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Adenocarcinoma arising in a heterotopic pancreas (Heinrich type III): a case report
© Inoue et al; licensee BioMed Central Ltd. 2010
Received: 5 November 2009
Accepted: 6 February 2010
Published: 6 February 2010
Heterotopic pancreatic cancer in the duodenum is a very rare disease. Only twelve cases have been reported worldwide to date. We report a rare case of malignant transformation of heterotopic pancreas (Heinrich type III) in the duodenum with long-term survival of the patient, and review the 12 cases in the literature.
A 75-year-old Japanese man was admitted to our hospital complaining of nausea and vomiting. Endoscopy and upper gastrointestinal contrast study showed marked duodenal stenosis. A pylorus-preserving pancreaticoduodenectomy was performed. Histopathological examination of the surgically resected specimen showed malignant transformation of heterotopic pancreas (Heinrich type III) in the duodenum. The postoperative course was uneventful, and the patient was discharged from the hospital on postoperative day 30. He is well and shows no signs of recurrence at the time of writing, six years after the surgery.
Adenocarcinoma arising within the heterotopic pancreas appears to be rare. It is difficult to obtain a correct diagnosis preoperatively. The management of heterotopic pancreas depends on the presence or absence of symptoms. If the patient is asymptomatic or benign, conservative treatment with regular follow-up is recommended. When the patient is symptomatic or there is a suspicion of malignancy, surgical management with intra-operative frozen section diagnosis is indicated.
Shultz was first to describe heterotopic pancreas in 1727, although histological data was not available until the report by Klob in 1859. Heterotopic pancreas is also called aberrant or ectopic pancreas. Although heterotopic pancreas is rarely found during surgical exploration of the upper part of the abdomen, occurring in less than 0.5% of abdominal laparotomies, heterotopic pancreas is found in 1-2% of autopsies . It is usually asymptomatic, but most diseases affecting the pancreas can occur within these heterotopias. Heterotopic pancreas has been classified by Heinrich . Adenocarcinoma arising within heterotopic pancreas appears to be a rare occurrence, with fewer than 30 cases being reported in the literature worldwide.
Heterotopic pancreas with its complete absence of both anatomic and vascular connections to the main pancreas is considered to result from the altered development of one of the two primitive pancreatic anlagens that normally fuse to form the uncinate-head and body-tail of the normal gland. This alteration of embryonic growth results in an ectopic rest being dropped from the dorsal pancreatic rudiment. It is then left to develop in a site away from the usual location of the body and tail of the pancreas, therefore, heterotopic pancreas arose in surrounding or adjacent organs.
Heterotopic pancreas has been reported by Busarb et al.  to occur at a large number of sites within the abdomen, involving not only the hollow viscera but also the omentum, mesentry, and spleen. Most cases reported in the literature occurred in duodenum (29.3%), stomach (27.4%), jejunum (15.7%), ileum (5.9%), Meckel's diverticulum (5.1%), and gallbladder (2.7%). The majority of reports refer to the stomach, because the chance of endoscopic observation and organ resection is much more frequent than in other organs. Heterotopic pancreas is mainly single, though multiple in some cases, with the size of the nodule ranging from 0.1 cm to 2.0 cm but usually smaller than 1 cm . The classic radiologic and endoscopic features of heterotopic pancreas are awell-circumscribed intramural nodule, locating mainly in the submucosal layer, with a central umbilication that may be the site of ductal drainage to the mucosal surface.
The majority of cases appear to be asymptomatic, and most symptoms attributable to heterotopic pancreas include non-specific ones, such as epigastric pain, nausea, and vomiting. Heterotopic pancreas may undergo acute and chronic pancreatitis, bleeding and perforation, gastric outlet obstruction, pseudocyst formation, intussusceptions and, rarely, malignant transformation.
Heterotopic pancreas has been classified into three types, according to its constitutional components by Heinrich . Namely, Type I consists of typical pancreatic tissues with acini, ducts, and islet cells similar to those seen in normal pancreas. Type II is composed of pancreatic tissues with ducts and acini. Islet cells are absent. Type III is composed of pancreatic tissue with large numbers of ducts and few acini. Class III is characterized by an absence of islet cells, rare acini, and numerous ducts, some of which are cystically dilated. Some Class III cases lack acinar tissue entirely. Furthermore, heterotopic pancreas is similar to so-called adenomyoma. Adenomyoma, first described histologically by Maugnus-Alsleben , is characterized by cystic dilation and poorly differentiated ducts surrounded by proliferating numerous smooth-muscle bundles without acini or islets in the submucosal layer. Heinrich Class III is virtually almost the same as adenomyoma in this regard, and thus adenomyoma can be considered a subtype of heterotopic pancreas .
Review of cases of carcinoma of duodenal heterotopic pancreas
Bookman MR (1932)
Potet F (1970)
Leger L (1976)
Ikeda R (1980)
Al Jitawi SA (1984)
Wakahara T (1988)
Franke U (1989)
body weight loss
Senmaru N (1995)
Waku T (1996)
Nam JY (2003)
Kaneko K (2006)
pain and jaundice
Tison C (2007)
Our case was discovered not at an earlier stage, but as an advanced cancer that simulated a T3 pancreatic cancer during operation. Mainly, it is because the tumor existed in an area ranging from the submucosal layer to the proper muscular layer, proliferating invasively from the proper muscular layer to the serosa without any clinical symptoms for a long time. We performed pancreaticoduodenectomy with thorough dissection of the regional lymph nodes, and considered the possibility of it having a malignant character though without intraoperative frozen section diagnosis. Although prognosis of malignant heterotopic pancreas is poor in general, our case is alive and well with no sign of recurrence six years after the surgery.
The management of heterotopic pancreas depends on the presence of symptoms. If the patient is asymptomatic or benign, conservative treatment with regular follow-up is recommended. When the patient is symptomatic or the heterotopic is suspected of being malignant, surgical management with intraoperative frozen section diagnosis is needed.
Written consent was obtained from the patient for publication of the case report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of the journal.
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