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DOI:10.2214/AJR.05.1142
AJR 2007; 189:W262-W263
© American Roentgen Ray Society

Case Report

Giant Heterotopic Pancreas in the Jejunal Mesentery

Sang Soo Shin1, Yong Yeon Jeong2 and Heoung Keun Kang1

1 Department of Radiology, Chonnam National University Hospital, 8, Hack-dong, Dong-Gu, Gwangju, South Korea, 501-757.
2 Department of Radiology, Chonnam National University Hwasun Hospital, Jeollanam-do, South Korea, 519-809.

Received July 2, 2005; accepted after revision September 13, 2005.

 
WEB

This is a Web exclusive article.

Address correspondence to S. S. Shin (kjradsss{at}dreamwiz.com).

Keywords: congenital malformation • pancreas


Introduction
Top
 Introduction
Case Report
 Discussion
 References
 
Heterotopic pancreas is a congenital anomaly defined as pancreatic tissue that has no contact with the orthotopic pancreas and has its own duct system and vascular supply [1]. This anomaly has been reported to occur in several locations in the abdomen and mediastinum, most frequently in the stomach, duodenum, and upper part of the jejunum [2, 3]. The lesions are usually small, ranging from a few millimeters to a few centimeters, are asymptomatic, and are incidentally found during laparotomy or autopsy [4, 5]. Most of the cases of heterotopic pancreas reported in the literature have been described as a round or lobulated mass in the gastrointestinal tract [4–6]. We describe a case of giant heterotopic pancreas in the jejunal mesentery that caused gastrointestinal bleeding.


Case Report
Top
 Introduction
 Case Report
Discussion
 References
 
A 38-year-old man presented to the emergency department after one episode of syncope. He was alert and reported a 2-day history of melena. The heart rate was 96 beats/min, and the systolic blood pressure was 120 mm Hg. The hemoglobin level was 7.3 g/dL. Physical examination showed no abdominal tenderness.

Upper and lower gastrointestinal endoscopy revealed no ulcer or mass. Subsequent CT of the abdomen showed an elongated soft-tissue mass in the jejunal mesentery that conglomerated with adjacent loops of jejunum (Figs. 1A and 1B). The attenuation of this lesion was similar to that of orthotopic pancreas and extended to the periduodenal fat plane. Small-bowel follow-through images showed concentric luminal narrowing of the duodenal loop with intact mucosal folds and dilated loops of proximal jejunum with nodular filling defects (Fig. 1C). The preoperative radiologic diagnosis was jejunal intussusception caused by lymphoma of the jejunal mesentery.


Figure 1
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  		Fig. 1A —38-year-old man with giant heterotopic pancreas in jejunal mesentery. Contrast-enhanced CT scan shows elongated soft-tissue mass (arrow) in jejunal mesentery with attenuation similar to that of orthotopic pancreas (arrowhead).

 

Figure 2
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  		Fig. 1B —38-year-old man with giant heterotopic pancreas in jejunal mesentery. Contrast-enhanced CT scan shows conglomeration (arrows) of elongated soft-tissue mass in jejunal mesentery and adjacent jejunum.

 

Figure 3
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  		Fig. 1C —38-year-old man with giant heterotopic pancreas in jejunal mesentery. Small-bowel follow-through image shows dilated loop of proximal jejunum (arrows) with nodular filling defects (arrowheads).

 
Laparotomy revealed a soft-tissue mass 20 cm in diameter in the jejunal mesentery within approximately 15 cm from the ligament of Treitz. This mass appeared to infiltrate the adjacent jejunal wall, extended to the periduodenal fat plane, and encased the duodenal loop. The proximal jejunum was dilated, but no intussusception was found. The lesion was excised in toto with part of the adjacent jejunum (Fig. 1D). Histologic examination of the resected specimen showed that the lesion consisted mainly of pancreatic acini. Microscopic examination also revealed pancreatic tissue in the submucosa and muscularis propria of the jejunum (Fig. 1E). Several foci of ulceration were found adjacent to pancreatic tissue in the submucosa of the jejunal wall. There was no evidence of pathologic changes such as inflammation and malignant transformation in the pancreatic tissue. The postoperative course was uneventful. The patient was well and had no evidence of gastrointestinal bleeding 9 months after surgery.


Figure 4
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  		Fig. 1D —38-year-old man with giant heterotopic pancreas in jejunal mesentery. Photograph of gross specimen shows yellowish soft-tissue mass (arrows) in jejunal mesentery that abuts on jejunum.

