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Ectopic pancreas presenting with pancreatitis and a mesenteric mass
Journal of Pediatric Surgery (2013) 48, E29–E32
www.elsevier.com/locate/jpedsurg
Ectopic pancreas presenting with pancreatitis and a mesenteric mass Michael Ginsburg a,⁎, Osman Ahmed a , Kuntal A. Rana b , Redouane Boumendjel c , Abraham H. Dachman a , Mario Zaritzky a a
Department of Radiology, University of Chicago Medical Center, 5841 S. Maryland Avenue, Chicago, IL 60637, USA Department of Radiology, Rush University Medical Center, 1653 W. Congress Pkwy, Jelke Bldg, Suite 181, Chicago, IL 60612 c Department of Pathology, The University of Chicago, 5841 S. Maryland Avenue, Chicago, IL 60637, USA b
Received 12 July 2012; revised 31 October 2012; accepted 31 October 2012
Key words: Ectopic pancreas; Pediatric pancreatitis; Mesenteric mass
Abstract Ectopic pancreas is defined by the presence of abnormally situated pancreatic tissue that lacks contact with normal pancreas and possesses its own duct system and vascular supply. Ectopic pancreas in the gastrointestinal tract is not uncommon. Moreover, there are several reported cases of adult ectopic pancreatitis in the literature, but to date, only two cases of pediatric ectopic pancreatitis have been reported. We describe a 15-year-old female with acute right upper quadrant pain and elevated serum lipase and amylase, in whom the radiological diagnosis was mesenteric soft tissue mass with adjacent inflammatory changes. The surgical pathology diagnosis, however, was mesenteric ectopic pancreas complicated by pancreatitis. We advocate for ectopic pancreatitis to be considered in a pediatric patient with acute abdominal pain, laboratory findings consistent with pancreatitis, and imaging findings of a mesenteric mass and normal orthotopic pancreas. © 2013 Elsevier Inc. All rights reserved.
Ectopic pancreas is defined by the presence of abnormally situated pancreatic tissue that lacks contact with normal pancreas and possesses its own ductal system and vascular supply. It is thought to occur from displacement of endoderm derived pancreatic tissue during embryonic development [1]. Grossly, it presents as a firm, nodular mass. Histologically, it maintains its lobular architecture with acini, islets, and ductules mixed together. When it is located in the bowel, the mass is often submucosal (73%), but it has also been described in the muscular layer (17%) or subserosa (10%) [1,2]. ⁎ Corresponding author. Tel.: + 1 773 702 3550; fax: + 1 773 834 6237. E-mail address: [email protected] (M. Ginsburg). 0022-3468/$ – see front matter © 2013 Elsevier Inc. All rights reserved. http://dx.doi.org/10.1016/j.jpedsurg.2012.10.062
The location of ectopic pancreas is variable, with the most common locations being the duodenum (28% of cases), stomach (26%), and jejunum (16%) [3,4] and rarely in the mediastinum, esophagus, jejunum, ileum, Meckel diverticulum, liver, biliary tract, and the fallopian tubes [1–4]. It is the most common type of heterotopic tissue within the gastrointestinal system, with a reported prevalence of 2%– 14% in autopsy series [1,3,5]. The entity is usually asymptomatic, with most of the lesions detected incidentally at surgery or autopsy and most of the complications manifesting in adulthood [3–5]. Ectopic pancreatitis is an uncommon complication described histologically but only rarely diagnosed clinically or radiologically, with several cases reported in adult population [4–9]. Only two cases of
E30
M. Ginsburg et al.
1. Case presentation
Fig. 1 Axial contrast enhanced CT image shows a lobulated mesenteric soft tissue mass (white arrowhead) with morphology and homogeneous enhancement characteristics similar to those of the pancreas. The jejunal veins rotate in a clockwise fashion around the superior mesenteric artery in relation to the superior mesenteric vein (black arrowhead).
pediatric ectopic pancreatitis have been described in the literature [10,11]. This report describes a case of ectopic pancreas in a teenage female with acute abdominal pain, elevated serum amylase/lipase, and a contrast enhanced CT showing a mesenteric mass with surrounding inflammatory changes.
