Mucoepidermoid carcinoma of the salivary glands revisited with special reference to histologic grading and CRTC1/3-MAML2 genotyping.
Adolescent
Adult
Biomarkers, Tumor
/ genetics
Carcinoma, Mucoepidermoid
/ genetics
Female
Gene Fusion
Germany
Humans
Male
Middle Aged
Neoplasm Grading
Neoplasm Staging
Predictive Value of Tests
Progression-Free Survival
Registries
Salivary Gland Neoplasms
/ genetics
Time Factors
Trans-Activators
/ genetics
Transcription Factors
/ genetics
Young Adult
CRTC1-MAML2
Gene fusion
Head and neck neoplasms
Mucoepidermoid carcinoma; Neoplasm grading
Observer variation
Journal
Virchows Archiv : an international journal of pathology
ISSN: 1432-2307
Titre abrégé: Virchows Arch
Pays: Germany
ID NLM: 9423843
Informations de publication
Date de publication:
Nov 2021
Nov 2021
Historique:
received:
12
03
2021
accepted:
19
06
2021
revised:
08
06
2021
pubmed:
8
7
2021
medline:
23
11
2021
entrez:
7
7
2021
Statut:
ppublish
Résumé
Mucoepidermoid carcinoma (MEC) is the most common carcinoma of the salivary glands. Here, we have used two large patient cohorts with MECs comprising 551 tumors to study clinical, histological, and molecular predictors of survival. One cohort (n = 167), with known CRCT1/3-MAML2 fusion status, was derived from the Hamburg Reference Centre (HRC; graded with the AFIP and Brandwein systems) and the other (n = 384) was derived from the population-based Cancer Registry of North Rhine-Westphalia (LKR-NRW; graded with the AFIP system). The reliability of both the AFIP and Brandwein grading systems was excellent (n = 155). The weighted kappa for inter-rater agreement was 0.81 (95% CI 0.65-0.97) and 0.83 (95% CI 0.71-0.96) for the AFIP and Brandwein systems, respectively. The 5-year relative survival was 79.7% (95% CI 73.2-86.2%). Although the Brandwein system resulted in a higher rate of G3-MECs, survival in G3-tumors (AFIP or Brandwein grading) was markedly worse than in G1/G2-tumors. Survival in > T2 tumors was markedly worse than in those with lower T-stage. Also, fusion-negative MECs had a worse 5-year progression-free survival. The frequency of fusion-positive MECs in the HRC cohort was 78.4%, of which the majority (86.7%) was G1/G2-tumors. In conclusion, the AFIP and Brandwein systems are useful in estimating prognosis and to guide therapy for G3-MECs. However, their significance regarding young age (≤ 30 years) and location-dependent heterogeneity of in particular G2-tumors is more questionable. We conclude that CRTC1/3-MAML2 testing is a useful adjunct to histologic scoring of MECs and for pinpointing tumors with poor prognosis with higher precision, thus avoiding overtreatment.
Identifiants
pubmed: 34231055
doi: 10.1007/s00428-021-03146-x
pii: 10.1007/s00428-021-03146-x
pmc: PMC8572836
doi:
Substances chimiques
Biomarkers, Tumor
0
CRTC1 protein, human
0
CRTC3 protein, human
0
MAML2 protein, human
0
Trans-Activators
0
Transcription Factors
0
Types de publication
Comparative Study
Journal Article
Multicenter Study
Langues
eng
Sous-ensembles de citation
IM
Pagination
975-985Subventions
Organisme : German Federal Ministry of Education and Science (BMBF)
ID : 01ER1704
Organisme : Swedish state under the agreement between the Swedish government and the country councils (ALF)
ID : ALFGBG-721711
Informations de copyright
© 2021. The Author(s).
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