Two Decades of Progress in the Management of a Rare Disease—Peritoneal Mesothelioma

2015 ◽  
Vol 11 (01) ◽  
pp. 67
Author(s):  
Paul H ugarbaker ◽  

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.

2016 ◽  
Vol 12 (10) ◽  
pp. 928-935 ◽  
Author(s):  
Paul H. Sugarbaker ◽  
Kiran K. Turaga ◽  
H. Richard Alexander ◽  
Marcello Deraco ◽  
Mary Hesdorffer

Malignant peritoneal mesothelioma is a rare disease, with approximately 800 new patients per year in the United States. Its natural history is defined by progression restricted to the peritoneal space. In the past, patients with this disease had a limited lifespan of approximately 1 year. Numerous single-institution studies as well as a systematic review have reported median survival of 3 to 5 years with a combination of cytoreductive surgery and hyperthermic perioperative chemotherapy. These markedly improved survival statistics were achieved in experienced centers with 1% mortality and 20% morbidity rates. Data have shown that knowledgeable patient selection is required to prevent patients unlikely to benefit from undergoing these interventions. The conclusion is that patients with peritoneal mesothelioma can experience long-term progression-free survival or significant palliation with cytoreductive surgery plus hyperthermic perioperative chemotherapy. This management plan should be considered the standard of care for properly selected patients with malignant peritoneal mesothelioma at experienced centers around the world.


2018 ◽  
Vol 36 (6_suppl) ◽  
pp. 481-481
Author(s):  
François Audenet ◽  
Nikhil Waingankar ◽  
Bart Ferket ◽  
Scot Anthony Niglio ◽  
Kathryn E. Marqueen ◽  
...  

481 Background: TUR + neoadjuvant cisplatin-based chemotherapy achieves a pathologic complete response in 30-40% of patients with MIBC. Prior studies have demonstrated that long-term survival is possible for a subset of patients with MIBC treated with TUR plus chemotherapy alone, but such analyses have been limited by small sample sizes and poor generalizability. The objective of our study was to describe the characteristics and outcomes of patients managed with this approach using a large national registry. Methods: Within the National Cancer Database (2004-2012), we identified 1,003 patients who were treated with TUR + multi-agent systemic chemotherapy, without radiation, as definitive treatment for cT2-T4aN0M0 urothelial carcinoma of the bladder. Baseline characteristics were compared relative to those of 12,138 patients treated during the same period of time with radical cystectomy ± perioperative chemotherapy. Treatment outcomes were assessed using Kaplan-Meier analysis. Results: Compared to patients who were treated with cystectomy ± perioperative chemotherapy, patients treated with TUR + chemotherapy alone were significantly older (≥75 years old 37% vs. 30%; p < 0.0001), had a higher clinical T stage (cT3: 14% vs. 12%; cT4: 12% vs 7%; p < 0.0001) and were more frequently treated in non-academic facilities (66% vs. 49%; p < 0.0001). There were no significant differences between groups regarding gender, Charlson comorbidity index, insurance type or income/education level. The 30-day and 90-day mortality with TUR + chemotherapy was 0.2% and 4%, respectively. The 5-year survival rate for all patients treated with TUR + chemotherapy was 30.5% (95% CI 26.8, 34.2), and limited to patients with cT2 disease was 33.1% (95% CI 28.7, 37.5). Conclusions: This large real-world cohort representing the continuum of practice settings in the United States confirms that long-term survival is achievable in a subset of patients treated with TUR + chemotherapy alone for MIBC. Refinement of this bladder-sparing approach integrating putative predictive biomarkers of pathologic complete response is now the focus of recently initiated prospective clinical trials.


2003 ◽  
Vol 89 (1) ◽  
pp. 31-35 ◽  
Author(s):  
Kaiumarz Sethna ◽  
Faheez Mohamed ◽  
Pierre Marchettini ◽  
Dominique Elias ◽  
Paul H Sugarbaker

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.


2012 ◽  
Vol 2012 ◽  
pp. 1-9 ◽  
Author(s):  
Paul H. Sugarbaker

Peritoneal metastases (PM) are a common presentation for patients with metastatic colorectal cancer (CRC), and the median survival of patients with PM is approximately one year. In a majority of patients, the disease remains limited to the peritoneal cavity. Therefore, investigators have applied cytoreductive surgery (CRS) and heated perioperative chemotherapy (HIPEC) as a standard approach for selected patients with PM from CRC. These investigators have demonstrated a very promising long-term survival in a subset of patients with a limited amount of isolated peritoneal metastatic disease. This paper presents the data that supports CRS and HIPEC as a treatment option for CRC patients with PM. These results of treatment are compared and contrasted to the results that can be expected with systemic chemotherapy alone.


2015 ◽  
Vol 156 (45) ◽  
pp. 1824-1833 ◽  
Author(s):  
Árpád Illés ◽  
Ádám Jóna ◽  
Zsófia Simon ◽  
Miklós Udvardy ◽  
Zsófia Miltényi

Introduction: Hodgkin lymphoma is a curable lymphoma with an 80–90% long-term survival, however, 30% of the patients develop relapse. Only half of relapsed patients can be cured with autologous stem cell transplantation. Aim: The aim of the authors was to analyze survival rates and incidence of relapses among Hodgkin lymphoma patients who were treated between January 1, 1980 and December 31, 2014. Novel therapeutic options are also summarized. Method: Retrospective analysis of data was performed. Results: A total of 715 patients were treated (382 men and 333 women; median age at the time of diagnosis was 38 years). During the studied period the frequency of relapsed patients was reduced from 24.87% to 8.04%. The numbers of autologous stem cell transplantations was increased among refracter/relapsed patients, and 75% of the patients underwent transplantation since 2000. The 5-year overall survival improved significantly (between 1980 and 1989 64.4%, between 1990 and 1999 82.4%, between 2000 and 2009 88.4%, and between 2010 and 2014 87.1%). Relapse-free survival did not change significantly. Conclusions: During the study period treatment outcomes improved. For relapsed/refractory Hodgkin lymphoma patients novel treatment options may offer better chance for cure. Orv. Hetil., 2015, 156(45), 1824–1833.


2021 ◽  
pp. 67-72
Author(s):  
Sung Jin Oh

Liver metastasis from gastric cancer has a very poor prognosis. Herein, we present two cases of liver metastases (synchronous and metachronous) from advanced gastric cancer. In the first case, the patient underwent radical subtotal gastrectomy. Liver metastases occurred 6 months after surgery while the patient was receiving adjuvant chemotherapy, but two hepatic tumors were successfully removed by radiofrequency ablation (RFA). In the second case, liver metastases occurred 15 months after surgery for gastric cancer. The patient also received RFA for one hepatic tumor, and other suspicious metastatic tumors were treated with systemic chemotherapy. Although these case presentations are limited for the efficacy of RFA treatment with systemic chemotherapy for hepatic metastases from gastric cancer, our findings showed long-term survival (overall survival for 108 and 67 months, respectively) of the affected patients, without recurrence. Therefore, we suggest that RFA treatment with systemic chemotherapy could be an effective alternative treatment modality for hepatic metastases from gastric cancer.


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