Pseudomyxoma Peritonei Originating from an Intestinal Duplication. (19th August 2013)

• Record Type: Journal Article
• Authors: Lemahieu, Julie; D'Hoore, André; Deloose, Stijn; Sciot, Raf; Moerman, Philippe
• Other Names: Hasebe T. Academic Editor.; Manne U. Academic Editor.; Nagashima Y. Academic Editor.; Wu M. L. Academic Editor.
• Is Part Of: Case reports in pathology. Volume 2013(2013)
• Publication Date: 2013-08-19
• Subjects: Pathology -- Periodicals; Pathology; Pathology; Electronic journals; Periodicals; Periodicals; Case Reports; Fulltext; Internet Resources; Periodicals; 616.07
• DOI: 10.1155/2013/608016
• Languages: English
• ISSNs: 2090-6781

Abstract

Abstract : Alimentary tract duplications are rare congenital anomalies. They most often become symptomatic in childhood and rarely undergo malignant transformation. Pseudomyxoma peritonei (PMP) is an equally uncommon condition, most frequently originating from a primary appendiceal mucinous neoplasm. We report an extremely unusual case of PMP arising from an intestinal duplication. A 67-year-old woman presented with vague upper abdominal pain, and, unexpectedly, explorative laparoscopy revealed diffuse jelly-like peritoneal implants. The histopathological diagnosis of a low-grade PMP or "disseminated peritoneal adenomucinosis" was made. At that moment, no primary tumor was found. During later surgery, a cystic lesion located in the mesentery of the small bowel could be resected. Histologically, the cyst wall clearly showed the concentric layering of a normal bowel wall. The mucosa, however, displayed a diffuse low-grade villous adenoma. We concluded that this histological picture was most consistent with a small intestinal duplication, containing a low-grade villous adenoma. The adenoma caused a mucocele, which subsequently leaked or ruptured, giving rise to noninvasive mucinous peritoneal implants or low-grade PMP, also known as "disseminated peritoneal adenomucinosis" (DPAM).