Small-intestinal cancer arising from heterotopic pancreas

Table of Contents

We report on a case of jejunal cancer arising from heterotopic pancreas, as depicted by small-bowel radiography and double balloon endoscopy.

A 64-year-old woman was admitted to our hospital with abdominal distension and epigastric pain. Small-bowel radiography with double contrast study showed a stenosis in the jejunum and a dilatation of the proximal small intestine ([Fig. 1]). Oral double balloon endoscopy showed a smooth, ulcerating tumor that involved the jejunum circumferentially ([Fig. 2]). Under laparotomy, a solid mass was seen with a stenosis about 45 cm distal to the ligament of Treitz. Intraoperative enteroscopy from the anal side of the stenosis revealed a nodular and friable tumor with an ulcer ([Fig. 3]). The segment of the jejunum containing the tumor together with some enlarged lymph nodes were removed.

Macroscopically, there was a mass with an umbilication and an irregular ulcer, which was covered with normal mucosa ([Fig. 4]). Histologically, there was pancreatic tissue within the submucosa and the muscularis propria in the proximal part of the tumor, and adenocarcinoma cells were seen peripherally in the pancreatic tissue ([Fig. 5] and [6]). These findings were compatible with the diagnosis of adenocarcinoma originating from heterotopic pancreas. Although we treated the patient by chemotherapy with gemcitabine, she died as a result of carcinomatous peritonitis 5 months after the surgery.

Although extremely rare, there have been cases in which small-intestinal heterotopic pancreas was presumed to have transformed into adenocarcinoma [1] [2] [3]. The images from our case can be summarized as asymmetrical luminal narrowing with a smooth tumor in the oral side and an ulcerating nodular tumor in the anal side. Our case suggests that enteroscopists should regard heterotopic pancreas as a possible premalignant lesion.

Fig. 1 Small-bowel radiography shows a protrusion with smooth margin accompanied by stenosis in the jejunum. There were barium flecks and fold convergences in the anal side of the stenosis.

Fig. 2 Oral double balloon endoscopy shows a submucosal tumor in the jejunum.

Fig. 3 Intraoperative enteroscopy from the anal side of the stenosis reveals a nodular and friable tumor with an ulcer in the center.

Fig. 4 A macroscopic view of the resected specimen shows that the tumor with an umbilication is covered by normal mucosa.

Fig. 5 Histologic examination of the specimen shows that the tumor is composed of areas of heterotopic pancreas (surrounded by blue line) and adenocarcinoma (surrounded by black line).

Fig. 6 A high-power view of the carcinomatous area indicates that the cancer cells are arranged in tubular and cribriform patterns with abundant fibrous stroma.

Endoscopy_UCTN_Code_CCL_1AC_2AC

References

• 1 Persson G E, Boiesen P T. Cancer of aberrant pancreas in jejunum. Case report.  Acta Chir Scand. 1988;  154 599-601
• 2 Makhlouf H R, Almeida J L, Sobin L H. Carcinoma in jejunal pancreatic heterotopia.  Arch Pathol Lab Med. 1999;  123 707-711
• 3 Arao J, Fukui H, Hirayama D. et al . A case of aberrant pancreatic cancer in the jejunum.  Hepatogastroenterology. 1999;  46 504-507