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  5. 88P Head, neck and shoulder desmoid-type fibromatosis: characteristics and outcome of these ultra-rare locations

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Article
en
2025

88P Head, neck and shoulder desmoid-type fibromatosis: characteristics and outcome of these ultra-rare locations

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en
2025
Vol 10
Vol. 10
DOI: 10.1016/j.esmoop.2025.104401

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Jean Yves Blay
Jean Yves Blay

Institution not specified

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N. Penel
S. Bonvalot
J. Elices-Diez
+17 more

Abstract

Results: Cohort1 (253 patients) included undifferentiated pleomorphic sarcoma (UPS), myxofibrosarcoma (MFS), synovial sarcoma (SS), leiomyosarcoma (LMS), dedifferentiated liposarcoma (DDLPS) and malignant peripheral nerve sheath tumor (MPNST).12 % (31/253) exhibited amp of NECTIN4.The UPS group comprised 50 cases with 11 (22%) with amp.For MFS amp was observed in 5 out of 25 cases (20%).For 100 LMS cases, 5 (5%) had the amp.Among 59 DDLPS samples, 8 presented the amp (13%).Only 1 case in the SS group, out of 10 cases analyzed, exhibited amp.No amp was observed in the MPNST group (9 cases).Cohort 2 consisted of 2,922 samples across 38 cancers.The STS collection consisted of 34 cases, including 19 cases of DDLPS, with 16% (3/19) showing amp, and 15 cases of LMS, with an amp rate of 7%.Conclusions: Our findings underscore the need for further investigation of Nectin-4 in STS, including immunohistochemical analysis and clinical trials with ADCs such as Enfortumab-vedotin, emphasizing the potential of cutting-edge therapies to improve outcomes in sarcomas.

How to cite this publication

N. Penel, S. Bonvalot, J. Elices-Diez, A. Italiano, B. Verret, Jenny Kurtz, Pascale Dubray‐Longeras, C. Guillemet, F. Duffaud, François Bertucci, J. Thery, Marie Vanseymortier, E. Bompas, Nelly Firmin, P. Boudou Rouquette, Jennifer Wallet, Sophie Piperno‐Neumann, Thibaud Valentin, Jean Yves Blay, M. Le Deley (2025). 88P Head, neck and shoulder desmoid-type fibromatosis: characteristics and outcome of these ultra-rare locations. , 10, DOI: https://doi.org/10.1016/j.esmoop.2025.104401.

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Publication Details

Type

Article

Year

2025

Authors

20

Datasets

0

Total Files

0

Language

en

DOI

https://doi.org/10.1016/j.esmoop.2025.104401

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