Outcomes and prognostic factors in primary intrathoracic soft tissue sarcoma

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.