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Angiosarcoma
Angiosarcoma is a rare, aggressive malignant endothelial soft-tissue sarcoma (cutaneous, breast, deep soft tissue, visceral), often with early hematogenous spread
Prevalence
10 / 100 000
N/A
US Estimated
N/A
Europe Estimated
Age of Onset
Adult
ICD-10
C49
Inheritance Pattern
Autosomal dominant
Autosomal recessive
Mitochondrial/Multigenic
X-linked dominant
X-linked recessive
Rare View
A rare and aggressive malignancy of endothelial cells that line blood vessels, often occurring in the skin, breast, liver, and other organs. It is characterized by a high rate of local recurrence and distant metastasis, requiring aggressive treatment.
5 Facts you should know
FACT
Only about 300 cases of angiosarcoma are diagnosed per year in the United States
FACT
Older people are more commonly affected, and the skin is the most affected area, with approximately 60% of cases being cutaneous
FACT
The scalp makes up ~50% of angiosarcoma cases
FACT
Known risk factors include chronic lymphedema, radiation therapy and various chemicals such as arsenic and vinyl chloride
FACT
5
For a subset of patients with angiosarcoma of head, neck, face, and scalp, ultraviolet damage may be a causative factor therefore immune checkpoint inhibition may be beneficial
Interest over time
Google searches
Common signs & symptoms
Cutaneous angiosarcoma
- Bruise-like or purplish skin lesions
- Rapidly enlarging nodules
- Ulceration or bleeding
Breast
- Swelling or skin discoloration
- Palpable mass (primary or post-radiation)
Liver
- Abdominal pain
- Weight loss
- Hepatomegaly
- Jaundice
General
- Fatigue
- Pain
- Shortness of breath (if pulmonary involvement)
Current treatments
Localized disease
wide surgical resection when feasible; radiation often used depending on site/extent. Angiosarcoma-specific recommendations often extrapolate from STS guidance.
Advanced/metastatic
systemic therapy commonly used (regimens vary by site and patient factors); taxanes (e.g., paclitaxel) are widely used options in practice; other agents may be considered in sarcoma frameworks.
Top Clinical Trials
| Title | Description | Phases | Status | Interventions | More Information |
|---|---|---|---|---|---|
| Testing the Addition of Nivolumab to Chemotherapy in Treatment of Soft Tissue Sarcoma | This phase II trial studies how well paclitaxel with and without nivolumab works in treating patients with soft tissue sarcoma that have not received taxane drugs, and how well nivolumab and cabozantinib work in treating taxane pretreated patients with soft tissue sarcoma. Nivolumab works through th... | Phase 2 | Active | Procedure: Biospecimen Collection Drug: Cabozantinib S-malate Procedure: Computed Tomography Procedure: FDG-Positron Emission Tomography Procedure: Magnetic Resonance Imaging Biological: Nivolumab Drug: Paclitaxel Other: Quality-of-Life Assessment Other: Questionnaire Administration | More Info |
| I-SPY TRIAL: Neoadjuvant and Personalized Adaptive Novel Agents to Treat Breast Cancer | The purpose of this study is to further advance the ability to practice personalized medicine by learning which new drug agents are most effective with which types of breast cancer tumors and by learning more about which early indicators of response (tumor analysis prior to surgery via magnetic reso... | Phase 2 | Recruiting | Drug: Standard Therapy Drug: AMG 386 with or without Trastuzumab Drug: AMG 479 (Ganitumab) plus Metformin Drug: MK-2206 with or without Trastuzumab Drug: AMG 386 and Trastuzumab Drug: T-DM1 and Pertuzumab Drug: Pertuzumab and Trastuzumab Drug: Ganetespib Drug: ABT-888 Drug: Neratinib Drug: PLX3397 Drug: Pembrolizumab - 4 cycle Drug: Talazoparib plus Irinotecan Drug: