European Journal of Radiology Extra 73 (2010) e25–e28
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Gastric cystic dystrophy in heterotopic pancreas demonstrating annular appearance Takahide Kakigi a,∗ , Taro Shimono c , Taizo Hiraoka a , Yumi Matsushima b , Hiroshi Tatsuta b , Kazumasa Nishimura a a b c
Departments of Radiology, Saiseikai Ibaraki Hospital, Osaka, Japan Departments of Gastroenterology, Saiseikai Ibaraki Hospital, Osaka, Japan Department of Radiology, Kinki University School of Medicine, Osaka, Japan
a r t i c l e
i n f o
Article history: Received 14 July 2009 Received in revised form 14 August 2009 Accepted 26 August 2009
Keywords: Cystic dystrophy Heterotopic pancreas Stomach CT MRI
a b s t r a c t Cystic dystrophy in heterotopic pancreas (CDHP), an uncommon complication of heterotopic pancreas, occurs most often in the duodenal wall. We report a rare case of CDHP in the stomach. Preoperative diagnosis was made by upper gastrointestinal endoscopy and abdominal radiological studies. Radiological studies revealed an annular cystic appearance of CDHP, characterizing this rare entity. We describe the radiological–pathological correlation of CDHP and discuss the imaging findings. © 2009 Elsevier Ireland Ltd. All rights reserved.
1. Introduction
2. Case report
Cystic dystrophy in heterotopic pancreas (CDHP) is defined by the presence of cysts surrounded by inflammation and fibrosis in the wall of the digestive tract, intermingled with heterotopic pancreatic ducts and lobules. This pathology is typically identified in the duodenum [1]. Cysts correspond to distended pancreatic ducts filled with mucin. Heterotopic pancreas is defined as pancreatic tissue in an extrapancreatic location with neither anatomical nor vascular continuity with the pancreas proper, and shows an incidence of 1–14% in autopsies. The most common locations are the stomach, duodenum, and jejunum [2]. To the best of our knowledge, only 4 cases of gastric CDHP in the English literature have included findings from computed tomography (CT) and magnetic resonance imaging (MRI) [3,4]. We describe herein the case of a patient with CDHP in the gastric antrum with suggestive radiological findings.
A 34-year-old man was admitted to our hospital with epigastric pain, vomiting after meal intake, and weight loss. Episodes of epigastric pain first developed 1 week earlier, and endoscopic diagnosis of duodenal ulcer was made in another hospital. Rabeprazole (an antiulcer drug in the class of proton pump inhibitors) was administered, to no effect. The patient had no history of abdominal surgery, diarrhea, or hematochezia. He did not drink alcohol and did not smoke. Body temperature was 37.9 ◦ C, blood pressure was 136/72 mmHg, and heart rate was 82 beats/min. No anemia or jaundice was apparent. The abdomen was soft and flat, and bowel sounds were decreased. Tenderness was identified in the upper abdomen, but without guarding or tenderness. Hematologic and serum chemistry tests showed an elevated white cell count of 13,300/mm3 (reference range: 4000–9000/mm3 ), amylase 418 IU/l (42–116 IU/l), and P-amylase 386 IU/l (17–52 IU/l). Urine testing yielded positive results (3+) for ketones and also showed an elevated amylase level of 4950 IU/l (50–600 IU/l). Acute pancreatitis was suspected. Abdominal ultrasonography (US) revealed a thickened hypoechoic lesion in the gastric antrum and a normal-appearing pancreas. Abdominal contrast-enhanced CT revealed a cystic lesion in the thickened antrum (Fig. 1A) and a mass showing similar enhance-
∗ Corresponding author at: Department of Radiology, Saiseikai Ibaraki Hospital, 2-1-45 Mitsukeyama, Ibaraki, Osaka 567-0035, Japan. Tel.: +81 72 622 8651; fax: +81 72 627 2022. E-mail address:
[email protected] (T. Kakigi). 1571-4675/$ – see front matter © 2009 Elsevier Ireland Ltd. All rights reserved. doi:10.1016/j.ejrex.2009.08.005
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Fig. 1. (A) Contrast-enhanced CT showing cystic lesion (arrows) in the thickened antrum. (B) Contrast-enhanced CT showing a mass lying at the base of the antrum along the greater curvature (arrows), displaying contrast material enhancement similar to that of normal pancreas (arrowheads). (C) CT in a parasagittal plane to depict the gastroduodenal lumen demonstrates an annular cystic lesion around the antral wall (arrow). A mass is seen at the base of the antrum (arrowheads). L, lumen.
ment to normal pancreas, lying at the base of the antrum along the greater curvature (Fig. 1B). Multiphase contrast-enhanced CT was not performed. Furthermore, plain CT in an oblique sagittal plane to depict the gastroduodenal lumen revealed an annular cystic lesion around the antral wall (Fig. 1C). MRI also revealed a cystic lesion in the thickened antral wall (Fig. 2). Upper gastrointestinal endoscopy showed deformity and swelling of the antrum without mucosal abnormality, and granular appearance of duodenal bulb mucosa.
Fig. 2. T2-weighted MRI demonstrating a cystic lesion (arrows) in the antrum.
