Claudin 6 expression is useful to distinguish myxofibrosarcomas from other myxoid soft tissue tumors.

Myxofibrosarcoma (MFS) is characterized by abundant myxoid stroma, a wide spectrum of cytological atypia, and frequent local recurrence. Some soft tissue tumors with myxoid stroma can histologically mimic MFS, but have different biological behaviors. Here we sought to identify a useful diagnostic marker for MFS. After our analysis of the gene expression dataset from the Gene Expression Omnibus database, we focused on claudin 6 (CLDN 6). The status of CLDN 6 was assessed by immunohistochemistry in 61 samples of MFS and other (benign) myxoid soft tissue tumors (28 myxoma samples, 12 nodular fasciitis samples), 18 low-grade fibromyxoid sarcoma, 30 myxoid liposarcoma, 29 extraskeletal myxoid chondrosarcoma and 27 dedifferentiated liposarcoma with myxoid feature samples. The correlation between the expression of CLDN 6 and clinicopathological findings in MFS was also investigated. Immunohistochemically, high expression of CLDN 6 was observed in approx. 65% of the MFSs, whereas the benign soft tissue tumors did not show a high expression of CLDN 6. The expression of CLDN 6 in the MFS was significantly higher than those of other tumor specimens. Among the MFSs, the high expression of CLDN 6 was correlated with high FNCLCC grades and high AJCC stages. CLDN 6 may be useful for the differential diagnosis from benign myxoid tumor and for predicting the aggressive biological behavior of MFS.