|Year : 2019 | Volume
| Issue : 2 | Page : 71-72
Heterotopic pancreas in the jejunum: A case report and literature review
Souad Eldruki1, Sami A Lawgaly2
1 Departments of Pathology, Benghazi Medical Center, Benghazi, Libya
2 Departments of Medicine, Benghazi Medical Center, Benghazi, Libya
|Date of Web Publication||24-Jun-2019|
Dr. Sami A Lawgaly
Department of Medicine, Benghazi Medical Center, Benghazi
Source of Support: None, Conflict of Interest: None
Ectopic pancreas is the presence of the pancreatic tissue outside its typical location; it is an uncommon developmental anomaly. It may occur at different sites in the gastrointestinal tract with the stomach being the most common site. Usually, it is a silent anomaly, but it may become symptomatic when complicated by inflammation, bleeding, obstruction, or malignant transformation. In this report, a 42-year-old male case of refractory immune thrombocytopenia was admitted for elective open splenectomy. During the operation, an incidental rounded mass lesion arising from the jejunum was resected, and the diagnosis of ectopic pancreas was made after the histopathology result. Despite being a rare condition, ectopic pancreas should remain in the differential diagnosis of any jejunal mass lesion.
Keywords: Choristoma, ectopic, jejunum, pancreas
|How to cite this article:|
Eldruki S, Lawgaly SA. Heterotopic pancreas in the jejunum: A case report and literature review. Libyan J Med Sci 2019;3:71-2
| Introduction|| |
Ectopic pancreas (also known as heterotopic or aberrant pancreas) is the presence of the pancreatic tissue outside its typical location; it is an uncommon developmental anomaly with incidence at autopsy ranging from 0.5% to 13% in the general population. It may occur at different sites in the gastrointestinal (GI) tract with the stomach being the most common site. Usually, ectopic pancreas produces no symptoms, but it may become symptomatic when it gets complicated. A case of 42-year-old male with an ectopic pancreas located in the jejunum was reported in our hospital.
| Case Report|| |
A 42-year-old male case of refractory immune thrombocytopenia was admitted for elective open splenectomy. His physical examination showed no abnormalities. Laboratory investigation showed no significant abnormalities apart from isolated low platelet count. Abdominal ultrasound examination showed normal-sized spleen and pancreas with no other abnormalities. Computed tomography (CT) and upper GI endoscopy were not performed. Intraoperative finding includes a normal-sized spleen with an accessory spleen at its hilum with a rounded mass lesion arising from the jejunum, 10–20 cm away from the duodenojejunal junction, and splenectomy was done with removal of the jejunal mass.
The gross examination of the jejunal mass showed a grayish-brown soft irregular mass measuring 3.7 cm × 2.7 cm × 1.5 cm. Cut section shows a gray-white growth measuring 1.5 cm × 1 cm × 1 cm. Histopathologic examination of the lesion showed a section of mucosa of the duodenojejunal junction with the presence of pancreatic tissue just beneath the mucosa, mainly in the submucosa that contains pancreatic acini, ducts (exocrine part), and Islets of Langerhans More Details (endocrine part) [Figure 1]a and [Figure 1]b.
|Figure 1: (a) Low-magnification view of a heterotopic pancreas nodule in the jejunal submucosal area noticed that the normal jejunal epithelium is seen (H and E, ×40). (b): A heterotopic pancreas nodule showing the dilated ducts and acini with islands of endocrine cells scattered in between. (H and E, ×100)|
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| Discussion|| |
Ectopic (or heterotropic) pancreas is the presence of pancreatic tissue out of its anatomical site with no vascular communication and it has the same phenotypic and genotypic characteristics of that the pancreas has., The normal pancreas originates from the primitive duodenum through several evaginations that migrate to occupy the normal anatomical pancreatic position. If one of these evaginations fails to migrate, ectopic tissues persist. Pancreatic metaplasia of the endodermal tissue at the site affected is also another theory.
The upper part of the GI tract represents the most common site of the heterotopic pancreata (90% of the cases). Stomach, duodenum, and jejunum are affected the most, and less commonly, it affects the ileum or rarely Meckel's diverticulum. Other unusual locations have been reported suck as the gallbladder, bile ducts, splenic hilum, umbilicus, Fallopian tube More Detailss, mediastinum, esophagus, colon, omentum, lung, mediastinum, lymph nodes, and the retroperitoneal space. This anomaly was described for the first time in 1727 when they report a case of ectopic pancreas in ileal diverticulum. Later on in 1859, Klob described the detailed histological appearance of a heterotopic pancreas as it usually consists primarily of ducts and the surrounding mucin-producing glands with islets of Langerhans. Occasionally, only ducts or islets are present. Histopathologically, when the pancreatic acini, ducts, islets of Langerhans, and intervening connective tissue are present, the diagnosis can be made, and the most characteristic gross feature is a central ductal orifice. Specifically, in the stomach, the involvement of submucosal layer, muscularis, and subserosal layer is 73%, 17%, and 10%, respectively. Heinrich in 1909 classified heterotopic pancreata into Four types which had been modified by Gaspar Fuentes et al. in 1973: Type 1: ducts plus acini plus endocrine islets (typical pancreatic tissue), Type 2: ducts plus acini, Type 3: ducts with few acini or dilated ducts only also called adenomyoma, and Type 4: islet cells only (endocrine pancreas).
Despite its congenital origin, it usually manifests itself in adult life when complicated by bleeding, inflammation, obstruction, or malignant transformation. It is usually an incidental finding, either at the time of laparotomy or during radiographic or endoscopic examination of the upper gut. However, a heterotopic pancreas may present with gastric outlet obstruction and upper GI bleeding or rarely as small bowel obstruction and obstructive jaundice. Adenocarcinoma, islet cell tumors, and cystic tumors were reported in a heterotopic pancreas. Ectopic pancreas may appear in the upper GI endoscopy as a visible polyp. If endoscopy was done and it did not reveal any polyp, then it is recommended to do a frozen section in the cases found incidentally during surgery. Despite the advances in the diagnostic techniques, it is still difficult to diagnose a heterotropic pancreas preoperatively since there is no specific marker to diagnose a heterotopic pancreas. Amylase may be elevated in case of complications such as small bowel obstruction, pancreatitis, or GI bleeding. Endoscopically, the lesion may be seen as a submucosal polyp with a central umbilication. On the CT scan, the ectopic tissue enhances brightly similar to the orthotropic pancreas. Reduced enhancement may indicate pancreatic inflammation., Other imaging studies include the barium swallow which may show the typical image of a rounded filling defect with central indentation with sensitivity and specificity of 87.5% and 71.4%, respectively. About the treatment options, in a review of literature, resection of the ectopic pancreatic tissue is advisable when the lesion is encountered incidentally at surgery.
In conclusion, heterotropic pancreas is a rare condition that remains a diagnostic challenge despite the available modern diagnostic modalities. Surgical excision is recommended, especially if there is a diagnostic uncertainty.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
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Conflicts of interest
There are no conflicts of interest.
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