Diagnosis of brain metastatic alveolar soft part sarcoma and application value of anti-translocation factor E3 antibody in it

Abstract

Objective To analyze the clinical pathological characteristics and prognosis of brain metastatic alveolar soft part sarcoma (ASPS) and observe the expression of anti-translocation factor E3 (TFE3) antibodies to evaluate the application value of anti-TFE3 antibodies in the diagnosis of ASPS.Methods The clinical data,imaging characteristics and morphology of 8 patients with brain metastatic ASPS were collected and analyzed; comparative study of the immunohistochemical expressions of TFE3 antibodies,rhabdomyosarcoma markers (MyoDl),synaptophysin (Syn),Chromogranin A (CgA) and S-100 was performed in the 8 cases of ASPS and 10 cases of paraganglioms.Results Eight patients with brain metastatic ASPS were found primary foci before their 30 years,and primary tumors were detectable in lower extremity in 4.Follow-up of 7 patients,6 were alive after craniotomy for 77,56,41,31,20 and 4 months.MR imaging showed single or multiple masses with clear boundary,peripheral edema,and inhomogeneous enhancement.Immunohistochemistry showed TFE3 antibodies positive expressions in all the 8 cases,MyoD1,desmin and CgA were positive in 4 and NSE in 3.PAS positive crystalline substances were noted in 4 cases.CgA and Syn were positively expressed in all 10 cases of paraganglioma.Conclusions Brain metastases from ASPS show prevalence for the male.TFE3 antibody has important value in diagnosis of ASPS.TFE3 antibodies,Syn protein may help to differentiate ASPS from paraganglioma. Key words: Alveolar soft-part sarcoma; Brain metastases; Translocation factor E3; Paraganglioma; Immunohistochemistry