Intestinal Obstruction Secondary to Pseudomyxoma Peritonei: A Case Report

Introduction Pseudomyxoma Peritonei (PMP) is a rare neoplastic syndrome characterized by disseminated cancerous mucinous lesions and/or mucinous ascites in the intraperitoneal area, peritoneal surface and omentum, principally related to appendiceal neoplasm. Patients are usually asymptomatic or paucisymptomatic, complaining nonspecific symptoms, including abdominal pain and distension, anorexia and nausea. PMP slow and uninterrupted growth leads to significant abdominal distension and complications such as intestinal obstruction and dyspnea. Preoperative diagnosis is based on ultrasonography and computed tomography; nonetheless, explorative laparotomy and histopathological exam provide the ultimate diagnosis. Cytoreductive surgery (CRS) associated with heated intraperitoneal chemotherapy (HIPEC) is considered a potentially curative treatment in selected patients. Case presentation We present an unusual case of a male patient, aged 58 years-old, hospitalized in the Surgery Department of “Jazzolino” Hospital of Vibo Valentia (Italy) for intestinal obstruction secondary to intraperitoneal dissemination of PMP. The patient, who did not present common risk factors for abdominal district tumors, had been complaining of dyspespisa and important abdominal distension for some months, until he arrived at the emergency room with widespread abdominal pain, closed hive, batracian abdomen and recurrence of a previous umbilical hernia. His blood test resulted normal; CEA = 10,2 ng/dL (range 0,00 to 5,10); Ca 19.9 within limits. Abdominal ultrasound and CT scans suggested PMP on an appendicular base. The patient underwent explorative laparotomy with findings of ascites, abundant gelatinous material in the subhepatic site and in the Douglas fissure, disintegration of the epiplons, covered by the same gelatinous material, and of the wall of the ileal loops, incorporated and with a gelatinous-like consistency. Samples of the ascitic effusion and gelatinous material were taken for cytological exam, while biopsies of the omentum were sent for histological exam, which confirmed the suspected diagnosis. Once stabilised, the patient was referred to a specialised centre for further treatment. Conclusions PMP is rare and difficult to detect. Imaging guides preoperative diagnosis, although the final diagnosis is reached through explorative laparotomy and histopathology exam. Complete CRS associated with HIPEC is considered to be the most suitable approach in selected patients. As an advanced disease and/or inadequate treatment lead to poor prognosis, patients diagnosed with PMP should be referred to specialised centres for further care.