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Gastric-duplication cyst with an aberrant pancreatic-ductal system: an unusual cause of recurrent abdominal pain
Brief Reports
included in the differential diagnosis of patients who are seen with unusual cholangiographic findings and cholestasis. DISCLOSURE All authors disclosed no financial relationships relevant to this publication. Abbreviations: CBD, common bile duct; LFT, liver function test; MPA, microscopic polyarteritis; p-ANCA, perinuclear antineutrophil cytoplasm antibodies; PAN, polyarteritis nodosa.
REFERENCES 1. Batts KP. Ischemic cholangitis. Mayo Clin Proc 1998;73:380-5. 2. Barquist ES, Goldstein N, Zinner MJ. Polyarteritis nodosa presenting as a biliary stricture. Surgery 1991;109:16-9.
3. Kasper HU, Dries V, Drebber U, et al. Florid ischemic cholangitis due to leucocytoclastic vasculitis. J Gastroenterol 2004;39:188-91. 4. Viola S, Meyer M, Fabre M, et al. Ischemic necrosis of bile ducts complicating Scho¨nlein-Henoch purpura. Gastroenterology 1999;117:211-4. 5. Jaeger C, Mayer G, Henrich R, et al. Secondary sclerosing cholangitis after long-term treatment in an intensive care unit: clinical presentation, endoscopic findings, treatment, and follow-up. Endoscopy 2006; 38:730-4.
Current affiliations: Department of Gastroenterology and Hepatology, Westmead Hospital, Sydney, Australia. Reprint requests: Michael Bourke, MBBS, Department of Gastroenterology and Hepatology, Westmead Hospital, Sydney West Area Health Service, Sydney, NSW 2145 Australia. Copyright ª 2009 by the American Society for Gastrointestinal Endoscopy 0016-5107/$36.00 doi:10.1016/j.gie.2008.03.1125
Gastric-duplication cyst with an aberrant pancreatic-ductal system: an unusual cause of recurrent abdominal pain Trupti Shinde, MD, Jennifer Lindner, DO, Jan Silverman, MD, Rad Agrawal, MD, Manish Dhawan, MD Pittsburgh, Pennsylvania, USA
Gastric duplication cysts (GDCs) are very rare in adults. Reported here is a case of a GDC communicating with an accessory pancreas via an aberrant duct in a 49-year-old woman with recurrent abdominal pain.
CASE REPORT A 49-year-old woman was seen at an outside hospital with epigastric pain. An EGD revealed an antral mass. On a CTof the abdomen, a cystic lesion of the antrum was seen. She was referred to our institution. Another EGD revealed an antral submucosal mass with a central umbilication (Fig. 1). EUS revealed a 37-mm 26-mm submucosal cyst suggestive of GDC (Fig. 2). However, results from the aspiration of the cyst revealed a high amylase level. Her symptoms subsided but recurred 4 months later. EUS confirmed cyst reaccumulation, and aspiration again showed a high amylase level. No malignant cells were seen. After aspiration, her symptoms improved again. Because of her recurrent symptoms, an endoscopic retrograde pancreatography (ERP) was performed, which revealed a second pancreatic-duct system that originated from the main pancreatic duct near the tail of the pancreas (Fig. 3). This second system extended parallel to the main pancreatic duct before attaching to the antral cyst. The patient went for surgery, and the accessory pancreas, aberrant pancreatic duct,
and cystic portion of the antrum were resected. The surgical specimen showed a 5-cm round structure identified as a cyst, which was attached to a 17-cm-long accessory pancreas with the aberrant ductal system (Fig. 4). The cyst was unilocular, with smooth lining. On microscopic examination, the cyst wall was lined by gastric mucosa with focal
www.giejournal.org
Volume 69, No. 2 : 2009 GASTROINTESTINAL ENDOSCOPY 377
Figure 1. An upper endoscopy image, showing an antral submucosal mass with a central umbilication.
Brief Reports
Figure 4. Surgical specimen, showing stomach, duplication cyst, and an aberrant pancreas.
Figure 2. EUS, revealing a cyst opening in the antrum of the stomach.
