High chromosomal instability in adenocarcinoma of the ileum arising from multifocal gastric heterotopia with gastritis cystica profunda
- First Online:
- Cite this article as:
- Schaefer, IM., Schüler, P., Enders, C. et al. Med Oncol (2011) 28: 1023. doi:10.1007/s12032-010-9604-2
Adenocarcinoma of the small intestine arising from heterotopic gastric mucosa is extremely rare. In this report, we present the case of a 68-year-old woman who complained of abdominal pain, weight loss and subileus. Gross examination of resected small bowel revealed multiple flat polypous lesions with cysts in the ileal submucosa, one of which containing an ulcerated, stenosing tumour. On microscopic examination, an adenocarcinoma of the ileum arising from multifocal gastric heterotopia with secondary gastritis cystica profunda was diagnosed. Comparative genomic hybridization of the adenocarcinoma revealed chromosomal gains at 1q, 3q, 5p, 8q, 11p, 12p, 13q and losses at Xp, 4q, 8p, 10p, 14q, 17p, 20p, compatible with a high degree of genomic instability.
KeywordsGastric heterotopiaGastritis cystica profundaAdenocarcinomaIleumComparative genomic hybridization
Despite the fact that the small intestine accounts for 90% of mucosal surface of the gastrointestinal tract, adenocarcinomas of the small bowel make up less than 5% of all gastrointestinal malignancies . Risk factors include male gender, age, non-Asian ethnicity, familial adenomatous polyposis (FAP), hereditary non-polyposis colorectal cancer (HNPCC) and Crohn’s disease . We report a case of multifocal gastric heterotopia with gastritis cystica profunda in the ileum and an adenocarcinoma in one of these lesions. Adenocarcinoma arising from heterotopic gastric mucosa is very infrequent. To date, only one similar case in the jejunum has been reported .
Heterotopic gastric mucosa is fairly infrequent but may be encountered throughout the gastrointestinal tract, usually as “inlet patch” in proximal oesophagus, duodenum or Meckel’s diverticulum . The peculiar observation in our case was not only the multifocal gastric heterotopia but the combination with gastritis cystica profunda. The latter is characterized by hyperplastic and cystic dilation of pseudopyloric glands with extension into the submucosa or even muscular wall of the stomach. Its aetiology is still unclear. Ischaemia, chronic inflammation, mucosal injury, the effects of surgery and suture material are suggested to play a role in pathogenesis . Gastritis cystica profunda is a benign lesion, although a possible precancerous nature has been hypothesized .
Malignant transformation of heterotopic gastric mucosa is described for oesophagus, gallbladder and adenomyosis of the stomach. In our case, adenocarcinoma developed within the heterotopia, giving rise to the question of gastric or intestinal origin. The immunostaining of CK7+/20− multifocal heterotopic gastric mucosa complementary to CK7−/20+ ileal mucosa favours a gastric origin of the tumour, although intestinal mucosa may sometimes be CK7+/CK20− as well.
Our CGH results revealed a high degree of genomic instability in the adenocarcinoma, but except for gains on 8q and losses on 4q, they differed from previous findings on intestinal type gastric cancer .
The present case highlights the fact that heterotopic gastric mucosa of the small intestine possesses malignant potential and requires close clinical monitoring when diagnosed.
We thank Prof. Jörg Männer from the Department of Anatomy and Embryology, University Medical Center, Göttingen, Germany, for his scientific support.