P2.15-11 Favorable Long Term Survival After Initially Palliative Resection for a Giant Primary Rib Osteosarcoma with Severe Mediastinal Shifting

Abstract

Osteosarcoma is the most common malignancy of the bone with high morbidity and mortality. Treatment of choice is chemotherapy (CHT) followed by surgery and adjuvant CHT. Primary rib osteosarcoma is a rare disease. We present a case of a young patient with a giant primary rib osteosarcoma undergoing palliative resection. A 29-year-old woman presented with dyspnea and pain in the left chest wall in August 2012. Computed tomography (CT) showed a large lesion destructing the 3rdleft rib with extension to the left upper lobe. Biopsy revealed an osteoplastic osteosarcoma and staging examinations showed no distant metastases (DM). CHT by the EURAMOS-1-protocol was started but was discontinued by the patient after 1 cycle due to side effects. 1.5 years later without any treatment or follow up, the patient was re-admitted with massive thoracic pain, severity of dyspnea and in a very limited general condition. CT showed a major tumor progression with severe mediastinal shift and total atelectasis of the left lung. Due to extreme large tumor size and lack of treatment alternatives, surgery in palliative intent was performed: clamshell incision, complete resection of the tumor including chest wall reconstruction. The postoperative course was challenging but she recovered well and was discharged after several months with no evidence of disease in combined positron-emission-tomography/CT. Thereafter, the patient again refused any further treatment and checkups. 4.5 years later she presented again with local recurrence in the left chest wall and in excellent overall condition without evidence for DM. Accordingly, a re-thoracotomy, partial resection of ribs 6-9, left diaphragm and pericardium and reconstruction was performed. The postoperative course was without complications and the surgery was well-tolerated. This case report shows an unexpected favorable outcome after resection in palliative intent for a giant primary rib osteosarcoma with severe mediastinal shifting. Currently, 7 years after diagnosis, the patient is free from disease.