Figure 5. Photomicrograph of cyst wall, demonstrating 4 layers: mucosa, muscularis mucosae, submucosa, and muscularis propria (H&E, orig. mag. 40).
Gastric duplication cysts make up only 3.8% of all duplications of the alimentary tract.1 Furthermore, GDC with an
aberrant pancreatic duct and accessory pancreas is extremely rare.1 Abnormal foregut development is believed to be responsible for this congenital anomaly.2 Several theories are proposed but two of them, Bremer’s theory of errors on recanalization and McLetchie’s theory, are most widely accepted.1 McLetchie proposed a neuroenteric hypothesis in which an embryonic entoectodermal adhesion gives rise to a ‘‘neuroenteric’’ band, which may form traction diverticula and lead to gut cyst formation.1,2 This theory explains the congenital anomaly in our patient, with the traction on the pancreatic bud by a neuroenteric band causing both gastric and pancreatic abnormalities. There are specific characteristics to define a gastric duplication cyst. The cyst wall should be contiguous with the
378 GASTROINTESTINAL ENDOSCOPY Volume 69, No. 2 : 2009
www.giejournal.org
Figure 3. ERP image, revealing an aberrant pancreatic-duct system originating from the main pancreatic duct near the tail of the pancreas, extending parallel to the main pancreatic duct, and attaching to the antral cyst. Main pancreatic duct (a), aberrant pancreatic duct (b), antral cyst (c).
ulceration and a well-circumscribed smooth-muscle layer (Fig. 5). The patient’s symptoms resolved completely after surgical resection.
DISCUSSION
Brief Reports
stomach. It should be surrounded by a coat of shared smooth muscle. A common blood supply should be present. It should be lined by alimentary-tract epithelium. Rarely, respiratory-tract epithelium, in conjunction with alimentary-tract epithelium, has been noted.3,4 In adults, diagnosis is very difficult before surgery. Gastric duplication cysts are usually asymptomatic.5,6 However, signs and symptoms may include epigastric pain, abdominal mass, or vomiting. Rarely, GI bleeding, pancreatitis, peritonitis, malignancy, or even acute abdomen may result.6-8 Often, a GDC may be mistaken as a pancreatic pseudocyst, which is more common.8 Normal levels of pancreatic enzymes and no personal history of pancreatitis may suggest a gastric duplication cyst. Unlike GDC, the pancreatic pseudocyst is generally associated with repeated attacks of pancreatitis. They lack an epithelial lining and contain amylase-rich fluid.8 US, CT, and EUS may help in the diagnosis. In our case, the diagnosis was made before surgery by EUS-guided cyst aspiration and ERP. Surgical resection led to a satisfactory outcome. This case provides a fascinating opportunity to explore embryogenesis of the alimentary tract, as well as highlighting the usefulness of EUS and ERP in preoperative diagnosis. DISCLOSURE All authors disclosed no financial relationships relevant to this publication. Abbreviations: ERP, endoscopic retrograde pancreatography; GDCs, gastric duplication cysts.
REFERENCES 1. Muraoka A, Tsuruno M, Katsuno G, et al. A gastric duplication cyst with an aberrant pancreatic ductal system: report of a case. Surg Today 2002;32:531-5. 2. Camoglio FS, Forestieri C, Zanatta C, et al. Complete pancreatic ectopia in a gastric duplication cyst: a case report and review of the literature. Eur J Pediatr Surg 2004;14:60-2. 3. O’Donnell PL, Morrow JB, Fitzgerald TL. Adult gastric duplication cysts: a case report and review of literature. Am Surg 2005;71:522-5. 4. Takahara T, Torigoe T, Haga H, et al. Gastric duplication cyst: evaluation by endoscopic ultrasonography and magnetic resonance imaging. J Gastroenterol 1996;31:420-4. 5. Johnstone DW, Forde KA, Markowitz D, et al. Gastric duplication cyst communicating with the pancreatic duct: a rare cause of recurrent abdominal pain. Surgery 1991;109:97-100. 6. Coit DG, Mies C. Adenocarcinoma arising within a gastric duplication cyst. J Surg Oncol 1992;50:274-7. 7. Horne G, Ming-Lum C, Kirkpatrick AW, et al. High-grade neuroendocrine carcinoma arising in a gastric duplication cyst: a case report with literature review. Int J Surg Pathol 2007;15:187-91. 8. Yang MC, Duh YC, Lai H, et al. Alimentary tract duplications. J Formos Med Assoc 1996;95:406-9.
