Citations 2
Outcomes for Sinonasal Undifferentiated Carcinoma (SNUC): An International Multi-Center Retrospective Cohort Study
Liu, Jacklyn , Takahashi, Yoko , Rehman, Umar , Turri-Zanoni, Mario, Mattavelli, Davide, Counsell, Nicholas , Ferrari, Marco, Rampinelli, Vittorio , Vermi, William , Lombardi, Davide , Saade, Rami , Park, Ki Wan , Emanuel, Oscar , Schartinger, Volker Hans , Franchi, Alessandro , Facco, Carla, Sessa, Fausto, Battocchio, Simonetta , Eriksen, Patrick René Gerhard , Haddouchi, Mohamed El, Maragliano, Roberta , Lelkaitis, Giedrius , Saraswathula, Anirudh, KAUR, RAMAN PREET, Mydlarz, Wojciech K. , Ishii, Masaru, Dave, Manas, Fenton, Tim R. , Lim, Alison E. , Okhovat, Saleh , Elegio, Gyleen, Pouvreau, Pierre, Thariat, Juliette, Digue, L., Ferrand, François-Régis, Costes-Martineau, Valerie, Castain, Claire, Kermadec, H. De , O’Neill, James Paul, Lacy, Peter D., Vaz, Francis, O’Flynn, Paul, Howard, David J., Stimpson, Paul, Wang, S, Royle, Gary , Steele, Christopher, Jay, A., Carnell, Dawn , Forster, Martin D., Thomson, David, Buchwald, Christian von, Lllorente, Jose Luis, Hermsen, Mario A., Jurmeister, Philipp , Capper, David, Gallia, Gary L. , Js, Tay, Mohyeldin, Ahmed, Fernandez-Miranda, Juan, LE, Q, West, Robert B., Patel, Z M, Hwang, P H, Facchetti, Fabio , Nicolai, Piero, Ferrarotto, Renata, Phan, Jack, Bossi, P., Castelnuovo, P., Moya-Plana, Antoine, Verillaud, B., Garnis, Cathie , Thamboo, Andrew , Olawuni, Felicia O., Moore, E.J., Choby, Garret M., Lal, Devyani, Akhave, Neal S. , Bell, Diana , Su, Shirley Y., Lund, Valerie J., London, Nyall R. , HANNA, Ehab, Lechner, Matt, Hwang, Peter H. , Thariat, Juliette , Phan, Jack , Le, Quynh‐Thu , Patel, Zara M. , Franchi, Alessandro , Su, Shirley Y. , Lund, Valerie J. , Lechner, Matt , Castelnuovo, Paolo , Ferrari, Marco , Turri‐Zanoni, Mario , Mattavelli, Davide , Moya‐Plana, Antoine , Rehman, Umar , Turri-Zanoni, Mario , Mattavelli, Davide , Ferrari, Marco , Schartinger, Volker H , Maragliano, Roberta , Lelkaitis, Giedrius , Fenton, Tim R , Thariat, Juliette , Ferrand, Francois-Regis , Howard, David J , Carnell, Dawn , Forster, Martin D , von Buchwald, Christian , Hermsen, Mario , Tay, Joshua K , Le, Quynh-Thu , Patel, Zara , Hwang, Peter H , Facchetti, Fabio , Phan, Jack , Bossi, Paolo , Castelnuovo, Paolo , Moya-Plana, Antoine , Olawuni, Felicia, Su, Shirley Y , Lund, Valerie J , Lechner, Matt , Sessa, Fausto , Bendtsen, Simone K. , Jakobsen, Kathrine Kronberg , Saraswathula, Anirudh , Ramanathan, Murugappan , Ishii, Masaru, Dupin, Charles Louis , Digue, Laurence, Costes‐Martineau, Valérie , Castain, Claire , Hintze, Justin , O'Flynn, Paul E., Steele, Christopher D. , Woods, Robbie , Llorente Pendás, José Luis , Capper, David , Nayak, Jayakar V. , Nicolai, Piero , Vérillaud, Benjamin , Choby, Garret , Hanna, Ehab Y.
Cancers | January 26, 2026 | Volume: 18 | Issue: 3 | Pages: 366
Background: Sinonasal undifferentiated carcinoma (SNUC) is an extremely rare, high-grade, and aggressive tumor of the sinonasal tract. Due to the rarity of this malignancy, current treatment guidelines are based on small and often/mainly single-center retrospective datasets. In the absence of a universally accepted standard of care for SNUC, treatment approaches vary across countries and institutions, reflecting real-world clinical practice. The primary aim of this study was to describe real-world treatment and outcomes for patients with confirmed SNUC. Methods: This was an international, multi-center, retrospective, observational cohort study that pooled patients into the largest SNUC dataset to date. Fifteen centers were enrolled to contribute data, including seven from Europe, four from the United States, three from the United Kingdom, and one from Canada. In the absence of a universally accepted standard of care for SNUC, treatment approaches varied across countries and institutions, reflecting real-world clinical practice. Patients included were those with histologically confirmed SNUC who were treated between 1997 and 2021. Results: This study yielded 485 patients treated for SNUC. The median age at diagnosis was 55.6 years (IQR: 44.5–67.6), and 63.7% were male. Most cases presented at advanced stages, with 70.8% as T4a or T4b. Overall survival (OS) outcomes were available for 412 patients, with a median follow-up of 26.0 months. The 5- and 10-year OS were 47.2% (95% CI: 40.8–53.3%) and 39.6% (95% CI: 32.5–46.6%), respectively. Advanced age, dichotomized T-stage (T4a/b vs. T1–3), M-stage, and orbital involvement were significant poor prognostic factors on univariable analysis (p’s < 0.01). On multivariable analysis, orbital involvement (HR: 2.73, 95% CI: 1.42–5.27, p = 0.003) and distance metastasis stage (HR: 3.00, 95% CI: 1.25–7.21, p = 0.014) were both independently associated with worse OS. Conclusions: This observational study presents the largest multi-center cohort analysis of SNUC to date, providing new insights into prognostic factors for a rare cancer treated at global centers of excellence. Orbital involvement and the presence of metastases are candidate independent risk factors associated with poorer OS.
