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- W2891484844 abstract "e22532 Background: Intimal sarcoma is a rare malignancy that, clinically and radiographically, often mimics pulmonary embolism. The intravascular tumor tends to disseminate rapidly and metastases can be present at first diagnosis. Methods: We reviewed all cases of intimal sarcoma which were diagnosed, treated and followed at the University Hospitals Leuven between April 2006 and April 2016. Results: We identified 13 patients with a median age of 51. In 6 patients initial findings were suggestive of thromboembolic disease. PDGFRA amplification was the most prevalent molecular finding, present in 11 patients. The MDM2 gene was amplified in 9 cases, the EGFR gene in three patients. The median overall survival was 13 months. Eleven patients underwent surgery. In 5 cases with inoperable and/or metastatic disease chemotherapy was given. Treatment with imatinib was initiated in 4 patients. Conclusions: Intimal sarcoma is an extremely rare and aggressive malignancy that has a very poor prognosis. Mimicking thromboembolic disease, diagnosis and treatment can be delayed. Surgery is the mainstay of treatment but is seldom curative. The disease is highly resistant to cytotoxic and targeted treatment. Given the fact that intimal sarcoma commonly expresses more than one molecular target, combination therapy might be an option, though toxicity may be a limitation." @default.
- W2891484844 created "2018-09-27" @default.
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- W2891484844 date "2017-05-20" @default.
- W2891484844 modified "2023-10-14" @default.
- W2891484844 title "Single-center experience with intimal sarcoma, an ultra-orphan, commonly fatal mesenchymal malignancy." @default.
- W2891484844 doi "https://doi.org/10.1200/jco.2017.35.15_suppl.e22532" @default.
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