino, Carlo De Werra, Sergio Aloia, Roberto Del Giudice, Bruno Amato, Gabriele Vigliotti, Gennaro Limite, Gennaro Quarto  degli Studi di Napoli “Federico II”, Dipartimento Universitario di Medicina Clinica e Chirurgia Direttore Prof Giovanni Di Minno, Italy Universita

a r t i c l e i n f o

a b s t r a c t

Article history: Received 10 March 2015 Received in revised form 19 March 2015 Accepted 10 April 2015 Available online xxx

Introduction: The annular pancreas is a congenital anomaly in which pancreatic tissue partially or completely surrounds the second portion of the duodenum. Its often located above of papilla of Vater (85%), rarely below (15%). This pancreatic tissue is often easily dissociable to the duodenum but there is same cases where it the tissue is into the muscolaris wall of the duodenum. Material and methods: We describe three case of annular pancreas hospitalized in our facility between January 2004 and January 2009. There were 2 male 65 and 69 years old respectively and 1 female of 60 years old, presented complaining of repeated episodes of mild epigastric pain. Laboratory tests (including tumor markers), a direct abdomen X-ray with enema, EGDS and total body CT scan were performed to study to better define the diagnosis. EUS showed the presence of tissue infiltrating the muscle layer all around the first part of duodenum. Biopsies performed found the presence of pancreatic tissue with focal areas of adenocarcinoma. Subtotal gastrectomy with Roux was performed. The histological examinations shows an annular pancreas of D1 with multiple focal area of adenocarcinoma. (T1aN0M0). Results: We performed a follow up at 5 years. One patients died after 36 months for cardiovascular hit. Two patients, one male and one female, was 5-years disease-free. Discussion: Annular pancreas is an uncommon congenital anomaly which usually presents itself in infants and newborn. Rarely it can present in late adult life with wide range of clinical severities thereby making its diagnosis difficult. Pre-operative diagnosis is often difficult. CT scan can illustrate the pancreatic tissue encircling the duodenum. ERCP and MRCP are useful in outlining the annular pancreatic duct. Surgery still remains necessary to confirm diagnosis and bypassing the obstructed segment. © 2015 IJS Publishing Group Limited. Published by Elsevier Ltd. All rights reserved.

Keywords: Ectopic pancreas Anular pancreas Gastric cancer

1. Introduction Congenital anomalies of the pancreas are extremely rare, often characterized by nonspecific clinical symptoms. So the response is very often completely fortuitous. The changes of the phases of the development are responsible for different forms of clinically detected anomalies [1,2]. The annular pancreas is a congenital anomaly in which pancreatic tissue partially or completely surrounds the second portion of the duodenum. Its often located above of papilla of Vater (85%), rarely below (15%). This pancreatic tissue is often easily dissociable to the duodenum but there is same cases where it the tissue is into the muscolaris wall of the duodenum. Rarely annular

* Corresponding author. Dipartimento Universitario di Medicina Clinica e Chirurgia, A.O.U. Federico II, Via Sergio Pansini no 5, Italy. E-mail address: [email protected] (G. Benassai).

pancreas develops all around the second portion of duodenum, causing sub-occlusion symptoms. This is also very rare anomaly, described for the first time in 1818 by Tiedemann. It appears asymptomatic, especially in infancy, while in adulthood, nonspecific symptoms may appear to relate mainly to obstruction of the second duodenal portion. It is due to abnormalities of the normal embryogenetic development of the pancreas itself. Its normally formed from the fusion of the dorsal and ventral pancreatic buds between the first 4e8 weeks of embryonic life. Annular pancreas results due to failure of the ventral bud to rotate and elongates to encircle the upper part of the duodenum. Annular pancreas is one of few medical conditions that can present with a wide range of clinical severities. It affects neonates to the elderly, thereby making the diagnosis difficult [3e8]. 2. Material and methods We describe tree case of annular pancreas hospitalized in our

http://dx.doi.org/10.1016/j.ijsu.2015.04.086 1743-9191/© 2015 IJS Publishing Group Limited. Published by Elsevier Ltd. All rights reserved.