Current affiliations: Departments of Medicine (T.S.), Pathology (J.L., J.S.), Gastroenterology (R.A. M.D.), Allegheny General Hospital, Pittsburgh, Pennsylvania, USA. Reprint requests: Trupti Shinde, MD, Internal Medicine, Allegheny General Hospital, Medicine Department, 320 E. North Ave, Pittsburgh, PA 15212. Copyright ª 2009 by the American Society for Gastrointestinal Endoscopy 0016-5107/$36.00 doi:10.1016/j.gie.2008.04.059
Pancreatic somatostatinoma and tuberous sclerosis: case report of an exceedingly rare association Jayaprakash Sreenarasimhaiah, MD, Luis A. Armstrong, MD, Shou-Jiang Tang, MD, Carlton Barnett, MD Dallas, Texas, USA
Tuberous sclerosis is an autosomal dominant condition characterized by the triad of mental deficiency, epilepsy, and the classic skin eruption known as adenoma sebaceum.1 Other findings include visceral lesions with histology similar to the skin lesions, renal angiomyolipomas, and pancreatic abnormalities. Findings in the pancreas can include pancreatic cysts, rare angiomyolipomas, and neuroendocrine tumors of the nonfunctioning islet cell variety.2 Results of certain reports suggest that functional and nonfunctional insulinomas may be associated with this disorder. To our knowledge, this is the first reported
A 24-year-old man with a history of tuberous sclerosis complained of 3 months of dull abdominal pain. He denied symptoms of GI bleeding, jaundice, nausea, or vomiting, or association with food intake. He reported a mild weight loss of 10 pounds during this period. His history also included
www.giejournal.org
Volume 69, No. 2 : 2009 GASTROINTESTINAL ENDOSCOPY 379
case of pancreatic somatostatinoma associated with tuberous sclerosis.
CASE REPORT
included in the differential diagnosis of patients who are seen with unusual cholangiographic findings and cholestasis. DISCLOSURE All authors disclosed no financial relationships relevant to this publication. Abbreviations: CBD, common bile duct; LFT, liver function test; MPA, microscopic polyarteritis; p-ANCA, perinuclear antineutrophil cytoplasm antibodies; PAN, polyarteritis nodosa.
REFERENCES 1. Batts KP. Ischemic cholangitis. Mayo Clin Proc 1998;73:380-5. 2. Barquist ES, Goldstein N, Zinner MJ. Polyarteritis nodosa presenting as a biliary stricture. Surgery 1991;109:16-9.
3. Kasper HU, Dries V, Drebber U, et al. Florid ischemic cholangitis due to leucocytoclastic vasculitis. J Gastroenterol 2004;39:188-91. 4. Viola S, Meyer M, Fabre M, et al. Ischemic necrosis of bile ducts complicating Scho¨nlein-Henoch purpura. Gastroenterology 1999;117:211-4. 5. Jaeger C, Mayer G, Henrich R, et al. Secondary sclerosing cholangitis after long-term treatment in an intensive care unit: clinical presentation, endoscopic findings, treatment, and follow-up. Endoscopy 2006; 38:730-4.
Current affiliations: Department of Gastroenterology and Hepatology, Westmead Hospital, Sydney, Australia. Reprint requests: Michael Bourke, MBBS, Department of Gastroenterology and Hepatology, Westmead Hospital, Sydney West Area Health Service, Sydney, NSW 2145 Australia. Copyright ª 2009 by the American Society for Gastrointestinal Endoscopy 0016-5107/$36.00 doi:10.1016/j.gie.2008.03.1125
Gastric-duplication cyst with an aberrant pancreatic-ductal system: an unusual cause of recurrent abdominal pain Trupti Shinde, MD, Jennifer Lindner, DO, Jan Silverman, MD, Rad Agrawal, MD, Manish Dhawan, MD Pittsburgh, Pennsylvania, USA
Gastric duplication cysts (GDCs) are very rare in adults. Reported here is a case of a GDC communicating with an accessory pancreas via an aberrant duct in a 49-year-old woman with recurrent abdominal pain.
