Clinical Review & Education
JAMA Surgery Clinical Challenge
An Underappreciated Cause of Postprandial Abdominal Pain Reilly D. Hobbs, MD, MBS; Jessica A. Cintolo, MD; Robert E. Roses, MD
A Endoscopic ultrasonographic image
B
Figure. A, Endoscopic ultrasonography of the gastric antrum mass reveals an area of gastric thickening (arrowheads) corresponding to computed tomographic findings. During endoscopy, this area was visualized grossly as a bulge in the atrium along the incisura. A fine-needle aspiration biopsy was
Magnetic resonance cholangiopancreatographic image
nondiagnostic, showing gastric antral-type mucosa without pathologic changes (inset). B, The coronal image in T2 phase reveals a 1.8 × 2.4 × 1.5-cm hypotense lesion (arrowhead) in the gastric antrum. The lesion was also enhanced on T1 arterial phase sequencing.
A 52-year-old woman with a history of diabetes mellitus and cigarette smoking who had a prior laparoscopic cholecystectomy was referred to our institution for severe postprandial epigastric abdominal pain that had been ongoing for several months and unintentional weight loss. Quiz at jamasurgery.com She had been prescribed total parenteral nutrition prior to referral. On presentation, her physical examination was remarkable for mild epigastric tenderness. The results of laboratory testing, including a complete blood cell count, chemistry values, liver function tests, and amylase and lipase levels, were unremarkable. Computed tomography demonstrated focal gastric thickening. Esophagogastroduodenoscopy with endoscopic ultrasonography was performed that confirmed a submucosal gastric mass (Figure, A). A fine-needle aspiration biopsy revealed benign and reactive glandular cells and debris. Magnetic resonance cholangiopancreatography demonstrated a 2.4-cm hypervascular and exophytic mass in the gastric antrum and locoregional lymphadenopathy (Figure, B).
jamasurgery.com
WHAT IS YOUR DIAGNOSIS?
A. Gastrointestinal stromal tumor B. Gastric cancer C. Peptic ulcer disease D. Ectopic pancreas
(Reprinted) JAMA Surgery September 2015 Volume 150, Number 9
Copyright 2015 American Medical Association. All rights reserved.
Downloaded From: http://archsurg.jamanetwork.com/ by a Florida State University User on 09/17/2015
907
Clinical Review & Education JAMA Surgery Clinical Challenge
Diagnosis D. Ectopic pancreas
Discussion Pancreatic heterotopia, also referred to as an ectopic pancreas, is defined by the presence of aberrant pancreatic tissue that lacks anatomic connection with the body of the pancreas.1 Gastric ectopic pancreas is often asymptomatic and found incidentally during surgery or during an autopsy. Cadaveric studies have shown that gastric ectopic pancreas is present in 0.5% to 13.5% of the population.2,3 Other sites of an ectopic pancreas include the duodenum, small bowel, gallbladder, esophagus, mesentery, and mediastinum; location in the stomach accounts for 25% to 38% of all cases.4 A minority of patients with a gastric ectopic pancreas develop symptoms, which may include malignant degeneration, gastric outlet obstruction, early satiety, and chronic abdominal pain. The workup for gastric ectopic pancreas should include thorough history taking and physical examination, along with appropriate laboratory, imaging, and endoscopic studies. Computed tomography and magnetic resonance imaging studies usually reveal a nonspecific submucosal gastric mass or thickening; the differential ARTICLE INFORMATION Author Affiliations: Department of Surgery, University of Pennsylvania, Philadelphia (Hobbs, Cintolo); Division of Endocrine and Oncologic Surgery, Department of Surgery, University of Pennsylvania, Philadelphia (Roses).
diagnosis includes a gastrointestinal stromal tumor, leiomyoma, carcinoid, or carcinoma.5 Dynamic upper gastrointestinal contrast studies demonstrate a gastric luminal filling defect in 20% to 50% of patients.6,7 Endoscopy is a useful adjunct and provides better localization. Endoscopic ultrasonography with fine-needle aspiration may afford a tissue diagnosis. Histologically, gastric ectopic pancreas may be confined to the submucosa or involve the muscularis and serosal layers.8,9 Exocrine tissue is identified at the time of biopsy in approximately 50% of cases.5 Definitive diagnosis is sometimes elusive because overlying normal gastric mucosa can obscure lesions. Strong consideration should be given to surgical exploration in the case of suspected pancreatic heterotopia if symptomatic or to rule out malignancy when the diagnosis is uncertain. Localized excision is favored over a more extensive gastric resection when the diagnosis can be reasonably confirmed. Our patient underwent an open exploration and gastric wedge resection of the lesser curvature mass. A frozen section of the resected mass revealed ectopic pancreatic tissue. After an uneventful postoperative course, the patient’s abdominal pain completely resolved.
