Embryonal tumor with multilayered rosettes:a clinicopathological analysis of 9 cases
Purpose To investigate the clinicopathological and molecular of embryonal tumor with multilayered rosettes(ETMR).Methods The clinical data and follow-up data of 9 cases of ETMR were collected,and the expression of Syn,LIN28A,vimentin,GFAP,Olig2,S-100,INI1,H3K27me3,and Ki67 was detected by immunohistochemistry EnVision two-step method.The amplification genes of C19MC were detected by fluorescence in situ hybridization(FISH).And relevant lit-eratures were reviewed.Results There were 6 males and 3 fe-males with 2∶1 of M:F;seven cases were located supratentorial-ly and two cases located subratentorially,one of the case located to the brainstem was resemble of diffuse intrinsic pontine glioma in imaging.Histopathologically,there 9 cases were diagnosed as embryonal tumor with abundant neuropil and true rosettes(four cases),ependymoblastoma(three cases),or medulloepithelio-ma(two cases).Immunohistochemistry showed that LIN28A,Syn and vimentin were positive,GFAP was variable,BRG1,INI1 and H3K27me3 were retained.The Ki67 proliferation index rangeed from 40％to 70％.C19MC amplification were detected in 8 samples by FISH.In all 9 cases,four cases had undergone gross total tumor resection,two cases only subtotal tumor re-moved,one patient was underwent biopsy,two patients were un-known.Seven patients were adjuvant therapy.Four patients had CSF seeded,but without extraneural metastases.The follow-up time ranged from 0 to 36 months.The overall survival(OS)was 36 months and the median survival was 10 months.Eight pa-tients died within 3 years after their initial diagnosis.Conclu-sion ETMR almost occurs in the cerebral hemisphere,and a few cases can occur in the brainstem and show the imaging char-acteristics of DIPG.ETMR have highly aggressive and poor prognosis.The combination of histological,LIN28A immunohis-tochemistry,and C19MC tests is helpful for diagnosis and differ-ential diagnosis.
embryonal tumor with multilayered rosettesLIN28AC19MCprognosis
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