Journal Article Ahead of print
PubMed: 41719753 DOI: 10.1016/j.ejca.2026.116590
Natural history and treatment sensitivity of alveolar soft part sarcoma: A national multicenter analysis from the French sarcoma group.
Eur J Cancer 237:116590
Authors: Eva Kalfala, Alexandra Meurgey, Clémence Hénon, Mehdi Brahmi, Maud Toulmonde, Pascaline Boudou-Rouquette, Emmanuelle Bompas, Nicolas Penel, Nelly Firmin, Florence Duffaud, Isabelle Desmoulins, Agnès Ducoulombier, Mathilde Cancel, Sarah Watson, Mohamed Khettab, Tiffany Darbas, Anne Kieffer, Cécile Guillemet, Justine Gantzer, Christophe Perrin, Anne-Laure Genevois, Daniel Orbach, Sylvie Chabaud, Jean-Yves Blay, Armelle Dufresne
Affiliations
- Faculté de Médecine Lyon Sud, Université Claude Bernard Lyon 1, Lyon, France; Centre Léon Bérard, Lyon, France.
- Centre Léon Bérard, Lyon, France.
- Department of medical oncology, Institut Gustave Roussy, Villejuif, France.
- Institut Bergonié, Bordeaux, France.
- Hôpital Cochin-Port Royal, AP-HP, Paris, France.
- Institut de Cancérologie de l'Ouest, Nantes, France.
- Centre Oscar Lambret, Lille, France.
- Department of medical oncology, ICM, INSERM U1194, IRCM, Univ Montpellier, Montpellier, France.
- Hôpital La Timone, AP-HM et Aix-Marseille Université (AMU), Marseille, France.
- Centre Georges-François Leclerc, Dijon, France.
- Centre Antoine Lacassagne, Nice 06100, France.
- Centre Hospitalo-Universitaire Bretonneau, Tours, France.
- Institut Curie Paris, Paris, France.
- CHU de La Réunion, Saint Pierre, France.
- CHU Dupuytren, Limoges, France.
- Institut de cancérologie de Lorraine, Vandœuvre-lès-Nancy, France.
- Centre Henry Becquerel, Rouen, France.
- Institut de Cancérologie Strasbourg Europe, Strasbourg, France.
- Centre Eugène Marquis, Rennes, France.
- SIREDO (Care, Innovation & Research in Childhood, Adolescent & Young-Adult Oncology) Oncology Center, Institut Curie, Paris Sciences et Lettres University, Paris, France.
- Direction Recherche Clinique et Innovation, Centre Léon Bérard, Lyon, France.
- Centre Léon Bérard, Lyon, France.
Abstract
BACKGROUND
Alveolar soft part sarcoma (ASPS) is an ultra-rare sarcoma with limited chemotherapy sensitivity affecting mostly young patients. Tyrosine kinase inhibitors (TKIs) and immunotherapy are therapeutic advances with promising results in this disease. The aim of this study was to describe the characteristics, management and survival of patients with ASPS in France.
PATIENTS AND METHODS
The French NETSARC+ database was screened for all adult patients with ASPS managed in French sarcoma centers between 2010 and 2023.
RESULTS
Sixty-four patients from 18 centers were analyzed. The 5-year overall survival (OS) of the entire cohort was 87.3 % (95 % CI, 75.0-93.8 %). Fifty-six percent of patients had localized tumors at diagnosis; all were treated surgically, and 47 % received perioperative radiotherapy. With a median follow-up of 91 months, their 5-year OS was 96.3 % (95 % CI, 76.5-99.5 %). Half of the patients experienced metastatic recurrence with a median metastasis-free survival of 87.9 months (95 % CI, 29.6-NE). In patients with metastases, the first-line systemic treatment yielded an overall response rate of 7 %, 26 %, and 40 % for chemotherapy, TKIs, and immunotherapy, respectively. The 5-year OS after metastasis diagnosis was 67.7 % (95 % CI: 50.5-80.0 %).
CONCLUSION
Our cohort reinforces existing data on clinical characteristics and demonstrated prolonged survival in both localized and metastatic stages. Among systemic treatments, immunotherapy achieved the highest response rates. We confirmed that ASPS may follow an indolent course in some cases and highlighted recent advances in its management, driven by standardized local treatments in reference centers and innovative systemic therapies.
MeSH
Natural History, Drug Therapy, Immunotherapy, Sarcoma, Neoplasm Metastasis, Diagnosis, Sarcoma, Alveolar Soft Part, Therapeutics, History, Chemotherapy