Pseudomyxoma Peritonei Originating from an Intestinal Duplication

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...

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Main Authors: Julie Lemahieu, André D'Hoore, Stijn Deloose, Raf Sciot, Philippe Moerman
Format: Article
Language:English
Published: Wiley 2013-01-01
Series:Case Reports in Pathology
Online Access:http://dx.doi.org/10.1155/2013/608016
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author Julie Lemahieu
André D'Hoore
Stijn Deloose
Raf Sciot
Philippe Moerman
author_facet Julie Lemahieu
André D'Hoore
Stijn Deloose
Raf Sciot
Philippe Moerman
author_sort Julie Lemahieu
collection DOAJ
description 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).
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spelling doaj-art-ce8cf2cf920048b39e5000dd5b8d44992025-08-20T03:19:38ZengWileyCase Reports in Pathology2090-67812090-679X2013-01-01201310.1155/2013/608016608016Pseudomyxoma Peritonei Originating from an Intestinal DuplicationJulie Lemahieu0André D'Hoore1Stijn Deloose2Raf Sciot3Philippe Moerman4Department of Imaging and Pathology, University Hospitals Leuven, Minderbroedersstraat 12, 3000 Leuven, BelgiumDepartment of Surgery, University Hospitals Leuven, BelgiumDepartment of Pathology, Jan Yperman Ziekenhuis, Ieper, BelgiumDepartment of Imaging and Pathology, University Hospitals Leuven, Minderbroedersstraat 12, 3000 Leuven, BelgiumDepartment of Imaging and Pathology, University Hospitals Leuven, Minderbroedersstraat 12, 3000 Leuven, BelgiumAlimentary 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).http://dx.doi.org/10.1155/2013/608016
spellingShingle Julie Lemahieu
André D'Hoore
Stijn Deloose
Raf Sciot
Philippe Moerman
Pseudomyxoma Peritonei Originating from an Intestinal Duplication
Case Reports in Pathology
title Pseudomyxoma Peritonei Originating from an Intestinal Duplication
title_full Pseudomyxoma Peritonei Originating from an Intestinal Duplication
title_fullStr Pseudomyxoma Peritonei Originating from an Intestinal Duplication
title_full_unstemmed Pseudomyxoma Peritonei Originating from an Intestinal Duplication
title_short Pseudomyxoma Peritonei Originating from an Intestinal Duplication
title_sort pseudomyxoma peritonei originating from an intestinal duplication
url http://dx.doi.org/10.1155/2013/608016
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