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Vascular Tumors and Angiosarcomas
Research Guide
What is Vascular Tumors and Angiosarcomas?
Vascular tumors and angiosarcomas are a cluster of malignant and benign neoplasms arising from vascular endothelial cells, with angiosarcomas representing highly aggressive sarcomas often associated with radiation exposure, and including subtypes such as epithelioid hemangioendothelioma, hepatic angiosarcoma, and radiation-associated angiosarcoma.
Research on vascular tumors and angiosarcomas encompasses 29,386 papers focused on treatment outcomes, diagnostic markers, genetic alterations, risk factors, and therapeutic options. The cluster examines radiation therapy's role in angiosarcoma development and liver transplantation for hepatic angiosarcoma. Growth rate over the past five years is not available.
Topic Hierarchy
Research Sub-Topics
Radiation-Associated Angiosarcoma
Researchers investigate the pathogenesis, latency periods, and molecular changes in angiosarcomas arising post-radiotherapy, particularly in breast cancer survivors. Studies evaluate surveillance protocols and surgical resection outcomes.
Hepatic Angiosarcoma Pathogenesis and Treatment
This area covers etiology including vinyl chloride exposure, genomic profiling, and multimodal therapies like liver transplantation for hepatic angiosarcoma. Research assesses prognosis and neoadjuvant chemotherapy responses.
Epithelioid Hemangioendothelioma Clinical Outcomes
Studies characterize the natural history, prognostic markers, and therapies for epithelioid hemangioendothelioma across hepatic, pulmonary, and soft tissue sites. Focus includes anti-angiogenic agents and liver transplant survival.
Molecular Genetics of Vascular Sarcomas
Researchers profile recurrent mutations like MYC amplifications and KDR alterations in angiosarcomas using NGS. Studies correlate genotypes with phenotypes, sites, and targeted therapy responses.
Systemic Therapy for Metastatic Angiosarcoma
Clinical trials evaluate taxanes, anti-angiogenics, and immunotherapies for advanced angiosarcoma, analyzing progression-free survival by subtype. Research explores pazopanib and trabectedin efficacy.
Why It Matters
Vascular tumors and angiosarcomas impact oncology through targeted therapies inhibiting angiogenesis, as demonstrated in clinical trials for related vascular-dependent cancers. For instance, bevacizumab, an anti-vascular endothelial growth factor antibody, prolonged time to progression in metastatic renal-cell cancer patients in a randomized trial (Yang et al., 2003). Pazopanib improved outcomes in metastatic soft-tissue sarcoma, including vascular subtypes, in the PALETTE phase 3 trial, with median progression-free survival of 4.6 months versus 1.6 months for placebo (van der Graaf et al., 2012). Sunitinib (SU11248) showed activity in metastatic renal cell carcinoma by targeting VEGF and PDGF receptors (Motzer et al., 2005). These VEGF-targeted agents provide prognostic insights, as Heng et al. (2009) identified factors for overall survival in 645 patients treated with such therapies.
Reading Guide
Where to Start
"Endostatin: An Endogenous Inhibitor of Angiogenesis and Tumor Growth" by O’Reilly et al. (1997), as it provides a foundational understanding of endogenous angiogenesis inhibition central to vascular tumor biology, with 4478 citations.
Key Papers Explained
O’Reilly et al. (1997) "Endostatin: An Endogenous Inhibitor of Angiogenesis and Tumor Growth" establishes natural suppression of vascular tumor growth, building to Kim et al. (1993) "Inhibition of vascular endothelial growth factor-induced angiogenesis suppresses tumour growth in vivo" which demonstrates VEGF blockade's in vivo effects. Neufeld et al. (1999) "Vascular endothelial growth factor (VEGF) and its receptors" details VEGF isoforms and receptors, linking to clinical advances like Yang et al. (2003) "A Randomized Trial of Bevacizumab..." showing bevacizumab's efficacy, and Motzer et al. (2005) on sunitinib targeting VEGF/PDGF.
Paper Timeline
Most-cited paper highlighted in red. Papers ordered chronologically.
Advanced Directions
Current research emphasizes VEGF-targeted agents' mechanisms and prognostic models, as in Heng et al. (2009) and Ellis and Hicklin (2008), with pazopanib's role in sarcomas per van der Graaf et al. (2012). No recent preprints or news available.
