[Peritoneal pseudomyxoma: an overview emphasizing pathological assessment and therapeutic strategies]

Peggy Dartigues1, Sylvie Isaac2, Laurent Villeneuve3

  • 1Département d'anatomie pathologique, institut Gustave-Roussy, 94805 Villejuif, France.

Annales De Pathologie
|March 18, 2014
PubMed

Insights

Pseudomyxoma peritonei, a rare condition often linked to appendiceal neoplasms, involves gelatinous ascites and mucinous tumors. Treatment combining surgery and chemotherapy significantly improves patient prognosis.

Area of Science:

  • Gastroenterology and Oncology
  • Pathology
  • Surgical Oncology

Context:

  • Pseudomyxoma peritonei (PMP) is a rare clinical entity characterized by gelatinous ascites and peritoneal mucinous tumor deposits.
  • It is most commonly associated with mucinous appendiceal neoplasms.
  • PMP presents unique diagnostic and therapeutic challenges due to its low incidence.

Purpose:

  • To provide a comprehensive overview of pseudomyxoma peritonei.
  • To summarize its pathology, etiology, and pathomechanisms.
  • To discuss current therapeutic strategies and the role of specialized networks.

Summary:

  • PMP involves mucinous tumor spread within the peritoneum, often originating from the appendix.
  • The WHO 2010 classification distinguishes between low-grade and high-grade mucinous carcinoma.
  • Multimodal treatment including cytoreductive surgery and hyperthermic intraperitoneal chemotherapy (HIPEC) has improved outcomes.

Impact:

  • Highlights the importance of specialized centers and multidisciplinary collaboration for optimal PMP management.
  • Emphasizes the improved prognosis with current therapeutic approaches.
  • Aims to guide clinicians and pathologists in diagnosing and treating this rare malignancy.