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Patients with pseudomyxoma peritonei associated with disseminated peritoneal adenomucinosis have a significantly more favorable prognosis than patients with peritoneal mucinous carcinomatosis
Author(s) -
Ronnett Brigitte M.,
Yan Hui,
Kurman Robert J.,
Shmookler Barry M.,
Wu Lee,
Sugarbaker Paul H.
Publication year - 2001
Publication title -
cancer
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 3.052
H-Index - 304
eISSN - 1097-0142
pISSN - 0008-543X
DOI - 10.1002/1097-0142(20010701)92:1<85::aid-cncr1295>3.0.co;2-r
Subject(s) - pseudomyxoma peritonei , medicine , mucinous carcinoma , pathology , adenocarcinoma , mucinous tumor , appendix , ascites , gastroenterology , cancer , pancreas , paleontology , biology
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, often with 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. Cancer 2001;92:85–91. © 2001 American Cancer Society.