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J Thorac Oncol. 2010 Jun;5(6):898-906.

Primary sarcoma of the mediastinum: a report of 16 cases referred to the British Columbia Cancer Agency.

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1
British Columbia Cancer Agency, Vancouver Island Centre, Victoria.

Abstract

BACKGROUND:

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

METHODS:

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).

RESULTS:

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.

CONCLUSION:

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.

PMID:
20521357
[Indexed for MEDLINE]
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