[Clinical Features and Management of Pseudomyxoma Peritonei Appendiceal Origin].

Abstract

Pseudomyxoma peritonei(PMP)is a rare clinical entity that typically arises from the rupture of a low-grade appendiceal mucinous neoplasm(LAMN), resulting in accumulation of gelatinous mucinous material throughout the peritoneal cavity. Despite its low-grade histological features, PMP can be clinically aggressive due to continuous mucin production, progressive peritoneal dissemination, and organ compression. Patients often present with abdominal distension, palpable mass, or gastrointestinal symptoms, and the disease may occasionally be discovered during surgery for presumed appendicitis or ovarian tumors. Computed tomography(CT)is the key imaging modality for diagnosis, revealing characteristic findings such as scalloping of visceral surfaces, omental cake, and mucinous ascites. Although the majority of cases are appendiceal in origin, misdiagnosis as ovarian tumors is common, especially in women. Diagnostic pitfalls include failure to recognize mucinous nature on imaging and insufficient suspicion preoperatively. The standard of care is complete cytoreductive surgery(CRS)combined with hyperthermic intraperitoneal chemotherapy(HIPEC). This aggressive but potentially curative approach requires substantial expertise and should ideally be performed at specialized centers. In cases where PMP is unexpectedly encountered during surgery, initial management should avoid extensive procedures that may compromise future CRS. Referral to a specialized institution is strongly recommended. In this review, we discuss the clinical pathophysiology, diagnostic strategies, and treatment approaches for PMP, with particular emphasis on the importance of early recognition and appropriate surgical referral pathways to optimize patient outcomes.