iCREATE: imaging features of primary and metastatic alveolar soft part sarcoma from the EORTC CREATE study
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RESEARCH ARTICLE
Open Access
iCREATE: imaging features of primary and metastatic alveolar soft part sarcoma from the EORTC CREATE study Naami Charlotte Mcaddy1* , Hind Saffar1, Saskia Litière2, Pieter Jespers2, Patrick Schöffski3,4 and Christina Messiou1,2,4
Abstract Background: Alveolar Soft Part Sarcoma (ASPS) is a rare, slow-growing, but highly vascular soft tissue sarcoma, characterised by a high rate of metastases at presentation. Although imaging features of the primary are well described, less detail is available on the imaging pattern of metastatic ASPS. The EORTC 90101 (CREATE) study assessed the efficacy of Crizotinib in patients with metastatic ASPS and presents a unique opportunity to describe the imaging phenotype of primary and metastatic ASPS, based on prospectively collected imaging. Methods: A retrospective review of the staging CT scans of 32 patients with ASPS from the CREATE study was undertaken and the imaging features of primary and metastatic disease were assessed. Results: Imaging of the primary tumour was available in 7/32 cases (28%). All primary tumours demonstrated marked vascularity with prominent feeding vessels (7/7, 100%). The most frequent sites of metastases included lung (30/32, 94%), nodal (7/32, 22%), bone (5/32, 16%) and muscle/subcutaneous (5/32, 16%). Features of hypervascularity were identified at all sites, more appreciable in the lungs, with feeding vessels frequently demonstrated in pulmonary metastases (21/32, 66%). Conclusion: Analysis of imaging from the CREATE cohort of patients with metastatic ASPS demonstrates that metastases from ASPS are predominantly hypervascular and demonstrate feeding vessels comparable to primary ASPS, suggesting potential sensitivity of this rare sarcoma for antivascular/antiangiogenic treatment approaches. Keywords: Alveolar soft part sarcoma, Soft tissue sarcoma, Metastatic disease, Computer tomography
Introduction Alveolar Soft Part Sarcoma (ASPS) is a rare soft tissue tumour accounting for less than 1% [1] of soft tissue sarcomas. It most commonly occurs in younger patients between 15 and 35 years with a slight female preponderance [2]. It frequently arises in the lower extremities [3] but may also arise from the trunk and retroperitoneum and, in younger children, the orbit and tongue [4]. It is a relatively slow growing tumour, associated with minimal * Correspondence: [email protected] 1 Department of Radiology, The Royal Marsden Hospital, London, UK Full list of author information is available at the end of the article
clinical symptoms [5] characterised by a high rate of metastases at presentation [6]. Imaging is essential for assessing the primary tumour and extent of metastatic spread. Whilst the radiological features of primary ASPS have been described with computer tomography (CT) and magnetic resonance (MR) in serial case reports, comparatively less information is available on the imaging characteristics of the metastases. The European Organisation for Research and Treatment of Cancer (EORTC) recently conducted a prospectiv
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