| Abstract|| |
Pancreatic and gastric heterotopias are rare congenital anomalies which have been reported throughout the length of the gastrointestinal tract. Combined gastric and pancreatic heterotopias, although very rare, have been described mainly in the duodenum followed by jejunum with ileum being a rare site. The reported incidence of this combined heterotopias is low, ranging from <1% to 13%. Extensive literature search has revealed that only Four cases of combined pancreatic and gastric heterotopias have been reported in the small intestine till date. Hence, we report this case for its rarity and unusual presentation as intussusception in a young male.
Keywords: Ileum, intussception, mixed gastric and pancreatic heterotopias
|How to cite this article:|
Singh T, Tripathi S, Tewari S, Varma K, Misra V. Combined pancreatic and gastric heterotopia in small intestine presenting as intussusception—A rare congenital anomaly. Indian J Pathol Microbiol 2021;64, Suppl S1:175-7
|How to cite this URL:|
Singh T, Tripathi S, Tewari S, Varma K, Misra V. Combined pancreatic and gastric heterotopia in small intestine presenting as intussusception—A rare congenital anomaly. Indian J Pathol Microbiol [serial online] 2021 [cited 2021 Jun 23];64, Suppl S1:175-7. Available from: https://www.ijpmonline.org/text.asp?2021/64/5/175/317919
| Introduction|| |
Heterotopia is the presence of normal tissue in an abnormal location presumably due to an error during embryological development. The reported incidence of combined gastric and pancreatic heterotropia (CGPH) is low, ranging from <1% to 13%. Till date, only four cases of CGPH have been reported in the small intestine, ours being the fifth case.,, It is usually asymptomatic but may present with symptoms, the most common being abdominal pain followed by melena, diarrhea, gas, and bloating. Grossly it can present as a polypoidal mass which was seen in our case and this can present as lead point of intussusception. Intussusception is a rare presentation and has been reported only in one previous case where it was associated with lipoma. Delayed or missed diagnosis is frequent as presenting symptoms are indistinguishable from more common infectious, inflammatory, or neoplastic diseases; therefore, a high degree of suspicion and awareness regarding this entity is essential.
Hence, we report this case with unusual diagnosis at a rare site presenting as intussusception in a young male.
| Case History|| |
A 14 year old male presented with an episode of acute pain in abdomen not associated with nausea, vomiting, or hematemesis. There was no past history of diarrhea, constipation, or hematochezia. On examining further, no tenderness or ascites was found. The ultrasonography of the whole abdomen showed fluid-filled dilated small bowel loops suggestive of intestinal obstruction [Figure 1]a. Patient underwent an emergency exploratory laprotomy, in which intussusception of the small intestine was seen which was followed by a procedure of resection anastomosis and peritoneal lavage. The resected part of bowel loop was sent to Department of Pathology for histopathological examination.
|Figure 1: (a) Ultrasonography of the whole abdomen showing fluid-filled dilated small bowel loops suggestive of intestinal obstruction. (b) Single, sessile polypoidal mass seen measuring 1 × 1 × 1 cm in size|
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A resected ileal segment (intussusceptum) was received measuring 21 cm in length. On cutting through anti-mesentric border, a sessile polypoidal mass that was arising from the mucosa measuring 1 × 1 × 1 cm in size at the leading edge of intussusceptum was seen [Figure 1]b. Mucosa in rest of the intestinal segment showed reddish-brown ischemic areas. Sections from various representative areas were taken and processed.
On microscopy, sections examined showed a polypoidal mass composed of gastric and pancreatic mucosa. The gastric component showed normal appearing cypts, foveolae, antral, and fundic glands, some of which were cystically dilated. Focal areas of parietal cell hyperplasia were also seen along with areas of dilated and congested blood vessels and mild inflammatory infiltrate. The pancreatic component showed exocrine pancreatic glands and ducts with granular eosinophilic cytoplasm and moderate chronic inflammatory infiltrate. No pancreatic endocrine component was found. Adjacent areas showed normal appearing intestinal mucosa with focal areas showing transmural ischemic necrosis. At places, mixed gastric and pancreatic mucosa was seen [Figure 2]a,[Figure 2]b,[Figure 2]c,[Figure 2]d.
|Figure 2: (a) Sections show transition zone between normal appearing intestinal mucosa (left) and mixed gastric and pancreatic heterotropia (right) [H and E ×100]. (b) Sections show gastric pits and foveolae lined by pale staining mucous cells and surrounded by parietal cells [H and E ×100]. (c) Section showing exocrine pancreatic glands and ducts with granular eosinophilic cytoplasm [H and E ×400]. (d) Section showing both gastric and pancreatic component [H and E ×100]|
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There was no evidence of granuloma or malignancy in the sections examined. A histopathological diagnosis of mixed gastric and pancreatic heterotopia with no evidence of dysplasia was given.
| Discussion|| |
The word “heterotopia” is derived from a Greek word, which means “other places.” Hence, gastric and pancreatic heterotropia means presence of gastric or pancreatic tissue, outside their typical location, without their vascular or anatomical continuity with the pancreas or stomach proper. The reported incidence of heterotopic pancreas or gastric tissue in the general population is low, ranging from <1% to 13%.
The etiology of this entity remains unclear, but these are believed to be congenital abnormalities occurring during embryonic development, such as rotation of the foregut and fusion of the dorsal and ventral pancreatic buds, although a metaplastic process has also been implicated.,
CGPH has been earliest reported by Burne et al. in transverse colon. Heterotopic, pancreatic, and gastric tissues are more often found in the upper gastrointestinal tract than in the lower intestinal segments, including the hepatobiliary tree. It is most common in the duodenum and jejunum. Involvement of other organs such as esophagus, terminal ileum, Meckel's diverticulum, colon, ampulla of Vater, gallbladder, spleen, mesentery, liver, bile duct, omentum, and urinary bladder are rare but have been previously reported. In our case, a polypoid mass of heterotropic tissue was seen in distal ileum, which is a rare site.
The clinical manifestations depend on the site and size of heterotopia. In a study of 87 cases of heterotropic gastric mucosa, majority showed clinical features of peptic ulcer disease, enteritis, or polyp formation, whereas pancreatic heterotopia becomes clinically symptomatic when its size reaches 1 cm and may present with pain, anemia, or melena. In the rare cases where CGPH reported together, clinical presentations resulted from a combination of these symptoms., Our case was asymptomatic as the size of polyp was around 1 cm and was discovered as an incidental finding on histopathology.
Imaging studies and endoscopic visualization are neither specific nor sensitive enough to diagnose heterotopias, and histopathological examination remains the gold standard for identifying such a process where the gastric and pancreatic components can be readily identified as was seen in our case where gastric foveolae and pyloric glands along with exocrine pancreas with duct system were seen. Usually, they have a benign course; however, preneoplastic lesions and neoplasms including adenocarcinomas and carcinoids arising from heterotopic tissue have been reported. Surgical resection was the only therapeutic option until recently, to remove distal small bowel lesions and to obtain the essential histology for diagnosis but newer interventions like endoscopic resection are now been practiced which is a safer and less invasive alternative. On follow-up, patient's symptoms related to intermittent intussusception were relieved and he is alive and well.
Thus, our case highlights that a carefully acquired clinical and radiological evaluation followed by histopathology with a high degree of suspicion helps in coming to a definitive diagnosis and proper management of this infrequent yet vicious lesion.
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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Department of Pathology, Moti Lal Nehru Medical College, Prayagraj - 211 001, Uttar Pradesh
Source of Support: None, Conflict of Interest: None
[Figure 1], [Figure 2]