Retroperitoeal malignant peripheral nerve sheath tumors: a clinicopathologic study of 13 cases / 临床与实验病理学杂志

ABSTRACT

Purpose To investigate the clinicopathology and the expression of H3K27me3 in retroperitoeal malignant pe-ripheral nerve sheath tumors (MPNST). Methods The clini-copathology and prognosis of 13 cases MPNST were analyzed. Immunohistochemical analysis was used to detect H3K27me3 in MPNST, synovial sarcoma, dedifferentiated liposarcoma and leiomyosarcoma. Results 13 cases of MPNST were high-grade. The mean diameter of tumors was 20 cm. 2-year survival rate of MPNST was about 60% . 5-year survival rate of MPNST was a-bout 30% . Compared to NF-1 associated and sporadic MPNST (P＜0. 05), the RT-induced MPNST had a poor prognosis. Re-currence and distant metastasis patient had a poor prognosis( P＜0. 05). Age had no significant effect on patient survival. In addition, immunohistochemical staining showed that the expres-sion of H3k27me3 was absent in 11 of 13 cases of MPNST. And compared with the expression of H3K27me3 in synovial sarco-ma, dedifferentiated liposarcoma and leiomyosarcoma, it had statistical significance of that expression in MPNST (P＜0. 05). Conclusion Retroperitoeal MPNST is common at high-grade. Tumor volume is relatively large and prognosis is poor. RT-in-duced, recurrence and distant metastasis play an important role in survival rate of MPNST. H3K27me3 which is more common absence in high-grade could be an effective marker of MPNST.