Pseudomyxoma peritonei in the pleural cavity

PURPOSE: Pseudomyxoma peritonei is a rare disease characterized by mucinous ascites and associated with ruptured mucocele, cystadenoma, and low-grade carcinoma arising from the appendix, ovaries, or colon. Metastases and extraperitoneal involvement are extremely rare events. METHOD: This is a case report of a patient with pseudomyxoma peritonei with pleural involvement. RESULTS: A 38-year-old male patient with a pseudomyxoma peritonei from appendiceal origin underwent an extensive cytoreduction procedure. During the operation pleural involvement was noted. This was later confirmed by thoracoscopy. An expectant policy was followed until the patient became symptomatic with progressive disease in the abdomen and both pleural cavities. With systemic chemotherapy (5-fluorouracil and leucovorin), a good clinical response was obtained, and the patient was alive with stable disease 2.5 years after the first diagnosis. CONCLUSIONS: Involvement of the pleural cavity by pseudomyxoma peritonei is rare and carries an unfavorable prognosis. Whenever possible, the same guidelines as for intra-abdominal disease should be followed: extensive cytoreductive procedures with local and/or systemic chemotherapy. In our patient we hope to achieve a prolonged palliation with systemic chemotherapy.