Fig. 2 Coronal contrast enhanced CT image shows a right upper quadrant round lobulated mesenteric soft tissue mass (white arrowhead) and surrounding inflammatory fat stranding. Displaced jejunal loops and veins to the right are noted (black arrowhead).
A 15 year-old female with no significant past medical history presented to the Emergency Room with a 24 h history of worsening right upper quadrant pain with occasional radiation to the right lower quadrant. Physical examination showed diffuse abdominal tenderness, most pronounced in the right upper quadrant and nonspecific guarding. Laboratory evaluation demonstrated elevated serum lipase (983 U/L; reference range, 11–65 U/L) and amylase (204 U/L; reference range, 28–100 U/L) levels and normal range liver function tests. A CT examination of the abdomen and pelvis with oral and intravenous contrast administration was performed to evaluate for possible causes for and complications presumed pancreatitis. The CT scan demonstrated a normal pancreas with no changes of acute pancreatitis and 3.3 × 2.3 cm soft tissue mass in the small bowel mesentery with adjacent inflammatory changes (Figs. 1 and 2). Additionally, dilated jejunal loops and their accompanying vessels were displaced to the right upper quadrant (Figs. 1 and 2). The transverse duodenum was normally positioned between the superior mesenteric artery and the aorta with the ligament of Treitz in a normal location, ruling out a proximal midgut malrotation as a source of the patient's pain. At the time, the CT scan was interpreted as a probable right paraduodenal hernia. Subsequently, the patient was taken to the operating room for a diagnostic laparoscopy. During the operation, a 3 cm mass was found in the proximal small bowel mesentery. The mass was adjacent to the transverse colon and omentum and appeared grossly inflamed. The wound was converted to an open procedure through a small midline incision to complete the resection. The mass and the adjacent small bowel were resected and a primary small bowel anastomosis was performed. The pathological diagnosis was small bowel segment with intramural and mesenteric ectopic pancreas, acute pancreatitis, and fat necrosis.
Fig. 3 Photograph of the specimen demonstrates 4.5 cm in long axis ectopic pancreas (P) adhered to the serosal surface of the jejunum with edematous mucosa and submucosa overlying it (white arrows). The normal surface of jejunal folds (J).
Ectopic pancreas with pancreatitis and a mesenteric mass
Fig. 4 Intact jejunal villi and mucosa (black arrowhead) with underlying ectopic pancreatic tissue (black arrow), including acini, islet cells and pancreatic ducts extending to the mucosal surface. (Hematoxylin and eosin: magnification, × 10).
2. Pathology findings Gross pathology demonstrated a 4.5 × 3.1 × 3.6 cm mass in the mesentery adhered to the serosal surface of the jejunum with edematous mucosa and submucosa overlying the mass (Fig. 3). Histological examination showed intact jejunal mucosa with normal overlying jejunal villi and underlying ectopic pancreatic tissue, including acini and islet cells, extending from the submucosa to the subserosa with a pancreatic duct like structure extending to the jejunal mucosa (Fig. 4). In the subserosal areas, fat necrosis and acute inflammation were observed that were consistent with ectopic pancreatitis.
3. Discussion Ectopic pancreas is a relatively frequent congenital anomaly with a reported incidence ranging from 2% to 14% that rarely causes clinical symptoms. While it is usually discovered as an incidental finding on imaging, endoscopy, surgery or autopsy, it can present with various clinical complications and cause both diagnostic and management dilemmas. Complications including pancreatitis, pseudocyst, gastrointestinal bleeding, small bowel obstruction, and transformation to pancreatic adenocarcinoma have been reported [1–12]. In our case, the clinical presentation of acute abdomen in a context of a radiological diagnosis of mesenteric mass and probable para-duodenal hernia prompted emergent surgical intervention. The subsequent pathology showed that the mesenteric mass was composed of ectopic pancreatic tissue that was histologically indistinguishable from orthotopic pancreas, as well as adjacent inflammation consistent with ectopic pancreatitis.
