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Gastrointestinal Oncology Peritoneal Mesothelioma Two Decades of Progress in the Management of a Rare Disease— Peritoneal Mesothelioma Paul H Sugarbaker, MD, FACS, FRCS Medical Director, Center for Gastrointestinal Malignancies, MedStar Washington Hospital Center, Washington, DC, US Abstract Diffuse malignant peritoneal mesothelioma (DMPM) is a rare but aggressive disease with a poor sustained response to systemic chemotherapy. Historically, the median survival has been less than 1 year. The disease rarely disseminates outside of the peritoneal space suggesting that local-regional treatment options may be effective in the long-term control of this malignancy. Establishment of a referral center to concentrate experience with the management of this disease has contributed greatly to progress in its management. Materials and methods: Through a series of Institutional Review Board-approved protocols, a long-standing morbidity/mortality assessment, numerous manuscripts published in the peer-reviewed literature, and participation in numerous national and international workshops, a new standard of care with expectations of long-term survival in a majority of patients has evolved. Results: The surgery for this disease has evolved through a sequence of peritonectomy procedures and visceral resections whose goal is to remove all visible evidence of disease. The first step in successful treatment is a complete cytoreduction. After the cancer resection in the operating room, hyperthermic perioperative chemotherapy (HIPEC) is used. A three-drug protocol combines heat-augmented chemotherapy within the peritoneal space and systemic chemotherapy that is heat-augmented as a continuous infusion intravenously. Catheters are placed for early postoperative intraperitoneal chemotherapy using paclitaxel for 5 days. Also placed at the time of the cytoreductive surgery is an intraperitoneal port for long-term combined intraperitoneal and systemic chemotherapy using cisplatin and pemetrexed for 6 months. Patients having the long-term bidirectional chemotherapy have shown statistically significant improved survival compared with those patients who had surgery alone plus the perioperative chemotherapy. There have been no mortality and the grade 4 adverse events have been prospectively accumulated as 12 %. Conclusions: With continued effort, the surgery and long-term regional chemotherapy for DMPM has continued to improve over 20 years. Currently, a management plan that involves cytoreductive surgery, perioperative chemotherapy, and long-term bidirectional chemotherapy has changed the natural history of this disease. A global registry has been initiated to confirm the benefits of these extended treatments. Keywords Intraperitoneal chemotherapy, cytoreductive surgery, peritonectomy procedures, prognostic indicators, intraperitoneal port Disclosure: Paul H Sugarbaker, MD, FACS, FRCS, has no conflicts of interests to declare. No funding was received in the publication of this article. Open Access: This article is published under the Creative Commons Attribution Noncommercial License, which permits any noncommercial use, distribution, adaptation, and reproduction provided the original author(s) and source are given appropriate credit. Received: January 23, 2015 Accepted: March 16, 2015 Citation: Oncology & Hematology Review, 2015;11(1):67–73 Correspondence: Paul H Sugarbaker, MD, FACS, FRCS, MedStar Washington Hospital Center 106 Irving St., NW, Suite 3900 Washington, DC 20010 US. E: Paul.Sugarbaker@medstar.net Progress in Diagnosis The two major symptoms in patients with peritoneal mesothelioma are abdominal pain and abdominal distention. The patient may also show constitutional symptoms, such as weight loss and fever. In women, diffuse malignant peritoneal mesothelioma (DMPM) is frequently diagnosed as an incidental finding. This fact may be responsible for the improved survival as a result of earlier detection of the disease in women. 1 The signs of this disease are ascites, an abdominal or pelvic mass, a new onset abdominal wall hernia, guarding and rebound tenderness especially in the lower abdomen, and a pleural effusion (see Table 1). 2 Tou ch MEd ica l MEdia Unfortunately, a definitive diagnosis still eludes the physician in a significant number of patients. The cytologic examination of fluid removed by paracentesis is negative in approximately 90 % of patients. Therefore, either laparoscopy with tissue biopsy or mini-laparotomy with tissue obtained for biopsy may be necessary for a definite diagnosis. Jacquet and colleagues have cautioned that laparoscopy ports or sites for percutaneous tissue sampling under computed tomography (CT)- guidance should be confined to the midline. 3 When fluid leaks out along these tracts from the peritoneal space, cancer cells will often be trapped within these tissues. The management of lateral laparoscopy port sites through the rectus muscle or needle tracts through the rectus 67