title: Pseudomyxoma peritonei originating from an intestinal duplication.
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).
authors.affiliation: Department of Imaging and Pathology, University Hospitals Leuven, Minderbroedersstraat 12, 3000 Leuven, Belgium.
authors.forename: Julie
authors.initials: J
authors.lastname: Lemahieu
authors.forename: André
authors.initials: A
authors.lastname: D'Hoore
authors.forename: Stijn
authors.initials: S
authors.lastname: Deloose
authors.forename: Raf
authors.initials: R
authors.lastname: Sciot
authors.forename: Philippe
authors.initials: P
authors.lastname: Moerman
references.citation: Pathol Int. 2006 May;56(5):272-8
references.pmid: 16669876
references.citation: Am Surg. 2011 Feb;77(2):233-4
references.pmid: 21337887
references.citation: World J Surg Oncol. 2012 Apr 10;10:55
references.pmid: 22490125
references.citation: Ann Surg. 1988 Aug;208(2):184-9
references.pmid: 3401062