Pseudomixoma peritonei: a rare entity

Pseudomyxoma peritonei (PMP) is a rare syndrome characterized by progressive accumulation of mucinous material within the peritoneal cavity, most commonly secondary to appendiceal mucinous neoplasms. Clinical presentation is often vague, including abdominal pain, distension, weight loss, and nonspecific gastrointestinal symptoms, which may delay diagnosis. Imaging plays a central role: ultrasound may reveal septated ascites, while contrast-enhanced computed tomography (CT) is the modality of choice, typically showing low-attenuation mucinous collections, peritoneal implants, and scalloping of visceral surfaces. Definitive diagnosis requires histopathological confirmation. We report the case of a 36-year-old man presenting with recurrent abdominal pain and significant weight loss. CT imaging revealed extensive abdominal occupation by mucinous material; diagnostic laparoscopy confirmed low-grade PMP, likely of appendiceal origin. The patient underwent complete cytoreductive surgery (CRS) according to Sugarbaker, followed by hyperthermic intraperitoneal chemotherapy (HIPEC). Postoperative complications, including pneumothorax and infectious collections, were successfully managed. Follow-up imaging demonstrated no evidence of recurrence. CRS combined with HIPEC remains the gold standard for PMP, offering favorable long-term outcomes, particularly in low-grade disease. This case highlights the importance of multidisciplinary management and structured radiological follow-up.