CASE REPORT A 49-year-old woman was seen at an outside hospital with epigastric pain. An EGD revealed an antral mass. On a CTof the abdomen, a cystic lesion of the antrum was seen. She was referred to our institution. Another EGD revealed an antral submucosal mass with a central umbilication (Fig. 1). EUS revealed a 37-mm 26-mm submucosal cyst suggestive of GDC (Fig. 2). However, results from the aspiration of the cyst revealed a high amylase level. Her symptoms subsided but recurred 4 months later. EUS confirmed cyst reaccumulation, and aspiration again showed a high amylase level. No malignant cells were seen. After aspiration, her symptoms improved again. Because of her recurrent symptoms, an endoscopic retrograde pancreatography (ERP) was performed, which revealed a second pancreatic-duct system that originated from the main pancreatic duct near the tail of the pancreas (Fig. 3). This second system extended parallel to the main pancreatic duct before attaching to the antral cyst. The patient went for surgery, and the accessory pancreas, aberrant pancreatic duct,
and cystic portion of the antrum were resected. The surgical specimen showed a 5-cm round structure identified as a cyst, which was attached to a 17-cm-long accessory pancreas with the aberrant ductal system (Fig. 4). The cyst was unilocular, with smooth lining. On microscopic examination, the cyst wall was lined by gastric mucosa with focal
www.giejournal.org
Volume 69, No. 2 : 2009 GASTROINTESTINAL ENDOSCOPY 377
Figure 1. An upper endoscopy image, showing an antral submucosal mass with a central umbilication.
Brief Reports
Figure 4. Surgical specimen, showing stomach, duplication cyst, and an aberrant pancreas.
Figure 2. EUS, revealing a cyst opening in the antrum of the stomach.
Figure 5. Photomicrograph of cyst wall, demonstrating 4 layers: mucosa, muscularis mucosae, submucosa, and muscularis propria (H&E, orig. mag. 40).
Gastric duplication cysts make up only 3.8% of all duplications of the alimentary tract.1 Furthermore, GDC with an
aberrant pancreatic duct and accessory pancreas is extremely rare.1 Abnormal foregut development is believed to be responsible for this congenital anomaly.2 Several theories are proposed but two of them, Bremer’s theory of errors on recanalization and McLetchie’s theory, are most widely accepted.1 McLetchie proposed a neuroenteric hypothesis in which an embryonic entoectodermal adhesion gives rise to a ‘‘neuroenteric’’ band, which may form traction diverticula and lead to gut cyst formation.1,2 This theory explains the congenital anomaly in our patient, with the traction on the pancreatic bud by a neuroenteric band causing both gastric and pancreatic abnormalities. There are specific characteristics to define a gastric duplication cyst. The cyst wall should be contiguous with the
378 GASTROINTESTINAL ENDOSCOPY Volume 69, No. 2 : 2009
www.giejournal.org
Figure 3. ERP image, revealing an aberrant pancreatic-duct system originating from the main pancreatic duct near the tail of the pancreas, extending parallel to the main pancreatic duct, and attaching to the antral cyst. Main pancreatic duct (a), aberrant pancreatic duct (b), antral cyst (c).
ulceration and a well-circumscribed smooth-muscle layer (Fig. 5). The patient’s symptoms resolved completely after surgical resection.
