Author(s): Sethna K, Mohamed F, Marchettini P, Elias D, Sugarbaker PH
BACKGROUND: Cystic peritoneal mesothelioma is a rare disease associated with a favorable short-term prognosis. Longer follow-up documenting a persistence of symptoms and a high rate of recurrence after debulking surgery along with an uncertain natural history prompt a re-evaluation of prior treatment recommendations. No prior long-term clinical study of these patients is available. METHODS: The experience with five cases of cystic peritoneal mesothelioma, four females and one male, are reviewed. All of these patients were treated with cytoreductive surgery with peritonectomy procedures and heated intraoperative intraperitoneal chemotherapy. CT, pathology and current status were investigated in order to learn more about the natural history of this disease. RESULTS: All patients were symptomatic from abdominal distention and three of the four complained of severe pain. Female patients complained of long periods of recurrent abdominal and pelvic pain poorly managed by oral analgesics. In one patient prolonged conservative management over ten years resulted in transition to an invasive process with extensive lymph nodal metastases. Her prognosis for long-term survival is guarded because of mesothelioma extension into the chest. Disease control of both ascites and pain in the abdomen and pelvis was achieved in all five patients treated with cytoreductive surgery plus intraperitoneal chemotherapy. CONCLUSIONS: Cystic peritoneal mesothelioma should no longer be referred to as "benign" cystic mesothelioma. An aggressive approach with complete disease eradication is the correct goal of treatment. From our experience, cytoreductive surgery to remove all visible tumor and intraperitoneal chemotherapy to control microscopic residual disease will help patients with peritoneal cystic mesothelioma to remain symptom- and disease-free over an extended time period with a single surgical intervention. Disease eradication may prevent the transition to an aggressive and fatal disease process.