Please cite this article in press as: G. Benassai, et al., “Ductal adenocarcinoma in anular pancreas”, International Journal of Surgery (2015), http:// dx.doi.org/10.1016/j.ijsu.2015.04.086

2

G. Benassai et al. / International Journal of Surgery xxx (2015) 1e3

facility between January 2004 and January 2009. There were 2 male 65 and 69 years old respectively and 1 female of 60 years old. All patients presented complaining of repeated episodes of mild epigastric pain, nausea and vomiting. The symptoms had been present for more than 2 years, but had become more frequent in the last few months. There were also lower back pain, dyspnea on exertion. Several investigations were performed to study the case: laboratory tests (including tumor markers), a direct abdomen X-ray with enema, EGDS and total body CT scan in border to better define the diagnosis. The examinations showed, all three cases, narrowing in first part of the duodenum, suggesting a duodenal web, with dilated upper part of the stomach and no mural or intraluminal pathology in duodenum. In all patients EUS was performed. It shows the presence of tissue infiltrating the muscle layer all around the first part of duodenum. Biopsies performed found the presence of pancreatic tissue with focal areas of adenocarcinoma. Subtotal gastrectomy with Roux reconstruction was performed during procedure with sinking of the duodenal stump. We used fibrin glue to seal the pancreatic stump. The histological examinations show an annular pancreas of D1 with multiple focal area of adenocarcinoma (T1aN0M0). 3. Results In all patients no postoperative mortality. One patient has developed a pancreatic fistula high flow (33%), treated with parenteral nutrition and spontaneous closure in 4 weeks. We performed a follow up at 5 years. One patient died after 36 months for cardiovascular hit. Two patients, one male and one female, was 5years disease-free.

chronic pancreatitis due to either the difficulty in the release of the exocrine pancreatic secretion, linked to the anomaly of the excretory tract, or a possible increase in individual susceptibility to this condition. Finally, we also consider jaundice as a possible consequence of the obstructed flow of bile into the duodenum [12,13]. Excluding the changes in humoral parameters that may occur during acute attacks, also for annular pancreas, the diagnosis is made through imaging studies. The barium meal is certainly the gold standard for the diagnosis of this anomaly, being able to highlight three pathognomonic elements:  Homogeneous shrinking of the second duodenal portion  Distension of the proximal duodenum/asymmetry of the duodenal bulb  Reverse duodenal peristalsis CT scan allows to evaluate the behavior of pancreatic tissue around the duodenum, ERCP does lead to the same information coming, however, to study the behavior of the annular duct and excretory tract of the pancreas. Although this survey has a sensitivity of 85%, however it is considered invasive, non-executable during an acute inflammation and also its feasibility is limited by the grade of duodenal obstruction [14,15]. MRCP represents an excellent option for the study of the biliary and pancreatic excretory tract urinary tracts. EUS allows to evaluate the ratio of the annular pancreas with duodenal wall and its can use to get an histological evaluation during the procedure. There remains, however, to remember that only through laparoscopy or laparotomy we can confirm the diagnosis of annular pancreas [16e22]. Ethical approval

4. Discussion The annular pancreas is very rare anatomic anomaly in which a band of pancreatic tissue partially or completely surrounds the first, the second and the third portion of the duodenum. The tissue is generally dissociated from the duodenum, but sometimes it creeps into the deep muscle layer. He derives from an abnormal development of the pancreas as showed by Lecco's theory. If during this process of migration the duodenum is surrounded, it will be partially or completely enveloped by the pancreas, leading to a stenosis of the same [9]. Histologically we can distinct two forms: an intramural, in which pancreatic tissue is interposed to the muscle fibers in the duodenal wall, with small ducts that drain into the duodenum, or an extramural form, where the ventral pancreatic duct surrounds the duodenum and joins the major pancreatic duct. Depending on how the previously mentioned duct opens into the duodenum four distinct types can be faund: In type I the annular duct opens itself normally into Wirsung's duct; in type II the annular duct opens itself into the common bile duct, in type III in the annular duct opens itself into the common bile duct and the duct of Wirsung is missing; in type IV in the annular duct opens itself into Santorini's duct [10,11]. The symptoms of this condition can be quite varied and in some cases, even in the presence of a narrowing of the duodenal lumen, the gastric chyme can usually transit through, in other cases the obstruction is likely to cause nonspecific symptoms. The epigastric pain is a fairly common condition (86%), associated with nausea and vomit. Another fairly common complication is the development of peptic ulcers, especially duodenal (29e48% of cases), probably related to stasis of the chyme and gastric expansion of the segment upstream of the obstruction, condition which may favor the occurrence of hypergastrinemia. An other complication possible is

