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@ARTICLE{Purkait:300164,
author = {S. Purkait and S. Praeger$^*$ and J. Felsberg and D. Pauck
and K. Kaulich$^*$ and M. Wolter and D. Koppstein$^*$ and G.
Reifenberger$^*$},
title = {{S}trong nuclear expression of {HOXB}13 is a reliable
surrogate marker for {DNA} methylome profiling to
distinguish myxopapillary ependymoma from spinal
ependymoma.},
journal = {Acta neuropathologica},
volume = {149},
number = {1},
issn = {0001-6322},
address = {Heidelberg},
publisher = {Springer},
reportid = {DKFZ-2025-00650},
pages = {29},
year = {2025},
abstract = {Spinal ependymoma and myxopapillary ependymoma are the two
most common spinal ependymal tumor types that feature
distinct histological characteristics, genetic alterations
and DNA methylation profiles. Their histological distinction
may be difficult in individual cases and molecular
diagnostic assessment, in particular DNA methylome
profiling, may then be required to assign the correct
diagnosis. Expression of the homeobox gene HOXB13 at the
mRNA and protein levels has been reported as a frequent
finding in myxopapillary ependymoma that may serve as a
diagnostic marker for these tumors. Here, we evaluated the
diagnostic role of HOXB13 immunostaining in 143 spinal
neoplasms, comprising 54 histologically classified
myxopapillary ependymomas, 46 histologically classified
spinal ependymomas, and various other tumor types.
Immunohistochemical results for HOXB13 protein were compared
to molecular findings obtained by bead array-based DNA
methylation and DNA copy number profiling, as well as next
generation gene panel sequencing-based mutational analysis.
Our findings indicate strong nuclear HOXB13 expression as a
reliable diagnostic marker for molecularly confirmed
myxopapillary ependymoma. Moreover, we provide evidence that
differential HOXB13 protein expression is related to
differential HOXB13-associated CpG site methylation in
myxopapillary vs. spinal ependymomas, which can be assessed
by targeted DNA methylation analysis. Taken together,
immunohistochemistry for HOXB13 protein expression and
targeted DNA methylation analysis of HOXB13 represent useful
surrogate approaches that may substitute for DNA methylome
profiling in routine diagnostics and facilitate precise
classification of spinal ependymal tumors. In particular,
strong nuclear HOXB13 immunoreactivity may serve as a novel
diagnostic criterion for the classification of myxopapillary
ependymoma.},
keywords = {Humans / Homeodomain Proteins: genetics / Homeodomain
Proteins: metabolism / Ependymoma: genetics / Ependymoma:
diagnosis / Ependymoma: metabolism / Ependymoma: pathology /
DNA Methylation / Spinal Cord Neoplasms: genetics / Spinal
Cord Neoplasms: diagnosis / Spinal Cord Neoplasms:
metabolism / Spinal Cord Neoplasms: pathology / Biomarkers,
Tumor: genetics / Biomarkers, Tumor: metabolism / Male /
Female / Diagnosis, Differential / Adult / Middle Aged / NF2
mutation (Other) / DNA methylation (Other) / Differential
diagnostics (Other) / HOXB13 (Other) / Immunohistochemistry
(Other) / Myxopapillary ependymoma (Other) / Homeodomain
Proteins (NLM Chemicals) / HOXB13 protein, human (NLM
Chemicals) / Biomarkers, Tumor (NLM Chemicals)},
cin = {ED01},
ddc = {610},
cid = {I:(DE-He78)ED01-20160331},
pnm = {899 - ohne Topic (POF4-899)},
pid = {G:(DE-HGF)POF4-899},
typ = {PUB:(DE-HGF)16},
pubmed = {pmid:40137996},
doi = {DOI:10.1007/s00401-025-02866-7},
url = {https://inrepo02.dkfz.de/record/300164},
}