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Single-Center Experience with Intimal Sarcoma, an Ultra-Orphan, Commonly Fatal Mesenchymal Malignancy.
- Source :
-
Oncology research and treatment [Oncol Res Treat] 2017; Vol. 40 (6), pp. 353-359. Date of Electronic Publication: 2017 May 12. - Publication Year :
- 2017
-
Abstract
- 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.<br />Methods: We reviewed all cases of intimal sarcoma that were diagnosed, treated and followed at the University Hospitals Leuven between April 2006 and April 2016.<br />Results: We identified 13 patients with a median age of 51 years. In 6 patients initial findings were suggestive of thromboembolic disease. Platelet-derived growth factor receptor α (PDGFRA) amplification was the most prevalent molecular finding, present in 11 patients. The MDM2 gene was amplified in 9 cases, and the EGFR gene in 3 patients. The median overall survival was 13 months. 11 patients underwent surgery. In 5 cases with inoperable and/or metastatic disease chemotherapy was given. Treatment with imatinib was initiated in 4 patients.<br />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 1 molecular target, combination therapy might be an option, although toxicity may be a limitation.<br /> (© 2017 S. Karger GmbH, Freiburg.)
- Subjects :
- Adolescent
Adult
Age Distribution
Aged
Belgium epidemiology
Child
Child, Preschool
Female
Humans
Male
Middle Aged
Prognosis
Rare Diseases metabolism
Rare Diseases mortality
Rare Diseases therapy
Risk Factors
Sarcoma therapy
Sex Distribution
Survival Rate
Treatment Outcome
Tunica Intima pathology
Vascular Neoplasms therapy
Young Adult
Biomarkers, Tumor metabolism
Critical Illness mortality
Sarcoma metabolism
Sarcoma mortality
Vascular Neoplasms metabolism
Vascular Neoplasms mortality
Subjects
Details
- Language :
- English
- ISSN :
- 2296-5262
- Volume :
- 40
- Issue :
- 6
- Database :
- MEDLINE
- Journal :
- Oncology research and treatment
- Publication Type :
- Academic Journal
- Accession number :
- 28501860
- Full Text :
- https://doi.org/10.1159/000476036