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

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.