Acta Neuropathologica: Malignant transformation of polygonal low neurosurgery (PLNTY) in adolescents

PLNTY is a group of inert tumors in children and young people that have MAPK path changes that blend with FGFR3-TACC3, which is also a feature of glioblastoma.
so far, there have been no reports of tumor violations and malignant transformations meeting PNTY diagnostic criteria.
, we report a child patient diagnosed with FGFR3-TACC3 fusion who underwent relapsed glioblastoma-like lesions within 18 months.
a 15-year-old woman with no previous obvious medical history, manifested as a seizure, and an MRI showed a high signal of 1.2cm of uneloidated T1 and T2, enhancing and mineralizing the internal lesions of the left armpit accompanied by mild edema.
the full excision of lesions, pathological histology showed moderate glial cell hyperplation, less protrusion glioma-like cytology, false rose-like vascular surroundings, microcalcification, patchy CD34 expression, all consistent with PLNTY.
the Ki-67 proliferation index was low (-lt;2%, locally as high as 4.2%), and also found a silky split image.
degeneration necrosis of two small lesions was identified.
using MSK-solid fusion analysis, FGFR3 (exon 18) and TACC3 (exon 11) are detected in the box fusion.
does not recommend complementary treatment.
17 months after the excision, the review MRI showed that 4.1cm of the left temporal lobe was accompanied by a partial real cystic lesions associated with extensive edema, which was consistent with local recurrence.
near full cut, the relapsed tumor had no CD34 expression at all and showed a high level of lesions, WHO IV, at the glioblastoma level.
patients received radiation therapy of the nature of the bureau stove, while being given tymoamine, and then maintained treatment, and the final follow-up (about 34 months) did not explicitly relapse.
468 Gene Tumor Sequence Analysis (MSK-IMPACTTM) once again confirms FGFR3-TACC3 fusion, coupled with additional somatic cell changes in TP53, ATRX, PTEN, TEK, and RB1, which are common in high-level gliomas.
addition to the RB1 variant, all changes exist at the time of initial diagnosis.
both tumors showed increases in chromosomes 7 and 20, with general loss of hems.
, FGFR3-TACC3 fusion gliomas have common histological characteristics, but the progression of early low-level malignancies has not been reported.
report highlights the importance of comprehensive genomic analysis to help predict, especially in PLINTY.
Bale, T.A., Sait, S.F., Benhamida, J. et al. Malignant transformation of a polymorphous low grade neuroepithelial tumor of the young (PLNTY). Acta Neuropathol (2020). MedSci Original Source: MedSci Original Copyright Notice: All text, images and audio and video materials on this website that indicate "Source: Mets Medicine" or "Source: MedSci Originals" are owned by Mets Medicine and are not authorized to be reproduced by any media, website or individual, and are authorized to be reproduced with the words "Source: Mets Medicine".
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