Medline ® Abstracts for References 27,35,36
of 'Cancer of the appendix and pseudomyxoma peritonei'
Pseudomyxoma peritonei of appendiceal origin. The Memorial Sloan-Kettering Cancer Center experience.
Smith JW, Kemeny N, Caldwell C, Banner P, Sigurdson E, Huvos A
BACKGROUND: Pseudomyxoma peritonei is a rare clinical entity in which the peritoneal surfaces and omentum are involved with diffuse gelatinous mucinous implants. It originates from ruptured mucinous tumors of the appendix or ovary.
METHODS: The authors examined the experience with 34 patients with pseudomyxoma peritonei seen at Memorial Sloan-Kettering Cancer Center from 1952-1989. Of these, 17 cases were identified to be of appendiceal origin.
RESULTS: All patients underwent celiotomy and cytoreduction. The median survival time from diagnosis was 75 months. It was found that long-term survival can be achieved by operation alone. When conditions do recur, chemotherapy may be valuable.
CONCLUSIONS: Pseudomyxoma peritonei of appendiceal origin is a rare low-grade malignancy. Initial treatment consists of cytoreduction in an attempt to render the patient locally disease-free. Long-term survival can be obtained by operation alone, even if gross disease is present at the end of the procedure. Systemic chemotherapy should be reserved for patients with proven recurrence.
Department of Surgery, Memorial Sloan-Kettering Cancer Center, New York, NY 10021.
Pseudomyxoma peritonei. Long-term patient survival with an aggressive regional approach.
Gough DB, Donohue JH, Schutt AJ, Gonchoroff N, Goellner JR, Wilson TO, Naessens JM, O'Brien PC, van Heerden JA
Ann Surg. 1994;219(2):112.
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.
Department of Surgery, Mayo Clinic, Rochester, Minnesota.
Fernandez RN, Daly JM
Arch Surg. 1980;115(4):409.
Thirty-eight patients with pseudomyxoma peritonei were treated at the M. D. Anderson Hospital, Houston, from 1954 to 1978. The various treatment regimens used have provided actuarial survival rates of 54% at five years and 18% at ten years. Local or regional disease was the cause of death in 68% of patients, and no patient died of metastatic disease. Initial definitive surgery should consist of effective tumor reduction, omentectomy, appendectomy, and, in the female subject, bilateral oophorectomy. Most patients have been treated adjunctively with either fluorouracil or melphalan (L-phenylalanine mustard) depending on the presumed site of origin, but results in a small number of patients treated with either whole abdominal or strip abdominal radiotherapy suggest that this modality may offer improved survival. Treatment with adjunctive radiotherapy alone has provided a five-year survival rate of 75%, compared with 44% for chemotherapy.