Ewing tumors of the chest wall: Local control and long-term outcomes

Abstract
E21501 Background: Primary sarcomas of the lungs and mediastinum are rare and few data are reported on treatment and results of therapy. Methods: We reviewed our experience from 1980 and 2008 including 31 patients (pts). Pts characteristics: median age 41 (19–80 y), male/female 19/12; symptoms at diagnosis: dyspnoea (42%), chest and shoulder pain (39%), cough (35%), hemophtoae (13%), discomfort (10%). 4 pts had a previous history of mediastinal radiation for Hodgkin's and non-Hodgkin's linfomas. 5 mediastinal tumours were located as follows: 2 in anterior part, 1 in posterior and 2 in the middle (sarcomas of the heart). 26 lung sarcomas presented as a singular mass in 23 cases and as a metastatic disease in 3. Results: In 20/31 cases the tumour was immediately resected (3 mediastinal masses and 17 lung sarcomas). In 8/31 cases only biopsy was possible. FNA was done in 25 pts. Neoadjuvant chemotherapy was performed in 4 cases (3 resected). Resection was complete in 11/23 cases and in 12/23 incomplete. The histology were: peripheral nerve tumour 7, leiomyosarcoma 4, MFH 2, fibrosarcoma 2, liposarcoma 1, angiosarcoma 2, undifferentiated sarcoma 1, solitary fibrous tumour 2, rhabdomyosarcoma 2, synovialsarcoma 2, pulmonary artery sarcoma 1, pleuropolmonary blastoma 1, malignant hemangiopericytoma 1, mixoid chondrosarcoma 1, ectopic osteosarcoma 1, aggressive fibromatosis 1. Only 4 pts received neoadjuvant chemotherapy, 11 adjuvant CT, 5 exclusive CT + RT for inoperable disease. Radiotherapy was completed in 26 pts (21 adjuvant). Local relapse or metastatic progression were recorded in 16/23 pts and 12 received one or more lines of palliative CT. Data about survival are disposable only for the more recently recorded pts (1998–2008: 17 pts). Of these only 8 are alive (2 with disease). Volume of disease, complete resection and grading are the dominant prognostic factors. Conclusions: Primary sarcomas of the lungs and mediastinum have a very severe prognosis. Surgical resection is the fundamental therapy, but in the future the role of neoadjuvant CT will increase. No significant financial relationships to disclose.