92 - Postersitzung
17. Mai 2019, 12:30 - 14:00, Bellavista 2, 6. OG
Acute thrombosis masking Ewing sarcoma of the femoral vein- A systematic review.
D. Jaeger1, M. Hofmann1, Z. Rancic1, M. L. Valentin1, W. Scheurlen2, W. Schmidt2, A. Palma1, Presenter: D. Jaeger1 (1Zürich, 2Nürnberg/DE)
Ewing sarcoma (ES) is a highly aggressive, small round cell malignant neoplasm of bone and soft tissue that typically manifests in children and young adults. It is most commonly a primary bone tumor; however, extraosseous cases have been described. We report on a case of ES with the primary tumor in the right common femoral vein (CFV). Thrombosis, in this case, played a role masking the tumor.
Due to thrombus and total congestion of the right CFV open surgery and thrombectomy was mandatory. Intraoperative histology showed suspicious malignancy. In addition, we performed a systematic review of the literature. Protocol and eligibility criteria were specified in advance and registered at the PROSPERO registry. Published literature of primary manifestation of ES in the vascular system was selected.
A 14year old male, presented to the emergency department with progressive swelling and weakness of the right lower limb for 3 weeks, and no further clinical findings. Laboratory values were unremarkable, except for elevated creatine kinase (558U/l). Ultrasound showed local thrombosis of the CFV. MRI showed no involvement of surrounding soft tissue. Because of subacute clinical status and suspected local thrombosis, the patient initially underwent an angiographic procedure with unsuccessful thrombolysis. Further diagnostics consisted in PET-CT Scan that showed local signs of inflammation in the area of occlusion. Due to acute progression of thrombosis open surgery through longitudinal venotomy and excision of thrombus-like mass in toto was performed. Histology confirmed a CD99 positive small blue round cell tumor, most likely an ES. Postoperative course remained uneventful. Oncological treatment consisted in chemotherapy according to EWING 2008 Trial. None of the studies assessed in the systematic review met the inclusion criteria. No study describing primary ES in the venous system was found.
This is the first report on primary manifestation of ES in the venous system. Extraosseous ES shows a high rate of local recurrence and the outcome seems to differ from patients with skeletal ES. Early diagnosis and treatment is crucial. Neoadjuvant therapy provides better prognosis and systematic chemotherapy improves survival. Recent data shows need for an algorithm for early diagnosis, treatment and further therapy strategies according to international guidelines.