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@ARTICLE{Jurmeister:302800,
author = {P. Jurmeister$^*$ and M. Leitheiser and L. Bergmayr and E.
Payá Capilla and L. H. Mochmann and Y. Zhdanovich and F.
Engelhardt-Schott and K. Schleich and D. Klingler and E.
Chimal and C. M. Focke and G. E. Breimer and I. van Engen
van Grunsven and A. von Deimling$^*$ and D. Capper$^*$ and
F. Klauschen$^*$ and S. Ihrler},
title = {{B}iphasic {M}yoepithelial {C}arcinoma {W}ith 5p/5q {L}oss:
{M}orphomolecular {C}haracterization and {P}rovisional
{D}esignation of a {P}roposed {N}ovel {S}alivary {T}umor
{E}ntity.},
journal = {The American journal of surgical pathology},
volume = {49},
number = {9},
issn = {0147-5185},
address = {Philadelphia, Pa.},
publisher = {Lippincott Williams $\&$ Wilkins},
reportid = {DKFZ-2025-01340},
pages = {890-900},
year = {2025},
note = {2025 Jul 1;49(9):890-900},
abstract = {Salivary gland tumors are diagnostically challenging due to
major diversity of benign and malignant tumors with enormous
intra-tumorous and inter-tumorous heterogeneity and, hence,
frequently overlapping histologic features. DNA methylation
has greatly enhanced tumor classification in several organs
and led to the identification of previously unrecognized
entities. In a recent study on DNA methylation of salivary
gland tumors, we had identified a group of unclassifiable
tumors. In this study, we characterize this group through an
integrated analysis of clinical, histomorphologic,
immunohistochemical, and molecular features. This group of
12 tumors is characterized by small, clinically benign
appearing tumors with striking female predominance
$(91.7\%),$ the latter not paralleled in other salivary
tumor types. In addition to distinct DNA methylation
profiling, copy number analysis revealed unique alterations
with highly recurrent chromosome 5p/5q loss and frequent
amplification of the MDM2 locus on chromosome 12q.
Whole-exome and transcriptome sequencing detected no
recurrent mutations or fusions. The histomorphologic
features were only moderately distinct, comprising an
obligate, thereby variable admixture of biphasic-tubular and
monophasic-myoepithelial areas, low or absent nuclear
atypia, and minimal proliferation. Frequent invasive
behavior, a solitary lymph node metastasis, and the
molecular alterations, altogether, strongly support
classification as a, presumably low-grade, carcinoma.
Altogether, these findings clearly distinguish this tumor
group from histomorphologically similar tumor entities, in
particular myoepithelial carcinoma, epithelial-myoepithelial
carcinoma, and adenoid cystic carcinoma. We present thorough
arguments that this tumor group represents a distinct
salivary carcinoma entity rather than a variant of an
existing one. We propose the provisional designation
'Biphasic myoepithelial carcinoma with 5p/5q loss' for
discussion.},
keywords = {5p/5q loss (Other) / DNA methylation (Other) / MDM2 (Other)
/ myoepithelial carcinoma (Other) / salivary gland tumors
(Other)},
cin = {MU01 / B300 / HD01 / BE01},
ddc = {610},
cid = {I:(DE-He78)MU01-20160331 / I:(DE-He78)B300-20160331 /
I:(DE-He78)HD01-20160331 / I:(DE-He78)BE01-20160331},
pnm = {312 - Funktionelle und strukturelle Genomforschung
(POF4-312)},
pid = {G:(DE-HGF)POF4-312},
typ = {PUB:(DE-HGF)16},
pubmed = {pmid:40590272},
doi = {10.1097/PAS.0000000000002450},
url = {https://inrepo02.dkfz.de/record/302800},
}