Papers at a Glance
| # | Paper | Year | Venue | Citations | Open Access |
|---|---|---|---|---|---|
| 1 | Endostatin: An Endogenous Inhibitor of Angiogenesis and Tumor ... | 1997 | Cell | 4.5K | ✓ |
| 2 | Inhibition of vascular endothelial growth factor-induced angio... | 1993 | Nature | 3.6K | ✕ |
| 3 | Vascular endothelial growth factor (VEGF) and its receptors | 1999 | The FASEB Journal | 3.5K | ✕ |
| 4 | A Randomized Trial of Bevacizumab, an Anti–Vascular Endothelia... | 2003 | New England Journal of... | 2.7K | ✓ |
| 5 | Prognostic Factors for Overall Survival in Patients With Metas... | 2009 | Journal of Clinical On... | 2.1K | ✓ |
| 6 | Pazopanib for metastatic soft-tissue sarcoma (PALETTE): a rand... | 2012 | The Lancet | 2.0K | ✓ |
| 7 | Follow-up study of thymomas with special reference to their cl... | 1981 | Cancer | 1.7K | ✕ |
| 8 | A novel vascular endothelial growth factor, VEGF-C, is a ligan... | 1996 | The EMBO Journal | 1.7K | ✓ |
| 9 | VEGF-targeted therapy: mechanisms of anti-tumour activity | 2008 | Nature reviews. Cancer | 1.7K | ✕ |
| 10 | Activity of SU11248, a Multitargeted Inhibitor of Vascular End... | 2005 | Journal of Clinical On... | 1.6K | ✓ |
Frequently Asked Questions
What role does VEGF play in vascular tumors?
Vascular endothelial growth factor (VEGF) acts as a specific mitogen for vascular endothelial cells, with five isoforms generated by alternative splicing that differ in molecular mass and biological properties. "Vascular endothelial growth factor (VEGF) and its receptors" (Neufeld et al., 1999) details VEGF's interaction with receptors. Inhibition of VEGF-induced angiogenesis suppresses tumor growth in vivo (Kim et al., 1993).
How do anti-VEGF therapies treat vascular malignancies?
Anti-VEGF therapies like bevacizumab extend progression-free survival in metastatic renal-cell cancer, as shown in a randomized trial (Yang et al., 2003). Sunitinib targets VEGF and PDGF receptors in renal cell carcinoma patients (Motzer et al., 2005). Ellis and Hicklin (2008) explain mechanisms of anti-tumor activity in "VEGF-targeted therapy: mechanisms of anti-tumour activity".
What are prognostic factors in VEGF-targeted treatments?
In metastatic renal cell carcinoma patients treated with VEGF-targeted agents, prognostic factors for overall survival were analyzed in 645 anti-VEGF therapy-naïve patients (Heng et al., 2009). Baseline characteristics influenced outcomes in this multicenter study. Such factors guide clinical management.
What is the impact of pazopanib on soft-tissue sarcomas?
Pazopanib, in the PALETTE phase 3 trial, was tested in metastatic soft-tissue sarcoma, showing benefits over placebo (van der Graaf et al., 2012). It targets vascular endothelial growth factor receptors relevant to vascular tumors. Median progression-free survival improved significantly.
How does endostatin inhibit vascular tumor growth?
Endostatin serves as an endogenous inhibitor of angiogenesis and tumor growth (O’Reilly et al., 1997). It directly addresses vascular proliferation in tumors like angiosarcomas. The paper details its mechanisms in high-citation work.
What defines vascular tumors in this research cluster?
The cluster covers angiosarcoma, epithelioid hemangioendothelioma, radiation-associated angiosarcoma, hepatic angiosarcoma, and other malignant vascular tumors. Topics include treatment outcomes, diagnostic markers, genetic alterations, and risk factors. Therapeutic options like liver transplantation for hepatic cases are explored.
Open Research Questions
- ? What specific genetic alterations drive radiation-associated angiosarcomas?
- ? How effective is liver transplantation for long-term survival in hepatic angiosarcoma?
- ? Which diagnostic markers best distinguish epithelioid hemangioendothelioma from other vascular tumors?
- ? What are the optimal combinations of VEGF inhibitors with other therapies for metastatic angiosarcomas?
- ? How do risk factors like radiation exposure influence treatment resistance in vascular sarcomas?
Recent Trends
The field maintains a corpus of 29,386 papers on vascular tumors and angiosarcomas, with highly cited works from 1993-2012 focusing on VEGF pathways and inhibitors like endostatin (O’Reilly et al., 1997, 4478 citations), VEGF blockade (Kim et al., 1993, 3594 citations), and clinical trials such as PALETTE for pazopanib (van der Graaf et al., 2012, 2043 citations).
Five-year growth rate unavailable; no recent preprints or news reported.
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