88P Head, neck and shoulder desmoid-type fibromatosis: characteristics and outcome of these ultra-rare locations
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.
Authors
- Sophie Piperno‐Neumann (ORCID: https://orcid.org/0000-0001-9915-2810)
- A. Italiano
- F. Duffaud
- B. Verret (ORCID: https://orcid.org/0000-0002-8619-8868)
- J. Thery
- P. Boudou Rouquette
- S. Bonvalot
- Thibaud Valentin (ORCID: https://orcid.org/0000-0002-3189-8445)
- Nelly Firmin (ORCID: https://orcid.org/0000-0001-5839-6261)
- Jean‐Yves Blay (ORCID: https://orcid.org/0000-0001-7190-120X)
- Jennifer Wallet (ORCID: https://orcid.org/0000-0002-9521-8701)
- C. Guillemet
- Marie Vanseymortier
- Pascale Dubray‐Longeras
- E. Bompas
- N. Penel
- Jenny Kurtz
- François Bertucci (ORCID: https://orcid.org/0000-0002-0157-0959)
- M. Le Deley
- J. Elices-Diez
Institutions
- Inserm (FR)
- Institut Gustave Roussy (FR)
- Institut de Cancérologie Strasbourg (FR)
- Hôpital Cochin (FR)
- Assistance Publique – Hôpitaux de Paris (FR)
- Maternité Port Royal (FR)
- Institut universitaire du cancer de Toulouse Oncopole (FR)
- Centre Léon Bérard (FR)
- Institut de Cancérologie de l'Ouest (FR)
- Centre Jean Perrin (FR)
- Centre Oscar Lambret (FR)
- Institut Bergonié (FR)
- Laboratoire National Henri Becquerel (FR)
- Institut de Recherche en Cancérologie de Montpellier (FR)
- Institut Paoli-Calmettes (FR)
- Hôpital de la Timone (FR)
- Institut Curie (FR)
Publication Details
- Journal
- ESMO Open
- Published
- 2025-03-01
- DOI
- https://doi.org/10.1016/j.esmoop.2025.104401
- Primary Topic
- Soft tissue tumor case studies
- Type
- article
- Field-Weighted Citation Impact
- 0.00