Molecular Profiling for Precision Medicine in Adrenocortical Carcinoma: Real-World Data From the French Network ENDOCAN-COMETE.

Authors

Simon Lemaire, Grégoire Marret, Sophie Moog, Arnaud Jannin, Magalie Haissaguerre, Alexandre Lugat, Christophe Sajous, Sandrine Laboureau, Florence Eas, Helene Lassolle, Delphine Drui, Antoine Tabarin, Christine Do Cao, Julien Hadoux, Rossella Libé, Antoine Italiano, Christophe Massard, Éric Baudin, Ségolène Hescot

Published in

JCO precision oncology. Volume 10. Issue 11. Pages e2500839. Epub Nov 13, 2026.

Abstract

Adrenocortical carcinoma (ACC) is a rare tumor with poor prognosis. Precision medicine and molecularly matched treatment (MMT) represent a new opportunity in oncology.
We conducted a retrospective study of patients with advanced ACC within the French ENDOCAN COMETE network from 2009 to 2022 who have been providing access to molecular profiling (MP). We investigated the benefits of the MMT strategy in terms of response according to RECIST, median progression-free survival (PFS), overall survival (OS), and growth modulation index (GMI).
We enrolled 78 patients with ACC who underwent MP of the primary tumor in 41 patients. Most patients had metastasis at diagnosis, with at least one GRAS criterion. P53 alteration and activation of the Wnt/β-catenin pathway were the most frequent genetic alterations. No mutations were found in 20.5% of patients' tumors. The microsatellite instability phenotype was present in two cases. The median OS from the date of first metastasis was 33.3 months (95% CI, 24.7 to 56.2) with most patients receiving platinum-based chemotherapy and mitotane as first-line therapy. Twelve patients were treated with MMT as a second- or third-line therapy. An objective response rate was observed in two patients with a complete response under immunotherapy. The median PFS with MMT was 2.5 months. The mean GMI was 1.02, above 1.3 in three patients (4%).
Molecular alteration-based precision medicine is associated with potential antitumor benefits in a limited subset of patients with ACC.

PMID:
43024844
Bibliographic data and abstract were imported from PubMed on 14 Nov 2026.

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