Treatment outcomes of sunitinib and/or pazopanib in advanced alveolar soft part sarcoma: A Turkish Oncology Group (TOG) study

Alveolar soft part sarcoma (ASPS) is an ultra-rare, highly angiogenic sarcoma with limited responsiveness to chemotherapy. Tyrosine kinase inhibitors (TKIs) such as sunitinib and pazopanib have shown promising activity, but comparative real-world data remain scarce. This multicenter, retrospective study included 51 patients with metastatic ASPS treated with sunitinib and/or pazopanib between January 2013 and March 2025. Progression-free survival (PFS) and overall survival (OS) were analyzed using Kaplan-Meier methods, and prognostic factors were assessed via Cox regression. First-line TKI therapy achieved significantly longer median PFS compared with chemotherapy (24.9 vs. 5.8 months; p < 0.001), without a significant OS difference. In first-line comparisons, pazopanib demonstrated a trend toward longer PFS (27.2 vs. 15.2 months; p = 0.052) and significantly prolonged OS (45.0 vs. 21.0 months; p = 0.021) versus sunitinib. Across all lines, median PFS and OS were 24.9 and 38.3 months for pazopanib, and 17.7 and 21.0 months for sunitinib, respectively. Multivariate analysis identified prior chemotherapy and liver metastases as independent predictors of shorter PFS. Both agents were well tolerated, with mostly Grade 1-2 toxicities. In one of the largest real-world ASPS cohorts, frontline TKIs provided substantial disease control and prolonged survival compared with chemotherapy, with manageable toxicity. These findings reinforce the role of anti-angiogenic therapy as a preferred first-line approach in advanced ASPS. Prospective studies are warranted to optimize treatment sequencing in this ultra-rare sarcoma.

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Journal
Scientific Reports
Published
2025-11-28
DOI
https://doi.org/10.1038/s41598-025-29276-9
Primary Topic
Sarcoma Diagnosis and Treatment
Type
article
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article

Treatment outcomes of sunitinib and/or pazopanib in advanced alveolar soft part sarcoma: A Turkish Oncology Group (TOG) study

Metin Pehlivan, Şaban Seçmeler, İsmail Ertürk, Murat Ayhan et al.
Scientific Reports
Sarcoma Diagnosis and Treatment
article

Treatment outcomes of sunitinib and/or pazopanib in advanced alveolar soft part sarcoma: A Turkish Oncology Group (TOG) study

Metin Pehlivan, Şaban Seçmeler, İsmail Ertürk, Murat Ayhan, Özlem Aydın İsak, Sabin Göktaş Aydın, Kayhan Ertürk, Saadettin Kılıçkap, Seval Orman, Teoman Şakalar, Reşit Akyel, Ertuğrul Bayram, Mustafa Atçı, Nurgül Yaşar, Zehra Sucuoğlu İşleyen, Mert Başaran, Eyyüp Çavdar, Engin Kut, Serkan Menekşe, Seval Ay, Emir Çelik, Serkan Keskın, Emre Hafızoğlu, Deniz Can Güven, Olçun Ümit Ünal, Oğuzhan Selvi, Hatice Asoğlu Rüzgar
article en

Abstract

Alveolar soft part sarcoma (ASPS) is an ultra-rare, highly angiogenic sarcoma with limited responsiveness to chemotherapy. Tyrosine kinase inhibitors (TKIs) such as sunitinib and pazopanib have shown promising activity, but comparative real-world data remain scarce. This multicenter, retrospective study included 51 patients with metastatic ASPS treated with sunitinib and/or pazopanib between January 2013 and March 2025. Progression-free survival (PFS) and overall survival (OS) were analyzed using Kaplan-Meier methods, and prognostic factors were assessed via Cox regression. First-line TKI therapy achieved significantly longer median PFS compared with chemotherapy (24.9 vs. 5.8 months; p < 0.001), without a significant OS difference. In first-line comparisons, pazopanib demonstrated a trend toward longer PFS (27.2 vs. 15.2 months; p = 0.052) and significantly prolonged OS (45.0 vs. 21.0 months; p = 0.021) versus sunitinib. Across all lines, median PFS and OS were 24.9 and 38.3 months for pazopanib, and 17.7 and 21.0 months for sunitinib, respectively. Multivariate analysis identified prior chemotherapy and liver metastases as independent predictors of shorter PFS. Both agents were well tolerated, with mostly Grade 1-2 toxicities. In one of the largest real-world ASPS cohorts, frontline TKIs provided substantial disease control and prolonged survival compared with chemotherapy, with manageable toxicity. These findings reinforce the role of anti-angiogenic therapy as a preferred first-line approach in advanced ASPS. Prospective studies are warranted to optimize treatment sequencing in this ultra-rare sarcoma.

Scientific ReportsVol. 15(1)
Tekirdağ Namık Kemal University (TR), Manisa Celal Bayar University (TR), Zonguldak Bülent Ecevit University (TR), Fatih University (TR), Fatih University Medical Faculty Hospital (TR), Gülhane Askerî Tıp Akademisi (TR), Memorial Ankara Hospital (TR), Dr Lütfi Kırdar Kartal Eğitim ve Araştırma Hastanesi (TR), Memorial Sisli Hospital (TR), Hacettepe University Hospital (TR), Şanlıurfa Mehmet Akif İnan Eğitim ve Araştırma Hastanesi (TR), Yedikule Teaching Hospital (TR), Izmir Tepecik Eğitim ve Araştırma Hastanesi (TR), Istanbul Eye Hospital (TR), Liv Hospital (TR), İstanbul Kanuni Sultan Süleyman Eğitim ve Araştırma Hastanesi (TR), Afyon Kocatepe University (TR), Cukurova University (TR), Hacettepe University (TR), Istanbul University (TR)
Openalex Percentile: Top 45%
Sarcoma Diagnosis and Treatment
DOIOpen Access PDFOpenAlex
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