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Pseudomyxoma peritonei originating from an intestinal duplication

Julie Lemahieu1, André D'Hoore, Stijn Deloose

  • 1Department of Imaging and Pathology, University Hospitals Leuven, Minderbroedersstraat 12, 3000 Leuven, Belgium.

Case Reports in Pathology
|September 12, 2013
PubMed

Insights

This case report details an extremely rare instance of pseudomyxoma peritonei (PMP) originating from a small intestinal duplication. The study highlights a unique cause for this uncommon condition, emphasizing the importance of considering rare origins.

Area of Science:

  • Gastroenterology
  • Surgical Pathology
  • Oncology

Background:

  • Alimentary tract duplications are rare congenital anomalies, typically presenting in childhood.
  • Pseudomyxoma peritonei (PMP) is an uncommon condition, usually arising from appendiceal neoplasms.
  • Malignant transformation of intestinal duplications is exceptionally rare.

Purpose of the Study:

  • To report an extremely unusual case of PMP originating from a small intestinal duplication.
  • To describe the clinical presentation, diagnostic process, and histopathological findings.
  • To elucidate the pathogenesis of PMP in this unique context.

Main Methods:

  • Case report of a 67-year-old woman with abdominal pain.
  • Explorative laparoscopy revealing diffuse peritoneal implants.
  • Histopathological examination of resected cystic lesion and peritoneal implants.
  • Surgical resection of a mesenteric cystic lesion.

Main Results:

  • Diagnosis of low-grade PMP (disseminated peritoneal adenomucinosis) was established.
  • No primary tumor was initially identified.
  • A resected small intestinal duplication cyst revealed a low-grade villous adenoma.
  • The adenoma led to a mucocele, causing noninvasive mucinous peritoneal implants.

Conclusions:

  • A small intestinal duplication with a villous adenoma can be a rare primary source of PMP.
  • This case expands the known etiologies of PMP.
  • Understanding rare origins is crucial for accurate diagnosis and management of PMP.

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