Histology analysis of the mucinous tissue and appendix confirmed diagnosis of pseudomyxoma peritonei from low-grade mucinous adenocarcinoma. Accordingly, open laparotomy was performed and a total of 8 lbs of pseudomyxoma tumour was removed from the abdomen. The family gave necessary consent for debulking surgery. Two surgeons agreed to remove the majority of the tumour mass fearing possible bowel adhesions and obstructions in the operating room. The appendix was found to be ruptured and surrounded by gelatinous material. Extensive pseudomyxoma tumour and ‘omental caking’ were identified on the peritoneal surface during laparoscopic surgery. The patient and his family consented to proceed with laparoscopic surgery to identify the potential source and spread of the tumour in the abdomen. However, no definite source was established. CT-guided biopsy of the abdominal mass was consistent with pseudomyxoma peritonei. Echocardiography was performed, which showed a restrictive pattern of ventricular filling and pericardial thickening suggestive of metastases. The most likely radiological diagnosis was pseudomyxoma peritonei with rare spread into the thorax. Rectal examination and the rest of the physical examination were completely benign. The abdomen was soft, bowel sound was present, there was no guarding or rigidity, tenderness was noted on deep palpation in the right lower and upper abdomen, flanks were full, there was no hepatosplenomegaly and absent lymphadenopathy. On physical examination, vital signs were within normal limits. A review of systems was negative for chest pain, cough, dyspnoea, fever and leg swelling. He denied any history of nausea, vomiting, change in bowel habit, urinary symptoms, weight loss, loss of appetite, melena and umbilical and inguinal herniation. The patient also noticed increase in abdominal girth, which he attributed to dietary habit and lack of exercise. The pain was a continuous 5/10, and unrelated to food and bowel movement. A rarer presentation of this rare disease includes intrathoracic extension of mucinous material at the time of presentation and a few case reports have been published so far.Ī 35-year-old Caucasian man with no significant medical history presented to our outpatient office, with vague diffuse abdominal pain for the last 4 months, which localised more to the right lower abdomen over the past 3 weeks. 3 It can present as intestinal obstruction from bowel adhesion in the late stage of the disease.

4 The most common clinical presentation includes increase in abdominal girth with occasional protrusion of umbilical and inguinal hernias. The patient usually remains asymptomatic for a long time before the diagnosis is performed. 2 3 The mucus producing cells continue to proliferate on the peritoneal surface and mucinous fluid gradually fills the peritoneal cavity, resulting in the characteristic ‘jelly belly’. This usually leads to appendicular rupture, resulting in dissemination throughout the peritoneal cavity in the form of gelatinous deposits. 1 Although carcinoid tumour is the commonest appendicular malignancy, mucinous adenocarcinoma is unique as it causes cystic dilatation of the appendix due to accumulation of copious amounts of gelatinous material. Primary appendiceal malignancy is found in less than 2% of surgically removed appendices and accounts for less than 1% of intestinal malignancies.

Ovarian malignancy is another important source in women. 1 The annual incidence has been reported to be two per million individuals, the majority being secondary to mucinous adenocarcinoma of the appendix. Pseudomyxoma peritonei is an extremely rare disease, characterised by a ‘gelatinous abdomen’ or mucinous ascites from intra-abdominal neoplastic mucin secreting cells proliferating on the peritoneal surface.

Jelly belly pseudomyxoma peritonei