2. Bussolati G. Heterotopic pancreas. Am J Surg Pathol. 1996;20(11):1427-1428. 3. Eisenberger CF, Gocht A, Knoefel WT, et al. Heterotopic pancreas—clinical presentation and pathology with review of the literature. Hepatogastroenterology. 2004;51(57):854-858.
Corresponding Author: Reilly D. Hobbs, MD, MBS, Department of Surgery, University of Pennsylvania, 4 Maloney Bldg, 3400 Spruce St, Philadelphia, PA 19104 ([email protected]).
4. Sumiyoshi T, Shima Y, Okabayashi T, et al. Heterotopic pancreas in the common bile duct, with a review of the literature. Intern Med. 2014;53 (23):2679-2682.
Section Editor: Pamela A. Lipsett, MD, MHPE.
5. Ormarsson OT, Gudmundsdottir I, Mårvik R. Diagnosis and treatment of gastric heterotopic pancreas. World J Surg. 2006;30(9):1682-1689.
Published Online: July 1, 2015. doi:10.1001/jamasurg.2015.0697. Conflict of Interest Disclosures: None reported. REFERENCES
7. Kilman WJ, Berk RN. The spectrum of radiographic features of aberrant pancreatic rests involving the stomach. Radiology. 1977;123(2):291296. 8. Jang KY, Park HS, Moon WS, Kim CY, Kim SH. Heterotopic pancreas in the stomach diagnosed by endoscopic ultrasound-guided fine needle aspiration cytology. Cytopathology. 2010;21(6):418420. 9. DeBord JR, Majarakis JD, Nyhus LM. An unusual case of heterotopic pancreas of the stomach. Am J Surg. 1981;141(2):269-273.
6. Lai EC, Tompkins RK. Heterotopic pancreas: review of a 26 year experience. Am J Surg. 1986;151 (6):697-700.
1. Gokhale UA, Nanda A, Pillai R, Al-Layla D. Heterotopic pancreas in the stomach: a case report and a brief review of the literature. JOP. 2010;11 (3):255-257.
908
JAMA Surgery September 2015 Volume 150, Number 9 (Reprinted)
Copyright 2015 American Medical Association. All rights reserved.
Downloaded From: http://archsurg.jamanetwork.com/ by a Florida State University User on 09/17/2015
jamasurgery.com
JAMA Surgery Clinical Challenge
An Underappreciated Cause of Postprandial Abdominal Pain Reilly D. Hobbs, MD, MBS; Jessica A. Cintolo, MD; Robert E. Roses, MD
A Endoscopic ultrasonographic image
B
Figure. A, Endoscopic ultrasonography of the gastric antrum mass reveals an area of gastric thickening (arrowheads) corresponding to computed tomographic findings. During endoscopy, this area was visualized grossly as a bulge in the atrium along the incisura. A fine-needle aspiration biopsy was
Magnetic resonance cholangiopancreatographic image
nondiagnostic, showing gastric antral-type mucosa without pathologic changes (inset). B, The coronal image in T2 phase reveals a 1.8 × 2.4 × 1.5-cm hypotense lesion (arrowhead) in the gastric antrum. The lesion was also enhanced on T1 arterial phase sequencing.
A 52-year-old woman with a history of diabetes mellitus and cigarette smoking who had a prior laparoscopic cholecystectomy was referred to our institution for severe postprandial epigastric abdominal pain that had been ongoing for several months and unintentional weight loss. Quiz at jamasurgery.com She had been prescribed total parenteral nutrition prior to referral. On presentation, her physical examination was remarkable for mild epigastric tenderness. The results of laboratory testing, including a complete blood cell count, chemistry values, liver function tests, and amylase and lipase levels, were unremarkable. Computed tomography demonstrated focal gastric thickening. Esophagogastroduodenoscopy with endoscopic ultrasonography was performed that confirmed a submucosal gastric mass (Figure, A). A fine-needle aspiration biopsy revealed benign and reactive glandular cells and debris. Magnetic resonance cholangiopancreatography demonstrated a 2.4-cm hypervascular and exophytic mass in the gastric antrum and locoregional lymphadenopathy (Figure, B).
jamasurgery.com
WHAT IS YOUR DIAGNOSIS?
A. Gastrointestinal stromal tumor B. Gastric cancer C. Peptic ulcer disease D. Ectopic pancreas
(Reprinted) JAMA Surgery September 2015 Volume 150, Number 9
Copyright 2015 American Medical Association. All rights reserved.
