Clinical, imaging and molecular characteristics of myeloma.

Intra-spinal cord astrocytoma (IMA) is a rare disease.
characteristics of in-brain assoblastoma are detailed in the 2016 edition of WHO Classification of Central Nervous System Tumors, but the molecular characteristics of myeloma are not yet known.
Leititia Lebrun of the Department of Pathology at erasum University Hospital in Belgium analyzed the clinical, imaging and molecular characteristics of IMA and its relevance to prognosis, the results of which were published online in August 2020 in Acta Neuropathologica Communications.
Study Methodology The authors reviewed clinical data of 61 IMa cases treated from 1989 to 2019 (of which 26 were OFI.grade, 17 cases of CLASS II.class, 3 cases of class III and level IV.grade 12 cases, and 3 cases of low-grade tumors of grade I. or II. level) and used paraffin tissue specimens for targeted second generation sequencing.
the same time, the targeted second-generation sequencing results of 117 in-brain assoblastomas were collected between 2017 and 2019.
IMA's clinical, imaging and molecular characteristics and prognosis are statistically analyzed, and the molecular characteristics of myelin and in-brain assoblastomas are compared.
results: (1) MrI imaging of low-level myeloma (LG IMA) and high-level myeloma (HG IMA) is not significantly different in tumor strengthening and invasive characteristics and therefore cannot be identified by imaging.
(2) single-factor regression analysis showed that the median event-free event-free survival (event-free survival, EFS) of LG IMA patients was significantly better than that of HG IMA patients (67:June);
multi-factor regression analysis showed that high-level tumors were independent predictive factors for poor OS, and high-level tumors and unsealed excision (biopsy only) were independent factors for event-free survival (EFS) poor prognosmation.
(3) In LG IMA, a single-factor analysis showed that EFS in patients with grade I hair cell astromas was significantly better than that in patients with class II. diffuse aseptic cytomoma (median EFS, 115:30), and multifavour analysis showed that unsurmeved excision (biopsy only) was the only independent factor with poor prognosis.
(4) Common gene mutations in IMA include TP53 (13/61, 21%), H3F3A p.K27M (11/61, 18%) and ATRX (10/61,13%).
16% (10/54) of the specimen, the KIAA1549-BRAF fusion gene can be detected.
HG IMA, at least one gene mutation, H3F3A p.K27M (11/15,75%) and TP53 (8/15,53%), was most common, but no KIAA1549-BRAF fusion gene or BRAF p.V600E mutation was found.
(5) KIAA1549-BRAF fusion genes are the most common molecular characteristics in myelin and in the brain I. grade hair cell assoblastoma, but the fusion points are different.
, unlike in-brain II.class astromas, IDH mutation rate of LEVEL II.grade IMA is low (2/16, 12.5%), which is not typical IDH1 p.R132H, IDH2 p.R172H mutation, and the IDH mutation type II.class IMA prognosis is even worse.
TERT initiater mutations are most common in in-brain IV.class astromas, while H3F3A p.K27M mutations (11/12,92%) are almost always found in IV.grade IMa.
(6) incorporating molecular markers into multi-factor regression analysis, it is found that the H3F3A p.K27M mutation is a prognosmatic independent factor of IMA.
conclusions, IMA has different clinical, imaging, and molecular characteristics than in-brain asclusive cell tumors.
common molecular characteristics of in-brain assoblastoma, such as IDH mutations, EGFR, and TRT initiater mutations, the rate of occurrence and effect on prognosis are not applicable to IMA.
molecular characteristics of LG IMA (no typical IDH mutations) are similar to those of childhood diffuse gliomas, while the molecular characteristics of HG IMA (H3F3A p.K27M mutations) are similar to those of midline structural gliomas.
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