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K. Swalwell
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P3.08 - Poster Session/ Thymoma, Mesothelioma and Other Thoracic Malignancies (ID 226)
- Event: WCLC 2015
- Type: Poster
- Track: Thymoma, Mesothelioma and Other Thoracic Malignancies
- Presentations: 1
- Moderators:
- Coordinates: 9/09/2015, 09:30 - 17:00, Exhibit Hall (Hall B+C)
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P3.08-024 - A Rare Cause of Pancoast Syndrome (ID 1087)
09:30 - 09:30 | Author(s): K. Swalwell
- Abstract
Background:
Alveolar soft part sarcoma (ASPS) is a rare tumour that was initially described by Christopherson et al. in 1952. It accounts for 0.5 - 1% of all soft tissue sarcomas. It most commonly presents in the 15 - 35 year age group with slightly higher incidence in females than males by a ratio of 3:2.
Methods:
We describe a case of a 55-year old woman who had a right mastectomy, adjuvant chemotherapy and radiotherapy 3 years prior to her presentation to our service with a left sided supraclavicular mass that was causing shoulder pain and left upper limb paraesthesia. The patient did not present Horner's syndrome.
Results:
Preoperative PET-CT showed a 5.8 x 4.5 x 5.5cm mass, moderately FDG avid (SUVmax 6.7), arising from the 1st rib. An MRI of the brachial plexus showed no vertebral body involvement, no vessel infiltration, but suspicion of left T1 nerve sheath involvement (Fig 1.). The patient underwent a CT-guided biopsy that classified the lesion as sarcoma. After being discussed at the local sarcoma MDT the patient underwent en-block complete resection of the tumour and of the posterior part of the first rib. Histology showed morphology typical of an ASPS which had arisen from the first rib and extended into the overlying soft tissues. There was nuclear expression of TFE3 and the (PAS+) tumour cells also unusually expressed CD68 and HMB45 as well as vimentin and NSE. Figure 1
Conclusion:
Cases of primary intraosseous ASPS are rare. To our knowledge this is the second description in the medical literature of an ASPS arising in a rib causing Pancoast syndrome. Chemotherapy and radiation have not proven beneficial in the treatment of ASPS, although new drug trials are ongoing. Even with en bloc surgical excision and adjuvant therapy, 5-year overall survival ranges from 20% for patients with metastatic disease to 88% for patients with local disease only at the time of presentation.
