Expression of CEA, CA19-9, CA125, and EpCAM in pseudomyxoma peritonei

Abstract

Pseudomyxoma peritonei is a fatal clinical syndrome with mucinous tumor cells disseminated into peritoneal cavity and secreting abundant mucinous ascites. The serum tumor markers CEA, CA19-9, and CA125 are used to monitor pseudomyxoma peritonei remission, but their expression at tissue level has not been well characterized. Herein, we analyzed expression of these proteins and the adenocarcinoma marker EpCAM in 92 appendix-derived pseudomyxoma peritonei tumors by immunohistochemistry. All tumors were found to ubiquitously express CEA and EpCAM. In the majority of the tumors (94.6%), CEA showed polarized immunostaining, but in 5 aggressive high-grade tumors containing numerous signet ring cells, a nonpolarized staining was detected. We found preoperative CEA serum values to correlate with peritoneal cancer index. However, the serum values of the advanced cases with nonpolarized staining pattern were normal, and the patients died within 5 years after diagnosis. Thus, serum CEA measurements did not reflect aggressiveness of these tumors. CA19-9 showed strong immunopositivity in most of the tumors (91.3%), and mutated enzyme FUT3 was demonstrated from the cases showing negative or weak staining. CA125 was infrequently expressed by tumor cells (focal staining in 6.5% of the cases), but in most of the cases (79.3%), adjacent nonneoplastic mesothelial cells showed immunopositivity. As a conclusion, CEA and EpCAM are invariably expressed by pseudomyxoma peritonei tumor cells and could be exploited to targeted therapies against this malignancy.

Glycomic Profiling Highlights Increased Fucosylation in Pseudomyxoma Peritonei

“…However, CA19 -9 cannot be used in patients whose FUT3 is mutated (47,48), because other FUT enzymes do not compensate for the lack of ␣1,4-fucosylation activity of this enzyme. In our previous study, we demonstrated that 9% (8/92) of PMP cases showed impaired CA19 -9 expression because of FUT3 mutation (26). In our present study, we found that all these cases showed intense staining of sLe x , which can be synthesized by FUT 3-7 enzymes.…”

“…The patients received no preoperative oncological treatments. To avoid fucosyltransferase 3 (FUT3) mutated cases, occurring in 5-10% of the population, only CA19 -9 immunopositive cases were included based on our previous study (26). All tissue specimens were routinely fixed and embedded in paraffin at the Department of Pathology, HUSLAB, Helsinki University Hospital, between 2007 and 2016, and the study was approved by the Ethics Committee of the Helsinki University Hospital.…”

Pseudomyxoma peritonei induced by low-grade appendiceal mucinous neoplasm accompanied by rectal cancer: a case report and literature review

“…Pirjo Nummela et al found that pseudomyxoma peritonei tumour cells invariably express CEA and EpCAM. These authors propose that CEA and EpCAM could be exploited to develop targeted therapies against this malignancy [55]. Immunohistochemistry can aid in the diagnosis of PMP based on the following features: CK7 (+), CK20 (+), CDX2 (+), MUC2 (+), MUC5AC (+), ER (−) and PR (−) [56–59].…”

Pseudomyxoma extraperitonei in horseshoe kidney masquerading as renal hilar tumor: a case report

“…Some tumor markers have been documented to be valuable in the management of PMP – CEA, CA19.9, and CA125 – albeit these are relatively aspecific 17. Elevated levels of tumor markers indicate more aggressive disease, with increased risk of recurrence, progression, and poorer prognosis.…”