On hospital day 14, endoscopic US (20-MHz mini probe) showed a cystic lesion without solid mass in the submucosal layer of the antrum, and normal mucosa. Analysis of pink mucinous fluid that spurted from the anterior wall of the duodenal bulb showed elevated levels of both amylase (7067 IU/l) and P-amylase (6887 IU/l). On hospital day 21, a drainage tube was inserted in the duodenal bulb and cystography revealed the annular appearance of the cystic space (Fig. 3). CDHP was suspected. On hospital day 30, distal gastrectomy was performed due to continued epigastric pain. Gross examination of the surgical specimens showed heterotopic pancreas, 4 cm × 3.3 cm, lying in the prepylorus along the greater curvature (Fig. 4A). The overlying mucosa was almost intact, but seemed somewhat polypoid. Jelly-like substance was seen under the mucosa in sectioned specimens, and was suspected to correspond to contrast material in the cystic lesion (Fig. 4B). On histological examination, heterotopic pancreatic tissue was seen in the submucosa, muscularis propria and serosa of the antrum, showing all the elements of normal pancreas, including endocrine and exocrine tissues and pancreatic ducts. The cystic lesion corresponded to dilated pancreatic ducts with clear columnar epithelium. In the largest cystic lesion, the epithelium was partially eroded, and the cyst was surrounding by inflammatory and granulation tissue (Fig. 5). Inflammatory reaction was seen only in the periductal area of the heterotopic pancreas, particularly near the orifice of the mucosal lumen, and was not seen in endocrine or exocrine tissues. This finding was consistent with periductitis of the heterotopic pancreas, and not with pancreatitis.
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Fig. 5. Histological examination (hematoxylin stain, ×100) reveals cyst (asterisk) corresponding to dilated pancreatic ducts with a clear columnar epithelium. The epithelium is partially eroded, and there is surrounding by inflammatory and granulation tissue. Fig. 3. Cystography with the patient in a prone position. An annular cystic appearance is seen.
3. Discussion CDHP mainly occurs in alcoholic men in the fifth decade of life, but may occur in nonalcoholic individuals. In a series by Rebours et al. [1], chronic pancreatitis of the pancreas proper was associated with CDHP in two-thirds of cases. However, CDHP may occur in nonalcoholic patients without chronic pancreatitis, suggesting this as an independent pathology rather than a complication of chronic pancreatitis. Symptoms of CDHP can be epigastric pain, vomiting and nausea from intolerance of food intake. Weight loss can be related to pain, leading to auto-limitation of food intake and vomiting due to gastric or duodenal stenosis [5]. The pathophysiology of CDHP remains unclear. CDHP can be induced by acute and chronic pancreatitis, secondary to obstruction of excretory ducts of heterotopic pancreatic tissue [1,2,6,7]. In the present case, inflammatory reaction was mainly seen in the periductal area and was not apparent in endocrine or exocrine tissues. We suspect that inflammation may have occurred following obstruction of excretory ducts from heterotopic pancreatic tissue, leading to acute and chronic periductitis due to bacteria from the digestive wall. This mechanism could explain the occurrence of CDHP in nonalcoholic patients. Inflamma-
tory reaction was seen in not only periductal area but also acinar cells, which exist in proximal side of excretory ducts and contain amylase. Therefore we think that the amylase levels in the serum and urine increased. We observed pink mucinous fluid spurted from the duodenal bulb, but cystic lesion was not seen in the duodenal bulb. We suppose that the pressure of cystic lesion in the stomach increased and penetrated to the duodenal bulb. In our case, CT and MRI showed an annular cystic lesion surrounding the antrum and heterotopic pancreas. CT in an oblique sagittal plane for depiction of the gastroduodenal lumen is more useful for depicting these appearances. The annular cystic lesion corresponds to the submucosal extension of mucin. To the best of our knowledge, CT and MRI results for 169 cases of duodenal CDHP and 4 cases of gastric CDHP have been described [1,3–5,8,9]. However, an annular cystic appearance has not been reported in the English literature. An annular cystic appearance could be a suggestive finding, and CT and MRI may be almost equal to cystography in detecting this appearance. Differential diagnoses for cystic changes in the digestive wall mainly comprise gastrointestinal submucosal pseudocyst and other cystic entities including gastrointestinal duplication cyst, ectopic pancreatic cystic neoplasm with solid component, and submucosal mesenchymal cystic neoplasm (cystic schwannoma and
Fig. 4. (A) Gross examination of the surgical specimens showed heterotopic pancreas (arrowheads), 4 cm × 3.3 cm, lying in the prepylorus (arrows: pylorus ring) along the greater curvature. (B) Cut surface of the surgical specimens showing the heterotopic pancreas, lying in the prepylorus along the greater curvature (arrows). This was also confirmed histopathologically as heterotopic pancreas. Jelly-like substance is seen under the mucosa (arrowheads). We suspect that this could represent contrast material in cysts.
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gasrtointestinal stromal tumor with cystic degeneration). CDHP and pseudocysts may have common CT characteristics for digestive wall involvement, but several differences exist between CDHP and pseudocysts [10]. In the series by Procacci et al. [7], in comparison to pseudocyst, CDHP lesions are more frequently elongated or bilobate. Our case supports this finding. Surgical treatment is usually recommended for symptomatic patients with CDHP. Ponchon et al. offered an alternative to surgical treatment using endoscopic fenestration of cysts, but this approach is feasible only in cases with relatively few cysts that are not dispersed and are relatively large in size [8]. Octreotide (somatostatin analogue) treatment, however, is an alternative to surgery when a diagnosis has been established, particularly for patients with excessive alcoholic consumption and CDHP associated with chronic pancreatitis [6]. CT and MRI can provide useful information concerning cystic lesions within the gastric wall and heterotopic pancreas. We think that the “annular cystic appearance” may be one of suggestive and useful imaging finding for the diagnosis of CDHP. Acknowledgements The authors wish to thank Tomo Terano, MD, PhD, and Toshihiro Adachi, MD, PhD, for reviewing the pathological specimens.
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