Downloaded From: http://archsurg.jamanetwork.com/ by a Florida State University User on 09/17/2015
907
Clinical Review & Education JAMA Surgery Clinical Challenge
Diagnosis D. Ectopic pancreas
Discussion Pancreatic heterotopia, also referred to as an ectopic pancreas, is defined by the presence of aberrant pancreatic tissue that lacks anatomic connection with the body of the pancreas.1 Gastric ectopic pancreas is often asymptomatic and found incidentally during surgery or during an autopsy. Cadaveric studies have shown that gastric ectopic pancreas is present in 0.5% to 13.5% of the population.2,3 Other sites of an ectopic pancreas include the duodenum, small bowel, gallbladder, esophagus, mesentery, and mediastinum; location in the stomach accounts for 25% to 38% of all cases.4 A minority of patients with a gastric ectopic pancreas develop symptoms, which may include malignant degeneration, gastric outlet obstruction, early satiety, and chronic abdominal pain. The workup for gastric ectopic pancreas should include thorough history taking and physical examination, along with appropriate laboratory, imaging, and endoscopic studies. Computed tomography and magnetic resonance imaging studies usually reveal a nonspecific submucosal gastric mass or thickening; the differential ARTICLE INFORMATION Author Affiliations: Department of Surgery, University of Pennsylvania, Philadelphia (Hobbs, Cintolo); Division of Endocrine and Oncologic Surgery, Department of Surgery, University of Pennsylvania, Philadelphia (Roses).
diagnosis includes a gastrointestinal stromal tumor, leiomyoma, carcinoid, or carcinoma.5 Dynamic upper gastrointestinal contrast studies demonstrate a gastric luminal filling defect in 20% to 50% of patients.6,7 Endoscopy is a useful adjunct and provides better localization. Endoscopic ultrasonography with fine-needle aspiration may afford a tissue diagnosis. Histologically, gastric ectopic pancreas may be confined to the submucosa or involve the muscularis and serosal layers.8,9 Exocrine tissue is identified at the time of biopsy in approximately 50% of cases.5 Definitive diagnosis is sometimes elusive because overlying normal gastric mucosa can obscure lesions. Strong consideration should be given to surgical exploration in the case of suspected pancreatic heterotopia if symptomatic or to rule out malignancy when the diagnosis is uncertain. Localized excision is favored over a more extensive gastric resection when the diagnosis can be reasonably confirmed. Our patient underwent an open exploration and gastric wedge resection of the lesser curvature mass. A frozen section of the resected mass revealed ectopic pancreatic tissue. After an uneventful postoperative course, the patient’s abdominal pain completely resolved.
2. Bussolati G. Heterotopic pancreas. Am J Surg Pathol. 1996;20(11):1427-1428. 3. Eisenberger CF, Gocht A, Knoefel WT, et al. Heterotopic pancreas—clinical presentation and pathology with review of the literature. Hepatogastroenterology. 2004;51(57):854-858.
Corresponding Author: Reilly D. Hobbs, MD, MBS, Department of Surgery, University of Pennsylvania, 4 Maloney Bldg, 3400 Spruce St, Philadelphia, PA 19104 ([email protected]).
4. Sumiyoshi T, Shima Y, Okabayashi T, et al. Heterotopic pancreas in the common bile duct, with a review of the literature. Intern Med. 2014;53 (23):2679-2682.
Section Editor: Pamela A. Lipsett, MD, MHPE.
5. Ormarsson OT, Gudmundsdottir I, Mårvik R. Diagnosis and treatment of gastric heterotopic pancreas. World J Surg. 2006;30(9):1682-1689.
Published Online: July 1, 2015. doi:10.1001/jamasurg.2015.0697. Conflict of Interest Disclosures: None reported. REFERENCES
7. Kilman WJ, Berk RN. The spectrum of radiographic features of aberrant pancreatic rests involving the stomach. Radiology. 1977;123(2):291296. 8. Jang KY, Park HS, Moon WS, Kim CY, Kim SH. Heterotopic pancreas in the stomach diagnosed by endoscopic ultrasound-guided fine needle aspiration cytology. Cytopathology. 2010;21(6):418420. 9. DeBord JR, Majarakis JD, Nyhus LM. An unusual case of heterotopic pancreas of the stomach. Am J Surg. 1981;141(2):269-273.
6. Lai EC, Tompkins RK. Heterotopic pancreas: review of a 26 year experience. Am J Surg. 1986;151 (6):697-700.
1. Gokhale UA, Nanda A, Pillai R, Al-Layla D. Heterotopic pancreas in the stomach: a case report and a brief review of the literature. JOP. 2010;11 (3):255-257.
908
JAMA Surgery September 2015 Volume 150, Number 9 (Reprinted)
Copyright 2015 American Medical Association. All rights reserved.
Downloaded From: http://archsurg.jamanetwork.com/ by a Florida State University User on 09/17/2015
jamasurgery.com
