Roohani Siyer, Wattakiyanon Nattinee, Catton Charles, Wong Philip, Kirsch David G, Chung Peter, Cypel Marcelo, Wakeam Elliot, Waddell Tom, Ferguson Peter, Wunder Jay, Tsoi Kim M, Shultz David
Department of Radiation Oncology, Radiation Medicine Program, Princess Margaret Cancer Centre, Toronto, ON, Canada.
Department of Radiation Oncology, Charité-Universitätsmedizin Berlin, Corporate member of Freie Universität Berlin and Humboldt-Universität zu Berlin, Berlin, Germany.
J Thorac Dis. 2025 Aug 31;17(8):5499-5508. doi: 10.21037/jtd-2025-582. Epub 2025 Aug 21.
Intrathoracic soft tissue sarcomas are rare and heterogenous tumors with limited data guiding their management. Due to anatomical complexity and delayed diagnosis, treatment can be challenging. This study aimed to assess clinical outcomes and prognostic factors in intrathoracic soft tissue sarcoma patients treated at a high-volume referral sarcoma center.
This bi-institutional retrospective cohort study (2007-2022) analyzed patients with histopathologically confirmed intrathoracic soft tissue sarcomas. Data from two prospective registries were used to assess overall survival (OS), local recurrence (LR), and distant metastasis (DM) via Kaplan-Meier, cumulative incidence, and multivariable Cox regression.
Among 49 patients (median age: 56 years, 55.1% male), 40 (81.6%) had localized and 9 (18.4%) had metastatic disease at diagnosis. The median follow-up was 15.6 months. The median OS was 37.1 months for localized, 5.9 months for metastatic cases. Among localized cases, 55% had recurrences, evenly split between local and distant. LR incidence was 39.4% at 12 months, 53.7% at 24 months; DM incidence was 38.8% and 53.4%. OS was worse in patients >65 years [hazard ratio (HR): 2.69, P=0.04] and with metastatic disease (HR: 4.84, P=0.02). DM risk was higher in grade 2 (HR: 19.38, P=0.03) and grade 3 tumors (HR: 12.65, P=0.02) and those in the lungs, pleura, or diaphragm (HR: 3.56, P=0.046). LR was not associated with grade, margin status, or perioperative therapy. Treatment modality had no impact on DM.
Despite a small sample, this is the largest clinical cohort on intrathoracic soft tissue sarcomas, which carry high local and distant recurrence risks. Worse outcomes were linked to age >65 years, metastatic presentation, higher grade, and lung, pleura, or diaphragm location. Given their rarity, multi-institutional studies are needed to refine risk factors, treatment, and outcomes.
胸内软组织肉瘤是罕见的异质性肿瘤，指导其治疗的数据有限。由于解剖结构复杂且诊断延迟，治疗颇具挑战性。本研究旨在评估在一家大型肉瘤转诊中心接受治疗的胸内软组织肉瘤患者的临床结局和预后因素。
这项双机构回顾性队列研究（2007 - 2022年）分析了经组织病理学确诊的胸内软组织肉瘤患者。来自两个前瞻性登记处的数据用于通过Kaplan - Meier法、累积发病率和多变量Cox回归评估总生存期（OS）、局部复发（LR）和远处转移（DM）。
49例患者（中位年龄：56岁，55.1%为男性）中，40例（81.6%）在诊断时为局限性疾病，9例（18.4%）为转移性疾病。中位随访时间为15.6个月。局限性病例的中位OS为37.1个月，转移性病例为5.9个月。在局限性病例中，55%出现复发，局部复发和远处复发各占一半。12个月时LR发生率为39.4%，24个月时为53.7%；DM发生率分别为38.8%和53.4%。65岁以上患者的OS较差[风险比（HR）：2.69，P = 0.04]，转移性疾病患者的OS也较差（HR：4.84，P = 0.02）。2级（HR：19.38，P = 0.03）和3级肿瘤（HR：12.65，P = 0.02）以及位于肺、胸膜或膈肌的肿瘤患者发生DM的风险更高（HR：
