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Pseudomyxoma peritonei in the pleural cavity: report of a case
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.
Insights
Pseudomyxoma peritonei rarely involves the pleura. This case report details a patient treated with cytoreduction and chemotherapy, achieving stable disease for 2.5 years.
Area of Science:
- Oncology
- Gastroenterology
- Thoracic Surgery
Background:
- Pseudomyxoma peritonei (PMP) is a rare malignancy characterized by mucinous ascites.
- It typically originates from appendiceal, ovarian, or colonic sources.
- Extraperitoneal spread, such as pleural involvement, is exceptionally uncommon.
Observation:
- This case report describes a 38-year-old male with PMP of appendiceal origin.
- Pleural involvement was incidentally discovered during cytoreductive surgery.
- Thoracoscopic confirmation and subsequent symptomatic progression in abdominal and pleural disease were noted.
Findings:
- The patient underwent extensive cytoreduction for PMP with appendiceal origin.
- Systemic chemotherapy with 5-fluorouracil and leucovorin resulted in a significant clinical response.
- The patient achieved stable disease and survived for 2.5 years post-diagnosis.
Implications:
- Pleural involvement in PMP is a rare event with a generally poor prognosis.
- Treatment should align with intra-abdominal disease management, emphasizing cytoreductive surgery and chemotherapy.
- Systemic chemotherapy can achieve prolonged palliation in select PMP cases with pleural spread.
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