Medline ® Abstracts for References 27,29,44,52,53
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.
Patients with pseudomyxoma peritonei associated with disseminated peritoneal adenomucinosis have a significantly more favorable prognosis than patients with peritoneal mucinous carcinomatosis.
Ronnett BM, Yan H, Kurman RJ, Shmookler BM, Wu L, Sugarbaker PH
BACKGROUND: Pseudomyxoma peritonei (PMP) is a poorly understood condition characterized by disseminated intraperitoneal mucinous tumors, often with mucinous ascites. The term PMP has been applied historically as a pathologic diagnostic term to both benign and malignant mucinous neoplasms that produce abundant extracellular mucin, resulting in a variable and poorly predictable prognosis. A recent study reported a pathologic classification that separated patients into prognostically distinct groups, but the follow-up was relatively short.
METHODS: Long-term follow-up data were analyzed for a previously reported series of 109 patients with PMP to examine the prognostic utility of a pathologic classification system that divided patients into three groups: disseminated peritoneal adenomucinosis (DPAM), peritoneal mucinous carcinomatosis (PMCA), and peritoneal mucinous carcinomatosis with intermediate or discordant features (PMCA-I/D). Patients whose tumors were classified 25 DPAM (n = 65 patients) had disease that was characterized by histologically bland to low-grade adenomatous mucinous epithelium associated with abundant extracellular mucin and fibrosis, oftenwith an identifiable appendiceal mucinous adenoma that was the source of the peritoneal lesions. Patients whose tumors were classified 25 PMCA (n = 30 patients) had disease that was characterized by peritoneal lesions that displayed the cytologic and architectural features of mucinous carcinoma associated with extracellular mucin, often with an identifiable invasive mucinous adenocarcinoma of the gastrointestinal tract. Patients whose tumors were classified 25 PMCA-I (n = 11 patients) had peritoneal lesions that combined the features of DPAM and PMCA derived from well differentiated mucinous adenocarcinomas associated with adenomas. Patients whose tumors were classified 25 PMCA-D (n = 3 patients) had markedly atypical appendiceal adenomas associated with peritoneal lesions similar to PMCA.
RESULTS: Patients with DPAM had 5-year and 10-year survival rates of 75% and 68%, respectively (mean follow-up, 96 months; median follow-up, 104 months). Patients with PMCA and PMCA-I/D had a significantly worse prognosis, with 5-year and 10-year survival rates, respectively, of 50% and 21% for PMCA-I/D (mean follow-up, 58 months; median follow-up, 51 months) and 14% and 3% for PMCA (mean follow-up, 27 months; median follow-up, 16 months; P = 0.0001).
CONCLUSIONS: The term PMP should be used only as a clinical descriptor for patients who have the syndrome of mucinous ascites accompanied by a characteristic distribution of peritoneal mucinous tumors with the pathologic features of DPAM. DPAM should be used as a pathologic diagnostic term for patients with the bland peritoneal mucinous tumors associated with ruptured appendiceal mucinous adenomas and PMP. These patients should not be diagnosed with carcinoma, because they have disease that is distinct pathologically and prognostically from PMCA.
Department of Pathology, The Johns Hopkins Hospital, Baltimore, Maryland 21231, USA. email@example.com
Pseudomyxoma peritonei: a review of 62 cases.
van Ruth S, Acherman YI, van de Vijver MJ, Hart AA, Verwaal VJ, Zoetmulder FA
Eur J Surg Oncol. 2003;29(8):682.
AIM: Pseudomyxoma peritonei (PMP) is a rare disease characterized by the abundance of mucus in the abdomen without extra-peritoneal growth.
METHODS: Our patients with PMP have been treated with cytoreduction and hyperthermic intraperitoneal chemotherapy since 1996. The clinical and histopathological features of PMP and the relation of these features with disease-free interval and survival were assessed.
RESULTS: Sixty-two patients with PMP (24 M/38 F) were studied. Adenomatous mucosal changes were present in 31 patients. In females, the ovaries were normal in 5 patients and pseudomyxoma ovarii was present in 20 patients. Patients with minimal atypia and with 1% focal proliferation or less (n=38) had a better survival (p=0.0008) than those with more focal proliferation (n=14).
CONCLUSION: In most patients with PMP the appendix is affected; in females the ovaries are usually also involved. Focal proliferation appears to be a prognostic factor.
Department of Surgical Oncology, The Netherlands Cancer Institute/Antoni van Leeuwenhoek Hospital, Amsterdam, The Netherlands. firstname.lastname@example.org
Systematic review of the Sugarbaker procedure for pseudomyxoma peritonei.
Bryant J, Clegg AJ, Sidhu MK, Brodin H, Royle P, Davidson P
Br J Surg. 2005;92(2):153.
BACKGROUND: Pseudomyxoma peritonei, a rare progressive disease process within the peritoneum, is characterized by an abundance of mucinous fluid; if left untreated, the condition is fatal. The aim of this article is to assess the clinical effectiveness and costs of the Sugarbaker procedure for pseudomyxoma peritonei.
METHODS: A systematic review of the literature up to April 2004 was undertaken, with modelling of costs.
RESULTS: Five retrospective case-series reports met the inclusion criteria. Survival after operation was approximately 95 per cent at 2 years and 60-68 per cent at 10 years, with 41-52 per cent of patients having no evidence of disease at the end of follow-up. A Monte Carlo simulation model estimated the marginal cost for one patient over a maximum of 5 years to be about pound 9700 (standard deviation pound 1300).
CONCLUSION: Evidence of the effectiveness of the Sugarbaker procedure for pseudomyxoma peritonei is limitedin quantity and quality, but suggests there may be some benefit for patients. The marginal cost of the operation is about pound 9700, provided that trained and experienced staff are available to perform the procedure.
Southampton Health Technology Assessments Centre, Wessex Institute for Health Research and Development, University of Southampton, Southampton, UK. J.S.Bryant@soton.ac.uk
Peritoneal carcinomatosis from appendiceal cancer: results in 69 patients treated by cytoreductive surgery and intraperitoneal chemotherapy.
Sugarbaker PH, Zhu BW, Sese GB, Shmookler B
Dis Colon Rectum. 1993;36(4):323.
Sixty-nine patients presenting over a 10-year period with peritoneal carcinomatosis from appendiceal cancer were treated with cytoreductive surgery combined with intraperitoneal chemotherapy. The three-year survival is 89.5 percent in patients (38/69) with pseudomyxoma peritonei, 34.5 percent in patients (25/69) with cystadenocarcinoma, and 38.1 percent in patients (6/69) with adenocarcinoma (P<0.01). In this study, a classification of residual disease following the cytoreductive surgery was used. The prognosis of the patients with minimal residual disease was better than that of those with moderate or gross disease, showing a 91.6 percent three-year survival compared with 47.8 percent and 20 percent, respectively (P<0.01). The patients without lymphatic or hematogenous metastases had a better three-year survival than those with metastases (75.1 percent vs. 28.6 percent; P<0.01). These findings suggest that peritoneal carcinomatosis from appendiceal cancer can be treated with long-term disease-free survival. The patients with low malignant potential cancer, complete cytoreduction, and no metastases showed the most effective disease control.
Cancer Institute, Washington Hospital Center, Washington, D.C. 20010.