Ethical approval was requested and obtained from the “Azienda Universitaria Federico II” ethical committee. Author contribution Benassai G.: Participated substantially in conception, design, and execution of the study and in the analysis and interpretation of data; also participated substantially in the drafting and editing of the manuscript. Perrotta S.: Participated substantially in conception, design, and execution of the study and in the analysis and interpretation of data. Furino E.: Participated substantially in conception, design, and execution of the study and in the analysis and interpretation of data. De Werra C.: Participated substantially in conception, design, and execution of the study and in the analysis and interpretation of data. Aloia S.: Participated substantially in conception, design, and execution of the study and in the analysis and interpretation of data. Del Giudice R.: Participated substantially in conception, design, and execution of the study and in the analysis and interpretation of data. Amato B.: Participated substantially in conception, design, and execution of the study and in the analysis and interpretation of data. Vigliotti G.: Participated substantially in conception, design, and execution of the study and in the analysis and interpretation of data. Limite G.: Participated substantially in conception, design, and execution of the study and in the analysis and interpretation of data.

Please cite this article in press as: G. Benassai, et al., “Ductal adenocarcinoma in anular pancreas”, International Journal of Surgery (2015), http:// dx.doi.org/10.1016/j.ijsu.2015.04.086

G. Benassai et al. / International Journal of Surgery xxx (2015) 1e3

Quarto G.: Participated substantially in conception, design, and execution of the study and in the analysis and interpretation of data; also partecipated substantially in the drafting and editing of the manuscript. Conflict of interest The authors have no conflict of interest or any financial support. References [1] M. Maier, W. Wiesner, B. Mengiardi, Annular pancreas and agenesis of the dorsal pancreas in a patient with polysplenia syndrome, AJR Am. J. Roentgenol. 188 (2) (2007 Feb) W150eW153. [2] K. Wagle Prasad, S. Shetty Guruprasad, Mridula Sampat, Kayuri Patel, Ectopic pancreatic tissue mimicking ampullary tumor, Indian J. Gastroenterol. 24 (6) (2005 Nov-Dec) 265e266. [3] Grigorios Christodoulidis, Dimitris Zacharoulis, Sotiris Barbanis, Emmanuel Katsogridakis, Konstantine Hatzitheofilou, Heterotopic pancreas in the stomach: a case report and literature review, World J. Gastroenterol. 13 (45) (2007 December 7) 6098e6100. [4] George H. Sakorafas, Michael G. Sarr, Ectopic gastric submucosal pancreatic tissue, JOP 4 (6) (2003 Nov) 214e215. [5] O.T. Ormarsson, I. Gudmundsdottir, R. Marvik, Diagnosis and treatment of gastric heterotopic pancreas, World J. Surg. 30 (9) (2006 Sep) 1682e1689. [6] L. Zhang, S.O. Sanderson, R.V. Lloyd, T.C. Smyrk, Pancreatic intraepithelial neoplasia in heterotopic pancreas: evidence for the progression model of pancreatic ductal adenocarcinoma, Am. J. Surg. Pathol. 31 (8) (2007 Aug) 1191e1195. [7] F.J. Foo, U. Gill, C.S. Verbeke, J.A. Guthrie, K.V. Menon, Ampullary carcinoma associated with an annular pancreas, JOP. J. Pancreas (Online) 8 (1) (2007 Jan 9) 50e54. [8] K. Sandrasegaran, A. Patel, E.L. Fogel, N.J. Zyromski, H.A. Pitt, Annular pancreas in the adult, AJR Am. J. Roentgenol. 193 (2) (2009 Aug) 455e460. [9] H.M. Zheng, X.J. Cai, L.G. Shen, R. Finley, Surgical treatment of annular pancreas in adults: a case report, Chin. Med. J. Engl. 120 (8) (2007 Apr 20) 724e725. [10] H.M. Zheng, X.J. Cai, L.G. Shen, R. Finley, Surgical treatment of annular pancreas in adults: a report of two cases, Chin. Med. J. 120 (8) (2007) 724e725. [11] De Rai, P.a Zerbi, A.b Castoldi, L.a Bassi, et al., Surgical management of acute pancreatitis in Italy: lessons from a prospective multicentre study, HPB 12 (9)

