Intimal sarcoma:A clinicopathological analysis of two cases and review of literature
XU Yan-lan
HUANG Yu-xin
ZHANG Jun
Abstract：Objective To investigate the clinicopathological,immunophenotypic and genetic characteristics of intimal sarcoma(IS).Methods Two cases of IS diagnosed in the Affiliated Hospital of Guizhou Medical University were collected from May 2020 to May 2024.Clinicopathological information of the patients was collected and analyzed.Results Both cases are female with non-inductive chest distress and palpitations.In Case 1,thrombosis formation was predisposed in the ultrasonic result.In Case 2,myxoma was predisposed in imaging result.Under microscope,the neoplasm was composed of spindle and epithelioid heterotypic cells with varied amount of hemorrhage and necrosis.Myxoid change and tumor giant cells were observed.IHC staining showed that MDM2 was diffusely expressed in the nuclei of the neoplastic cells with scattered CDK4 expression.CK,EMA,CK7,Bcl-2,CK5/6,p16,CD34,CD31,Caldesmon,Calretinin and WT-1 positive expression were not observed in both cases.MDM2 gene amplification was identified in both cases using FISH examination.Conclusion Intimal sarcoma is a high-grade malignant mesenchymal tumor with poor prognosis.It commonly involves the large arteries of the systemic and pulmonary circulation,as well as the heart.The abnormal amplification of MDM2 gene is the determinative indication in the IS pathological diagnosis.Surgery is mainly the therapeutic regimen for IS.
Keywords：Intimal sarcomaMDM2Malignant mesenchymal tumorImmunohistochemistryFluorescence in situ hybridization
Publication Date：2025-03-28
Online Publishing Date：2025-08-15(First online date of this platform, not the publication date of the document)
Pages：5( 287-290,296 )