E31 In retrospect, the homogeneous morphology and enhancement of the mesenteric mass were similar to those of the orthotopic normal appearing pancreas. Computed tomography findings of ectopic pancreas are usually nonspecific; CT with intravenous contrast may demonstrate the lesion which enhances similarly to normal pancreatic tissue [5,13,14]. However, CT cannot distinguish ectopic pancreas from other conditions such as a gastrointestinal stromal tumor, desmoid, lymphoma, carcinoid tumor or metastases. It is the constellation of both clinical and radiological findings that might suggest the possibility of ectopic pancreas. Although, accurate preoperative diagnosis of heterotopic pancreas remains difficult, recent reports suggest that the visualization of the ectopic pancreatic duct with MRCP may provide a definitive diagnosis, permitting initial conservative management and precluding the need for an emergent operation [3,6,7]. However, due to potential life threatening complications and malignant transformation, local excision of this congenital anomaly is the appropriate treatment [1,15]. Ectopic pancreas should be considered in the differential diagnosis of a mesenteric mass, especially when its morphology and enhancement are similar to those of orthotopic pancreas. In a pediatric patient with acute abdominal pain and elevated serum amylase/lipase, the imaging findings of a normal orthotopic pancreas should prompt the consideration of ectopic pancreatitis.
Acknowledgments We express our gratitude to Dr. Kate Feinstein for aiding in the revision of this manuscript.
References [1] Lai EC, Tompkins RK. Heterotopic pancreas: review of a 26 year experience. Am J Surg 1986;151:697-700. [2] Kaneda M, Yano T, Yamamoto T, et al. Ectopic pancreas in the stomach presenting as an inflammatory abdominal mass. Am J Gastroenterol 1989;84:663-6. [3] Shanbhogue AK, Fasih N, Surabhi VR, Doherty, et al. A clinical and radiologic review of uncommon types and causes of pancreatitis. Radiographics 2009;29(4):1003-26. [4] Rubesin SE, Furth EE, Birnbaum BA, et al. Ectopic pancreas complicated by pancreatitis and pseudocyst formation mimicking jejunal diverticulitis. Br J Radiol 1997;70:311-3. [5] Cho JS, Shin KS, Kwon ST, et al. Heterotopic pancreas in the stomach: CT findings. Radiology 2000;217:139-44. [6] Wong JC, Robinson C, Jones EC, et al. Recurrent ectopic pancreatitis of the jejunum and mesentery over a 30-year period. Hepatobiliary Pancreat Dis Int 2011;10:218-20. [7] Silva AC, Charles JC, Kimery BD, et al. MR cholangiopancreatography in the detection of symptomatic ectopic pancreatitis in the small-bowel mesentery. AJR Am J Roentgenol 2006;187:W195-7. [8] Henry C, Prot T, Maitre S, et al. A rare cause of acute abdominal pain: mesenteric heterotopic pancreas complicated by acute pancreatitis. Gastroenterol Clin Biol 2000;24:370-1. [9] Canbaz H, Colak T, Düsmez Apa D, et al. An unusual cause of acute abdomen: mesenteric heterotopic pancreatitis causing confusion in clinical diagnosis. Turk J Gastroenterol 2009;20:142-5.
E32 [10] Curran JP, Anderson V. Pancreatic heterotopia as a cause of an acute abdomen. Clin Pediatr (Phila) 1986;25:466-8. [11] Fam S, O'Briain DS, Borger JA. Ectopic pancreas with acute inflammation. J Pediatr Surg 1982;17:86-7. [12] Jeong HY, Yang HW, Seo SW, et al. Adenocarcinoma arising from an ectopic pancreas in the stomach. Endoscopy 2002;34: 1014-7.
