Sarcoma arising in the mediastinum is a rare entity. This study evaluates treatment and survival in a cohort of patients with primary mediastinal sarcoma.

Between 1990 and 2006, 16 patients were referred to the British Columbia Cancer Agency with histologically confirmed sarcoma of mediastinal origin. Outcomes examined were disease-free survival (DFS) and overall survival (OS).

There were nine male and seven female patients. The median age at diagnosis was 56 years (range 21-70 years). Thirteen (81%) patients had localized disease, and three (19%) patients had distant metastasis at diagnosis. Surgical resection was performed in 8 of 13 patients with localized disease. At a median follow-up of 18 months, 12 patients have died of disease, three were alive with disease, and one was alive with no evidence of disease. In the entire cohort, median DFS was 12 months (range 0-107 months), and median OS was 18 months (range 1-193 months). Patients who underwent surgery experienced improved DFS (p = 0.054) and OS (p = 0.034). Eastern Cooperative Oncology Group performance status 0 to 1 was associated with improved DFS (p = 0.038) and OS (p = 0.007). The histologic subtype with the longest survival was well-differentiated liposarcoma. Age, gender, tumor location, T and N stage, tumor size, location, and grade were not associated with significant survival differences.

Surgical resection was associated with more favorable survival in patients with mediastinal sarcoma. However, the high rates of progression and mortality underscore the need for more effective adjuvant treatments.