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Pseudomyxoma peritonei. Long-term patient survival with an aggressive regional approach
D B Gough1, J H Donohue, A J Schutt
1Department of Surgery, Mayo Clinic, Rochester, Minnesota.
Objective:
The aims of this study were to analyze the natural history of patients with pseudomyxoma peritonei (PMP), evaluate clinical and pathologic variables as prognostic indicators, and review the authors' experience with different treatments.
Summary Background Data:
PMP is an unusual form of intra-abdominal neoplasm that presents with large amounts of extracellular mucin. Diffuse peritoneal spread occurs in most patients with PMP, and distant metastasis is infrequent. Debulking surgery, radiation therapy (radioisotope and external beam), and chemotherapy (both intraperitoneal and systemic) have all been advocated for optional patient management, but the variability of patients studied, the small patient numbers, and the prolonged course of this disease make the evaluation of results difficult.
Methods:
Fifty-six patients were treated for PMP at the Mayo Clinic between 1957 and 1983. The data were collected retrospectively. Univariate (log-rank test) and multivariate (Cox regression model) analyses were performed for disease recurrence and patient survival.
Results:
Most patients with PMP had carcinomas of the appendix (52%) or ovary (34%). All gross tumor could be removed only in the 34% of patients with limited disease. Although tumor progression occurred in 76% of patients, the 1-, 5-, and 10-year survival rates were 98%, 53%, and 32%, respectively. Adverse predictors of patient survival included weight loss (p = 0.001), abdominal distention (p = 0.004), use of systemic chemotherapy (p = 0.005), diffuse disease (p = 0.038), and invasion of other organs (p = 0.04). Intraperitoneal chemotherapy (p = 0.009) and radioisotopes (p = 0.0043) both were effective in prolonging the recurrence time of symptomatic PMP.
Conclusions:
Although PMP is an indolent disease, aggressive surgical debulking followed by intraperitoneal radioisotopes and/or chemotherapy should be considered because of the diffuse peritoneal involvement.
Insights
Pseudomyxoma peritonei (PMP) is an indolent cancer. Aggressive debulking surgery combined with intraperitoneal radioisotopes and chemotherapy can improve outcomes for patients with PMP.
Area of Science:
- Oncology
- Gastroenterology
- Surgical Oncology
Background:
- Pseudomyxoma peritonei (PMP) is a rare intra-abdominal neoplasm characterized by extensive extracellular mucin production.
- Diffuse peritoneal spread is common in PMP, with infrequent distant metastasis.
- Treatment evaluation is challenging due to patient variability, small cohorts, and disease indolence.
Purpose of the Study:
- Analyze the natural history of pseudomyxoma peritonei (PMP).
- Identify clinical and pathological prognostic indicators for PMP.
- Review treatment experiences for PMP patients.
Main Methods:
- Retrospective analysis of 56 patients treated for PMP at the Mayo Clinic (1957-1983).
- Univariate (log-rank) and multivariate (Cox regression) analyses for survival and recurrence.
- Evaluation of clinical and pathological variables as prognostic factors.
Main Results:
- Appendiceal (52%) and ovarian (34%) carcinomas were the most common PMP origins.
- Complete gross tumor removal achieved in 34% of patients with limited disease.
- Survival rates: 98% (1-year), 53% (5-year), 32% (10-year). Adverse predictors: weight loss, abdominal distention, systemic chemotherapy, diffuse disease, organ invasion.
- Intraperitoneal chemotherapy and radioisotopes prolonged recurrence time in symptomatic PMP.
Conclusions:
- Pseudomyxoma peritonei (PMP) is an indolent malignancy.
- Aggressive surgical debulking is recommended for PMP.
- Intraperitoneal radioisotopes and/or chemotherapy should be considered for diffuse peritoneal involvement in PMP.