M. Ginsburg et al. [13] Megibow AJ, Balthazar EJ, Cho KC, Medwid SW, Bimbaum BA, Noz ME. Bowel obstruction: evaluation with CT. Radiology 1991;180:313-8. [14] Wang C, Kuo Y, Yeung K, et al. CT appearance of ectopic pancreas: a case report. Abdom Imaging 1998;23:332-3. [15] Biswas A, Husain EA, Feakins RM, et al. Heterotopic pancreas mimicking cholangiocarcinoma. Case report and literature review. JOP J Pancreas 2007;8:28-34.
www.elsevier.com/locate/jpedsurg
Ectopic pancreas presenting with pancreatitis and a mesenteric mass Michael Ginsburg a,⁎, Osman Ahmed a , Kuntal A. Rana b , Redouane Boumendjel c , Abraham H. Dachman a , Mario Zaritzky a a
Department of Radiology, University of Chicago Medical Center, 5841 S. Maryland Avenue, Chicago, IL 60637, USA Department of Radiology, Rush University Medical Center, 1653 W. Congress Pkwy, Jelke Bldg, Suite 181, Chicago, IL 60612 c Department of Pathology, The University of Chicago, 5841 S. Maryland Avenue, Chicago, IL 60637, USA b
Received 12 July 2012; revised 31 October 2012; accepted 31 October 2012
Key words: Ectopic pancreas; Pediatric pancreatitis; Mesenteric mass
Abstract Ectopic pancreas is defined by the presence of abnormally situated pancreatic tissue that lacks contact with normal pancreas and possesses its own duct system and vascular supply. Ectopic pancreas in the gastrointestinal tract is not uncommon. Moreover, there are several reported cases of adult ectopic pancreatitis in the literature, but to date, only two cases of pediatric ectopic pancreatitis have been reported. We describe a 15-year-old female with acute right upper quadrant pain and elevated serum lipase and amylase, in whom the radiological diagnosis was mesenteric soft tissue mass with adjacent inflammatory changes. The surgical pathology diagnosis, however, was mesenteric ectopic pancreas complicated by pancreatitis. We advocate for ectopic pancreatitis to be considered in a pediatric patient with acute abdominal pain, laboratory findings consistent with pancreatitis, and imaging findings of a mesenteric mass and normal orthotopic pancreas. © 2013 Elsevier Inc. All rights reserved.
Ectopic pancreas is defined by the presence of abnormally situated pancreatic tissue that lacks contact with normal pancreas and possesses its own ductal system and vascular supply. It is thought to occur from displacement of endoderm derived pancreatic tissue during embryonic development [1]. Grossly, it presents as a firm, nodular mass. Histologically, it maintains its lobular architecture with acini, islets, and ductules mixed together. When it is located in the bowel, the mass is often submucosal (73%), but it has also been described in the muscular layer (17%) or subserosa (10%) [1,2]. ⁎ Corresponding author. Tel.: + 1 773 702 3550; fax: + 1 773 834 6237. E-mail address: [email protected] (M. Ginsburg). 0022-3468/$ – see front matter © 2013 Elsevier Inc. All rights reserved. http://dx.doi.org/10.1016/j.jpedsurg.2012.10.062
The location of ectopic pancreas is variable, with the most common locations being the duodenum (28% of cases), stomach (26%), and jejunum (16%) [3,4] and rarely in the mediastinum, esophagus, jejunum, ileum, Meckel diverticulum, liver, biliary tract, and the fallopian tubes [1–4]. It is the most common type of heterotopic tissue within the gastrointestinal system, with a reported prevalence of 2%– 14% in autopsy series [1,3,5]. The entity is usually asymptomatic, with most of the lesions detected incidentally at surgery or autopsy and most of the complications manifesting in adulthood [3–5]. Ectopic pancreatitis is an uncommon complication described histologically but only rarely diagnosed clinically or radiologically, with several cases reported in adult population [4–9]. Only two cases of
E30
M. Ginsburg et al.
1. Case presentation
Fig. 1 Axial contrast enhanced CT image shows a lobulated mesenteric soft tissue mass (white arrowhead) with morphology and homogeneous enhancement characteristics similar to those of the pancreas. The jejunal veins rotate in a clockwise fashion around the superior mesenteric artery in relation to the superior mesenteric vein (black arrowhead).
pediatric ectopic pancreatitis have been described in the literature [10,11]. This report describes a case of ectopic pancreas in a teenage female with acute abdominal pain, elevated serum amylase/lipase, and a contrast enhanced CT showing a mesenteric mass with surrounding inflammatory changes.
