Subtypes of different clinical characteristics of pineal mothericular tumor

Pineoblastoma (PB) is a rare childhood brain tumor derived from the pineal glandsThe survival rate of children is not highBryan KLi, of the Department of Blood and Oncology at the University of Toronto Children's Hospital in Canada, and others, studied the clinical and molecular expression spectrum of PB to divide pineal mukatooma into subtypesThe results were published online in Acta Neuropathologica in December 2019studyauthors collected 91 samples of PB or primary neuroblastoma (supratentor or neurogenicormal mal tumor, sPNETs/CNS-PNETs) and 2 cases of moderately differentiated pineal plunblasts, pptids, and clinical data from 29 centers in the Children's Rare Brain Tumor Collaboration GroupBy dividing the DNA methylation map of brain tumors, the authors classified 21 samples as other types of tumors, and the remaining 72 were divided into 5 subtypes: subtype 1, subtype 2, subtype 3, RB subtype and MYC subtypeBy studying the number of copies, exogenous groups, targeted sequencing, and miRNA expression, the authors found that each subtype has its own characteristic expression62% subtype 1 and 100% subtype 2 tumors have miRNA bio-formation genes, DICER1, DROSHA and DGCR8 with harmful loss of purityChanges in the carcinogenic MYC-miR-17/92-RB1 pathway were observed in RB subtypes and MYC subtypes, characterized by RB1 deficiency, miR-17/92 acquisition, and MYC amplificationthe results of the studythe final authors concluded that the pb subtype different clinical characteristics are that the subtype 1-3 occurred in the older children, the median age of 5.2-14.0 years; The median age of children with RB subtype and MYC subtype was 1.3-1.4 years, and the survival rate was lower, with 5 years OS at 37.5% and 28.6%, respectivelyIn all PB children, age less than 3 years at the time of diagnosis, combined metastatic diseases, non-radiation therapy, and chr 16q loss were significant factors in poor prognosisconclusions, PB shows significant molecular heterogeneity, and molecular subtypes are associated with clinical phenotypes and survival ratesAt the same time, determining the molecular subtype of PB can help reveal biological functions and new treatments and reduce the intensity of treatment in patients with good prognosis.