Molecular and clinical stratification of astroblastomas: Three distinct fusion-defined groups informing risk-adapted treatment strategies
Federico, Aniello , Schmitt-Hoffner, Felix , Fonseca, Adriana , Geisemeyer, Neal, Bruckner, Katharina , Mauermann, Monika, Sill, Martin , Stichel, Damian , Sturm, Dominik , Schüller, Ulrich , Tauziede-Espariat, Arnault, Capper, David, Abdullaev, Zied , Selt, Florian , Williamson, Lane, Miele, Evelina, Snuderl, Matija, Brandner, Sebastian, Lugt, Jasper van der , Bunt, Jens , Kolenova, Alexandra, Wilson, Yelena , Weintraub, Lauren , Spiegl-Kreinecker, Sabine, Aistleitner, Barbara, Zapotocky, Michal, Ramaswamy, Vijay, Korshunov, Andrey, Jones, Barbara C. , Kjaersgaard, Mimi, Kranendonk, Mariëtte E.G. , Haberler, Christine, Packer, R., Jäger, Natalie, Sahm, Prof Felix, Koster, J., Aldape, Kenneth, Hoff, Katja von , Gojo, Johannes , Kool, M., Varlet, Pascale , Capper, David , Selt, Florian , Antonelli, Manila , Brandner, Sebastian , Bunt, Jens , Raghunathan, Aditya , Ramaswamy, Vijay , Haberler, Christine , von Deimling, Andreas , Sahm, Felix , Aldape, Kenneth , Gojo, Johannes , Kool, Marcel , Von Hoff, Katja , Geisemeyer, Neal, Tauziède-Espariat, Arnault , Capper, David , Schrimpf, Daniel , Donson, Andrew M. , Miele, Evelina , Snuderl, Matija , Łastowska, Maria , Kramm, Christof Maria , Kolenová, Alexandra , Raghunathan, Aditya , Hansford, Jordan R. , Spiegl‐Kreinecker, Sabine , Baroni, Lorena , Zápotocký, Michal , Ramaswamy, Vijay , Korshunov, Andrey , Kjærsgaard, Mimi , Haberler, Christine , Deimling, Andreas von , Sahm, Felix , Pfister, Stefan M. , Kool, Marcel
Neuro-Oncology | December 18, 2025 | Volume: 28 | Issue: 4 | Pages: 1005-1019
Abstract Background Astroblastomas are rare brain tumors predominantly affecting children and young adults, for which molecular subtypes and clinical management remain undefined. Methods We analyzed tumor samples, molecular profiles, and clinical data from 200 patients, classified as “Astroblastoma, MN1-altered” under WHO criteria, using DNA methylation profiling, DNA/RNA profiling/sequencing, and survival analyses. Results DNA methylation analyses identified 3 groups: Group A (n = 143, characterized by MN1::BEND2 fusions, predominantly supratentorial location, with striking female predominance and favorable survival); Group B (n = 37, epigenetically and transcriptionally closely related to Group A, but characterized by EWSR1::BEND2 fusions, with spinal and infratentorial locations and poor prognosis); and Group C (n = 20, epigenetically and transcriptionally distinct, characterized by MN1::CXXC5 fusions, exclusively supratentorially located, with favorable survival). Progression-free and overall survival were significantly shorter in Group B (5-year PFS 14%; 10-year OS 54%) compared to A (5-year PFS 47%; 10-year OS 89%) and C (5-year PFS 75%; 10-year OS 89%). Radiotherapy improved PFS in Group B (hazard ratio 0.25), while no clear benefit was identified for Groups A and C. Conclusions Astroblastoma, MN1-altered, comprises 3 molecularly and clinically distinct groups, characterized by different fusion genes, including those without MN1. These new insights, including the identification of potential predictive biomarkers like 14q/16q loss, provide a framework for the development of risk-stratified therapeutic approaches. Importantly, we identified a molecularly defined high-risk group that benefits from radiation therapy. Our findings redefine Astroblastoma as a molecularly diverse tumor type, propose a refined classification, support the development of risk-adapted therapeutic strategies and provide a rational standard of care.