Case report of retroperitoneal ectopic pancreas with adrenal adenoma

Heterotopic pancreas is a congenital anomaly in which pancreatic tissue is anatomically separate from the main gland. The most common locations of this displacement include the upper gastrointestinal tract-specifically, the stomach, duodenum, and proximal jejunum. Less common sites are the esophagus, ileum, Meckel diverticulum, biliary tree, mesentery, and spleen. Uncomplicated heterotopic pancreas is typically asymptomatic, with the lesion being discovered incidentally during an unrelated surgery, during an imaging examination, or at autopsy. The most common computed tomographic appearance of heterotopic pancreas is that of a small oval intramural mass with microlobulated margins and an endoluminal growth pattern. The attenuation and enhancement characteristics of these lesions parallel their histologic composition. Acinus-dominant lesions demonstrate avid homogeneous enhancement after intravenous contrast material administration, whereas duct-dominant lesions are hypovascular and heterogeneous. At magnetic resonance imaging, the heterotopic pancreas is isointense to the orthotopic pancreas, with characteristic T1 hyperintensity and early avid enhancement after intravenous gadolinium-based contrast material administration. Heterotopic pancreatic tissue has a rudimentary ductal system in which an orifice is sometimes visible at imaging as a central umbilication of the lesion. Complications of heterotopic pancreas include pancreatitis, pseudocyst formation, malignant degeneration, gastrointestinal bleeding, bowel obstruction, and intussusception. Certain complications may be erroneously diagnosed as malignancy. Paraduodenal pancreatitis is thought to be due to cystic degeneration of heterotopic pancreatic tissue in the medial wall of the duodenum. Recognizing the characteristic imaging features of heterotopic pancreas aids in differentiating it from cancer and thus in avoiding unnecessary surgery. © RSNA, 2017.

To describe the computed tomographic (CT) findings of ectopic pancreas and to identify the features that differentiate it from other similarly manifesting gastric submucosal tumors such as gastrointestinal stromal tumor (GIST) and leiomyoma, which are the most common gastrointestinal submucosal tumors. This retrospective study was approved by the institutional review board and did not require informed consent. CT images of pathologically proved ectopic pancreases (n = 14), GISTs (n = 33), and leiomyomas (n = 7) in the stomach and duodenum were retrospectively reviewed. Analysis of the CT findings included evaluation of the location, contour, growth pattern, border, enhancement pattern, and enhancement grade of the tumor, as well as the presence of surface dimpling, prominent enhancement of overlying mucosa, and low intralesional attenuation. The attenuation of each lesion, the long diameter (LD), the short diameter (SD), and the LD/SD ratio were measured. Among these findings, statistically significant variables were determined by using the chi(2) test (to compare the categoric variables), the Student t test (for quantitative analysis), and the receiver operating characteristic curve (to determine the optimal cutoff of the LD/SD ratio). The typical location (prepyloric antrum and duodenum), endoluminal growth pattern, ill-defined border, prominent enhancement of overlying mucosa, and an LD/SD ratio of greater than 1.4 were found to be significant for differentiating ectopic pancreas from other tumors (P < .05 for each finding). When at least two of these five criteria were used in combination, the sensitivity and specificity for diagnosing ectopic pancreas were 100% (14 of 14) and 82.5% (33 of 40), respectively. When four of these criteria were used, a sensitivity of 42.9% and a specificity of 100% were achieved. By using specific CT criteria, ectopic pancreas can be differentiated from small GIST or leiomyoma with a high degree of accuracy. (c) RSNA, 2009.

Heterotopic pancreas is a rare condition and its preoperative diagnosis is difficult. It is generally asymptomatic, but it may become clinically evident depending on the size, location and the pathological changes. Heterotopic pancreas can occur anywhere in the gastrointestinal tract, but most commonly is found in the antrum of the stomach. We report an unusual case of a 31-year-old male patient with gastric outlet obstruction and chronic pancreatitis caused by a submucosal tumor at the pre-pyloric area revealed at endoscopy. Distal gastrectomy was performed and a histological diagnosis of heterotopic pancreas was established. The patient had an uneventful postoperative course and remained symptom free in the follow-up of six months. The difficulty of making an accurate preoperative diagnosis is highlighted and a review of the literature on this pathology is hereby presented. Although heterotopic pancreas is rare, it should be considered in the differential diagnosis of pancreatitis and gastric outlet obstruction caused by a submucosal gastric tumor.