 

Figure 5
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  		Fig. 1E —38-year-old man with giant heterotopic pancreas in jejunal mesentery. Photomicrograph of specimen from loop of jejunum shows pancreatic acini (arrows) focally replacing muscularis propria of jejunum. (H and E, x40)

 

Discussion
Top
 Introduction
 Case Report
 Discussion
References
 
The term heterotopic pancreas was first used by deCastro et al. [7] to describe pancreatic tissue in an aberrant location without vascular or ductal connection to the orthotopic pancreas. Heterotopic pancreas is a relatively frequent congenital anomaly, the reported incidence ranging from 2% to 15% [5]. The most frequent sites are the gastric antrum (30%), duodenum (30%), and jejunum (20%) [5]. On occasion, heterotopic pancreas has been found in the ileum, colon, spleen, liver, biliary tract, omentum, mesentery, and mediastinum [5, 6].

In the gastrointestinal tract, heterotopic pancreas has been reported to form lobulated intramural masses that are usually smaller than 3 cm in diameter [4–6]. The mass most commonly lies in the submucosa but can manifest as a subserosal nodule [5]. To our knowledge, there have been no reports of giant heterotopic pancreas in the mesentery. Several theories have been suggested to explain the occurrence of heterotopic pancreas [5, 8]. Because the heterotopic pancreatic tissue was located around the upper gastrointestinal tract near the pancreas, our case may be explained by the misplacement theory that heterotopic pancreas arises as a separation of tissue during embryonic rotation and fusion of the dorsal and ventral pancreatic buds [5].

The clinical significance of heterotopic pancreas is debatable. Although serious complications, which include pancreatitis, small-bowel obstruction, massive gastrointestinal bleeding, and malignant transformation, have been reported, heterotopic pancreas is usually found incidentally [4–6]. Heterotopic pancreas is capable of producing symptoms, depending on its location, its size, and involvement of the overlying mucosa. The patient in this case had a 2-day history of melena. This sign can be attributed to the presence of several histologically proven foci of ulceration of the jejunum adjacent to submucosal pancreatic tissue.

Heterotopic pancreas is well differentiated and can be indistinguishable histologically from orthotopic pancreas [5]. Thus it can be subject to the same pathologic changes as orthotopic pancreas. In this case, heterotopic pancreas appeared as a homogeneously enhanced mass with attenuation similar to that of orthotopic pancreas. The similarity might have been caused by histologic similarity.

Although the present case is unusual and although on CT scans it is difficult to differentiate mesenteric heterotopic pancreas from tumors such as lymphoma and metastatic lesions, the findings in our case suggest that heterotopic pancreas can be included in the differential diagnosis when a mesenteric mass has attenuation similar to that of orthotopic pancreas.


References
Top
 Introduction
 Case Report
 Discussion
 References
 

  1. Dolan RV, ReMine WH, Dockerty MB. The fate of heterotopic pancreatic tissue: a study of 212 cases. Arch Surg1974; 109:762 –765[Medline]
  2. Moen J, Mack E. Small bowel obstruction caused by heterotopic pancreas in an adult. Am Surg 1989;55 : 503–504[Medline]
  3. Thoeni RF, Gedgaudas RK. Ectopic pancreas: usual and unusual features. Gastrointest Radiol 1980;5 : 37–42[CrossRef][Medline]
  4. Eisenberger CF, Gocht A, Knoefel WT, et al. Heterotopic pancreas: clinical presentation and pathology with review of the literature. Hepatogastroenterology 2004;51 : 854–858[Medline]
  5. Makhlouf HR, Almeida JL, Sobin LH. Carcinoma in jejunal pancreatic heterotopia. Arch Pathol Lab Med 1999;123 : 707–711[Medline]
  6. Cho JS, Shin KS, Kwon ST, et al. Heterotopic pancreas in the stomach: CT findings. Radiology 2000;217 : 139–144[Abstract/Free Full Text]
  7. deCastro BJJ, Dockerty MB, Waugh JM. Pancreatic heterotopia: review of the literature and report of 41 authenticated surgical cases, of which 25 were clinically significant. Surg Gynecol Obstet1946; 82:527 –542
  8. Abel R, Keen CE, Bingham JB, Maynard J, Agrawal MR, Ramachandra S. Heterotopic pancreas as lead point in intussusception: new variant of vitellointestinal tract malformation. Pediatr Dev Pathol 1999; 2:367 –370[CrossRef][Medline]

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