Alveolar soft part sarcoma: progress toward improvement in survival? A population-based study

Autor: Tomohiro Fujiwara, Eiji Nakata, Toshiyuki Kunisada, Toshifumi Ozaki, Akira Kawai
Jazyk: angličtina
Rok vydání: 2022
Předmět:
Zdroj: BMC Cancer, Vol 22, Iss 1, Pp 1-12 (2022)
Druh dokumentu: article
ISSN: 1471-2407
DOI: 10.1186/s12885-022-09968-5
Popis: Abstract Background Alveolar soft part sarcoma (ASPS) is a rare histological subtype of soft-tissue sarcoma, which remains refractory to conventional cytotoxic chemotherapy. We aimed to characterize ASPS and investigate whether the oncological outcome has improved over the past decade. Methods One hundred and twenty patients with newly diagnosed ASPS from 2006 to 2017, identified from the Bone and Soft-Tissue Tumor Registry in Japan, were analyzed retrospectively. Results The study cohort comprised 34 (28%) patients with localized ASPS and 86 (72%) with metastatic disease at presentation. The 5-year disease-specific survival (DSS) was 68% for all patients and 86% and 62% for localized and metastatic disease, respectively (p = 0.019). Metastasis at presentation was the only adverse prognostic factor for DSS (hazard ratio [HR]: 7.65; p = 0.048). Patients who were > 25 years (80%; p = 0.023), had deep-seated tumors (75%; p = 0.002), and tumors > 5 cm (5–10 cm, 81%; > 10 cm, 81%; p
Databáze: Directory of Open Access Journals
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