3

(November 2010) 597e604. [12] G. Benassai, M. Mastrorilli, G. Quarto, A. Cappiello, U. Giani, P. Forestieri, F. Mazzeo, Factors influencing survival after resection for ductal adenocarcinoma of the head of the pancreas, J. Surg. Oncol. 73 (4) (2000) 212e218. [13] L. Cheng, F. Tian, T. Zhao, et al., Annular pancreas concurrent with pancreaticobiliary maljunction presented with symptoms until adult age: case report with comparative data on pediatric cases, BMC Gastroenterol. 13 (2013) 153. [14] N.J. Zyromski, J.A. Sandoval, H.A. Pitt, et al., Annular pancreas: dramatic differences between children and adults, J. Am. Coll. Surg. 206 (5) (2008) 1019e1027. € Yener, Congenital variants and lu, O. [15] A. Türkvatan, A. Erden, M.A. Türkog anomalies of the pancreas and pancreatic duct: imaging by magnetic resonance cholangiopancreaticography and multidetector computed tomography, Korean J. Radiol. 14 (6) (2013) 905e913. [16] M. Zeineb, B.A. Sadri, M. Nizar, et al., Annular pancreas intra operatively discovered: a case report, Clin. Pract. 1 (4) (2011) e82. [17] R. Pezzilli, G. Uomo, A. Gabbrielli, A. Zerbi, L. Frulloni, P. De Rai, et al., A prospective multicentre survey on the treatment of acute pancreatitis in Italy, Dig. Liver Dis. 39 (9) (September 2007) 838e846. [18] G. Benassai, M. Mastrorilli, G. Quarto, A. Cappiello, U. Giani, G. Mosella, Survival after pancreaticoduodenectomy for ductal adenocarcinoma of the head of the pancreas, Chir. Ital. 52 (3) (May 2000) 263e270. [19] L. Castoldi, P. De Rai, A. Zerbi, et al., Long term outcome of acute pancreatitis in Italy: results of a multicentre study, Dig. Liver Dis. 45 (10) (October 2013) 827e832. [20] G. Conzo, L. Circelli, D. Pasquali, A. Sinisi, L. Sabatino, G. Accardo, A. Renzullo, L. Santini, F. Salvatore, V. Colantuoni, Lessons to be learned from the clinical management of a MEN 2A patient bearing a novel 634/640/ 700 triple mutation of the RET proto-oncogene, Clin. Endocrinol. (Oxf) 77 (6) (2012 Dec) 934e936, http://dx.doi.org/10.1111/j.13652265.2012.04412.x. [21] A. Dicitore, M. Caraglia, G. Gaudenzi, G. Manfredi, B. Amato, D. Mari, L. Persani, C. Arra, C. Arra, G. Vitale, Type I interferon-mediated pathway interacts with peroxisome proliferator activated receptor-g (PPAR-g): at the cross-road of pancreatic cancer cell proliferation, Biochim. Biophys. Acta Rev. Cancer 1845 (1) (2014) 42e52, http://dx.doi.org/10.1016/j.bbcan.2013.11.003. [22] G. Conzo, E. Tartaglia, D. Esposito, C. Gambardella, C. Mauriello, M. Mascolo, D. Russo, G. Stornaiuolo, G.B. Gaeta, L. Santini, Suprarenal solitary fibrous tumor associated with a NF 1 gene mutation mimicking a kidney neoplasm. Implications for surgical management, World J. Surg. Oncol. 12 (1) (2014 Apr 7) 87, http://dx.doi.org/10.1186/1477-7819-12-87, 1-5.

Please cite this article in press as: G. Benassai, et al., “Ductal adenocarcinoma in anular pancreas”, International Journal of Surgery (2015), http:// dx.doi.org/10.1016/j.ijsu.2015.04.086

"Ductal adenocarcinoma in anular pancreas".

The annular pancreas is a congenital anomaly in which pancreatic tissue partially or completely surrounds the second portion of the duodenum. Its ofte...
180KB Sizes 0 Downloads 7 Views