DISCUSSION
Brief Reports
stomach. It should be surrounded by a coat of shared smooth muscle. A common blood supply should be present. It should be lined by alimentary-tract epithelium. Rarely, respiratory-tract epithelium, in conjunction with alimentary-tract epithelium, has been noted.3,4 In adults, diagnosis is very difficult before surgery. Gastric duplication cysts are usually asymptomatic.5,6 However, signs and symptoms may include epigastric pain, abdominal mass, or vomiting. Rarely, GI bleeding, pancreatitis, peritonitis, malignancy, or even acute abdomen may result.6-8 Often, a GDC may be mistaken as a pancreatic pseudocyst, which is more common.8 Normal levels of pancreatic enzymes and no personal history of pancreatitis may suggest a gastric duplication cyst. Unlike GDC, the pancreatic pseudocyst is generally associated with repeated attacks of pancreatitis. They lack an epithelial lining and contain amylase-rich fluid.8 US, CT, and EUS may help in the diagnosis. In our case, the diagnosis was made before surgery by EUS-guided cyst aspiration and ERP. Surgical resection led to a satisfactory outcome. This case provides a fascinating opportunity to explore embryogenesis of the alimentary tract, as well as highlighting the usefulness of EUS and ERP in preoperative diagnosis. DISCLOSURE All authors disclosed no financial relationships relevant to this publication. Abbreviations: ERP, endoscopic retrograde pancreatography; GDCs, gastric duplication cysts.
REFERENCES 1. Muraoka A, Tsuruno M, Katsuno G, et al. A gastric duplication cyst with an aberrant pancreatic ductal system: report of a case. Surg Today 2002;32:531-5. 2. Camoglio FS, Forestieri C, Zanatta C, et al. Complete pancreatic ectopia in a gastric duplication cyst: a case report and review of the literature. Eur J Pediatr Surg 2004;14:60-2. 3. O’Donnell PL, Morrow JB, Fitzgerald TL. Adult gastric duplication cysts: a case report and review of literature. Am Surg 2005;71:522-5. 4. Takahara T, Torigoe T, Haga H, et al. Gastric duplication cyst: evaluation by endoscopic ultrasonography and magnetic resonance imaging. J Gastroenterol 1996;31:420-4. 5. Johnstone DW, Forde KA, Markowitz D, et al. Gastric duplication cyst communicating with the pancreatic duct: a rare cause of recurrent abdominal pain. Surgery 1991;109:97-100. 6. Coit DG, Mies C. Adenocarcinoma arising within a gastric duplication cyst. J Surg Oncol 1992;50:274-7. 7. Horne G, Ming-Lum C, Kirkpatrick AW, et al. High-grade neuroendocrine carcinoma arising in a gastric duplication cyst: a case report with literature review. Int J Surg Pathol 2007;15:187-91. 8. Yang MC, Duh YC, Lai H, et al. Alimentary tract duplications. J Formos Med Assoc 1996;95:406-9.
Current affiliations: Departments of Medicine (T.S.), Pathology (J.L., J.S.), Gastroenterology (R.A. M.D.), Allegheny General Hospital, Pittsburgh, Pennsylvania, USA. Reprint requests: Trupti Shinde, MD, Internal Medicine, Allegheny General Hospital, Medicine Department, 320 E. North Ave, Pittsburgh, PA 15212. Copyright ª 2009 by the American Society for Gastrointestinal Endoscopy 0016-5107/$36.00 doi:10.1016/j.gie.2008.04.059
Pancreatic somatostatinoma and tuberous sclerosis: case report of an exceedingly rare association Jayaprakash Sreenarasimhaiah, MD, Luis A. Armstrong, MD, Shou-Jiang Tang, MD, Carlton Barnett, MD Dallas, Texas, USA
Tuberous sclerosis is an autosomal dominant condition characterized by the triad of mental deficiency, epilepsy, and the classic skin eruption known as adenoma sebaceum.1 Other findings include visceral lesions with histology similar to the skin lesions, renal angiomyolipomas, and pancreatic abnormalities. Findings in the pancreas can include pancreatic cysts, rare angiomyolipomas, and neuroendocrine tumors of the nonfunctioning islet cell variety.2 Results of certain reports suggest that functional and nonfunctional insulinomas may be associated with this disorder. To our knowledge, this is the first reported
A 24-year-old man with a history of tuberous sclerosis complained of 3 months of dull abdominal pain. He denied symptoms of GI bleeding, jaundice, nausea, or vomiting, or association with food intake. He reported a mild weight loss of 10 pounds during this period. His history also included
www.giejournal.org
Volume 69, No. 2 : 2009 GASTROINTESTINAL ENDOSCOPY 379
case of pancreatic somatostatinoma associated with tuberous sclerosis.
CASE REPORT