Fig. 2 Coronal contrast enhanced CT image shows a right upper quadrant round lobulated mesenteric soft tissue mass (white arrowhead) and surrounding inflammatory fat stranding. Displaced jejunal loops and veins to the right are noted (black arrowhead).
A 15 year-old female with no significant past medical history presented to the Emergency Room with a 24 h history of worsening right upper quadrant pain with occasional radiation to the right lower quadrant. Physical examination showed diffuse abdominal tenderness, most pronounced in the right upper quadrant and nonspecific guarding. Laboratory evaluation demonstrated elevated serum lipase (983 U/L; reference range, 11–65 U/L) and amylase (204 U/L; reference range, 28–100 U/L) levels and normal range liver function tests. A CT examination of the abdomen and pelvis with oral and intravenous contrast administration was performed to evaluate for possible causes for and complications presumed pancreatitis. The CT scan demonstrated a normal pancreas with no changes of acute pancreatitis and 3.3 × 2.3 cm soft tissue mass in the small bowel mesentery with adjacent inflammatory changes (Figs. 1 and 2). Additionally, dilated jejunal loops and their accompanying vessels were displaced to the right upper quadrant (Figs. 1 and 2). The transverse duodenum was normally positioned between the superior mesenteric artery and the aorta with the ligament of Treitz in a normal location, ruling out a proximal midgut malrotation as a source of the patient's pain. At the time, the CT scan was interpreted as a probable right paraduodenal hernia. Subsequently, the patient was taken to the operating room for a diagnostic laparoscopy. During the operation, a 3 cm mass was found in the proximal small bowel mesentery. The mass was adjacent to the transverse colon and omentum and appeared grossly inflamed. The wound was converted to an open procedure through a small midline incision to complete the resection. The mass and the adjacent small bowel were resected and a primary small bowel anastomosis was performed. The pathological diagnosis was small bowel segment with intramural and mesenteric ectopic pancreas, acute pancreatitis, and fat necrosis.
Fig. 3 Photograph of the specimen demonstrates 4.5 cm in long axis ectopic pancreas (P) adhered to the serosal surface of the jejunum with edematous mucosa and submucosa overlying it (white arrows). The normal surface of jejunal folds (J).
Ectopic pancreas with pancreatitis and a mesenteric mass
Fig. 4 Intact jejunal villi and mucosa (black arrowhead) with underlying ectopic pancreatic tissue (black arrow), including acini, islet cells and pancreatic ducts extending to the mucosal surface. (Hematoxylin and eosin: magnification, × 10).
2. Pathology findings Gross pathology demonstrated a 4.5 × 3.1 × 3.6 cm mass in the mesentery adhered to the serosal surface of the jejunum with edematous mucosa and submucosa overlying the mass (Fig. 3). Histological examination showed intact jejunal mucosa with normal overlying jejunal villi and underlying ectopic pancreatic tissue, including acini and islet cells, extending from the submucosa to the subserosa with a pancreatic duct like structure extending to the jejunal mucosa (Fig. 4). In the subserosal areas, fat necrosis and acute inflammation were observed that were consistent with ectopic pancreatitis.
3. Discussion Ectopic pancreas is a relatively frequent congenital anomaly with a reported incidence ranging from 2% to 14% that rarely causes clinical symptoms. While it is usually discovered as an incidental finding on imaging, endoscopy, surgery or autopsy, it can present with various clinical complications and cause both diagnostic and management dilemmas. Complications including pancreatitis, pseudocyst, gastrointestinal bleeding, small bowel obstruction, and transformation to pancreatic adenocarcinoma have been reported [1–12]. In our case, the clinical presentation of acute abdomen in a context of a radiological diagnosis of mesenteric mass and probable para-duodenal hernia prompted emergent surgical intervention. The subsequent pathology showed that the mesenteric mass was composed of ectopic pancreatic tissue that was histologically indistinguishable from orthotopic pancreas, as well as adjacent inflammation consistent with ectopic pancreatitis.
E31 In retrospect, the homogeneous morphology and enhancement of the mesenteric mass were similar to those of the orthotopic normal appearing pancreas. Computed tomography findings of ectopic pancreas are usually nonspecific; CT with intravenous contrast may demonstrate the lesion which enhances similarly to normal pancreatic tissue [5,13,14]. However, CT cannot distinguish ectopic pancreas from other conditions such as a gastrointestinal stromal tumor, desmoid, lymphoma, carcinoid tumor or metastases. It is the constellation of both clinical and radiological findings that might suggest the possibility of ectopic pancreas. Although, accurate preoperative diagnosis of heterotopic pancreas remains difficult, recent reports suggest that the visualization of the ectopic pancreatic duct with MRCP may provide a definitive diagnosis, permitting initial conservative management and precluding the need for an emergent operation [3,6,7]. However, due to potential life threatening complications and malignant transformation, local excision of this congenital anomaly is the appropriate treatment [1,15]. Ectopic pancreas should be considered in the differential diagnosis of a mesenteric mass, especially when its morphology and enhancement are similar to those of orthotopic pancreas. In a pediatric patient with acute abdominal pain and elevated serum amylase/lipase, the imaging findings of a normal orthotopic pancreas should prompt the consideration of ectopic pancreatitis.
Acknowledgments We express our gratitude to Dr. Kate Feinstein for aiding in the revision of this manuscript.
References [1] Lai EC, Tompkins RK. Heterotopic pancreas: review of a 26 year experience. Am J Surg 1986;151:697-700. [2] Kaneda M, Yano T, Yamamoto T, et al. Ectopic pancreas in the stomach presenting as an inflammatory abdominal mass. Am J Gastroenterol 1989;84:663-6. [3] Shanbhogue AK, Fasih N, Surabhi VR, Doherty, et al. A clinical and radiologic review of uncommon types and causes of pancreatitis. Radiographics 2009;29(4):1003-26. [4] Rubesin SE, Furth EE, Birnbaum BA, et al. Ectopic pancreas complicated by pancreatitis and pseudocyst formation mimicking jejunal diverticulitis. Br J Radiol 1997;70:311-3. [5] Cho JS, Shin KS, Kwon ST, et al. Heterotopic pancreas in the stomach: CT findings. Radiology 2000;217:139-44. [6] Wong JC, Robinson C, Jones EC, et al. Recurrent ectopic pancreatitis of the jejunum and mesentery over a 30-year period. Hepatobiliary Pancreat Dis Int 2011;10:218-20. [7] Silva AC, Charles JC, Kimery BD, et al. MR cholangiopancreatography in the detection of symptomatic ectopic pancreatitis in the small-bowel mesentery. AJR Am J Roentgenol 2006;187:W195-7. [8] Henry C, Prot T, Maitre S, et al. A rare cause of acute abdominal pain: mesenteric heterotopic pancreas complicated by acute pancreatitis. Gastroenterol Clin Biol 2000;24:370-1. [9] Canbaz H, Colak T, Düsmez Apa D, et al. An unusual cause of acute abdomen: mesenteric heterotopic pancreatitis causing confusion in clinical diagnosis. Turk J Gastroenterol 2009;20:142-5.
E32 [10] Curran JP, Anderson V. Pancreatic heterotopia as a cause of an acute abdomen. Clin Pediatr (Phila) 1986;25:466-8. [11] Fam S, O'Briain DS, Borger JA. Ectopic pancreas with acute inflammation. J Pediatr Surg 1982;17:86-7. [12] Jeong HY, Yang HW, Seo SW, et al. Adenocarcinoma arising from an ectopic pancreas in the stomach. Endoscopy 2002;34: 1014-7.
M. Ginsburg et al. [13] Megibow AJ, Balthazar EJ, Cho KC, Medwid SW, Bimbaum BA, Noz ME. Bowel obstruction: evaluation with CT. Radiology 1991;180:313-8. [14] Wang C, Kuo Y, Yeung K, et al. CT appearance of ectopic pancreas: a case report. Abdom Imaging 1998;23:332-3. [15] Biswas A, Husain EA, Feakins RM, et al. Heterotopic pancreas mimicking cholangiocarcinoma. Case report and literature review. JOP J Pancreas 2007;8:28-34.