Patritumab and Trastuzumab Drug: Pembrolizumab - 8 cycle Drug: SGN-LIV1A Drug: Durvalumab plus Olaparib Drug: SD-101 + Pembrolizumab Drug: Tucatinib plus trastuzumab and pertuzumab Drug: Cemiplimab Drug: Cemiplimab plus REGN3767 Drug: Trilaciclib with or without trastuzumab + pertuzumab Drug: SYD985 ([vic-]trastuzumab duocarmazine) Drug: Oral Paclitaxel + Encequidar + Dostarlimab (TSR-042) + Carboplatin with or without trastuzumab Drug: Oral Paclitaxel + Encequidar + Dostarlimab (TSR-042) with or without trastuzumab Drug: Amcenestrant Drug: Amcenestrant + Abemaciclib Drug: Amcenestrant + Letrozole Drug: ARX788 Drug: ARX788 + Cemiplimab Drug: VV1 + Cemiplimab Drug: Datopotamab deruxtecan Drug: Datopotamab deruxtecan + Durvalumab Drug: Zanidatamab Drug: Lasofoxifene Drug: Z-endoxifen Drug: ARV-471 Drug: ARV-471 + Letrozole Drug: ARV-471 + Abemaciclib Drug: Endoxifen + Abemaciclib Drug: Rilvegostomig + TDXd Drug: Dan222 + Niraparib Drug: Sarilumab + Cemiplimab + Paclitaxel Drug: GSK 5733584 Drug: GSK 5733584 + Dostarlimab | More Info |
| Precision Medicine Approaches for Neoadjuvant Therapy in High-risk Sarcoma Patients | This is a cohort study aimed at developing a stratified medicine approach for personalised neoadjuvant chemotherapy (NCT) in high-risk soft tissue sarcoma (STS) patients with dedifferentiated liposarcoma (DDLPS), leiomyosarcoma (LMS), synovial sarcoma (SS), vascular sarcomas, malignant peripheral ne... | Recruiting | Other: Observational Translational Study | More Info | |
| A Phase II Study by Using CICS-1 and SPM-011 Commissioned by CICS and STELLA PHARMA | The purpose of the study is to investigate efficacy and safety Boron Neutron Capture Therapy (BNCT) by using CICS-1 accelerator-based neutron capture therapy device with lithium targets developed by CICS, and the SPM-011 boron compound for use in BNCT developed by STELLA PHARMA in the treatment of u... | Phase 2 | Active | Radiation: BNCT | More Info |
| Registry For Children, Adolescents And Adults With Osteosarcoma And Biologically Related Bone Sarcomas | The Registry For Children, Adolescents And Adults With Osteosarcoma And Biologically Related Bone Sarcomas (COSS-Registry) is a non-interventional, multicentric, international, clinical and epidemiologic patient registry. The COSS-Registry collects key data on osteosarcomas or biologically related b... | Not yet recruiting | More Info | ||
| A French Multicenter Observational Retrospective Study of Rare Primary Liver Cancers | The aim of this French multicenter retrospective study is to describ rare primary hepatic cancers clinical, histological and radiological features, to obtain a biological tumor and blood collection, and to evaluate the efficacy of treatments received in clinical practice in order to determine optima... | Recruiting | More Info | ||
| Nivolumab and Ipilimumab in Treating Patients With Rare Tumors | This phase II trial studies nivolumab and ipilimumab in treating patients with rare tumors. Immunotherapy with monoclonal antibodies, such as nivolumab and ipilimumab, may help the body's immune system attack the cancer, and may interfere with the ability of tumor cells to grow and spread. This tri... | Phase 2 | Active | Procedure: Biospecimen Collection Procedure: Computed Tomography Procedure: Echocardiography Test Biological: Ipilimumab Procedure: Magnetic Resonance Imaging Biological: Nivolumab | More Info |
| Cardiac Angiosarcoma International Registry | Primary cardiac angiosarcomas (PCA) are highly aggressive malignant heart tumors arising from the endothelial cells (ECs) lining the blood vessels of the heart and account for approximately 25%-30% of all primary cardiac malignancies. It is considered to be the most fatal and aggressive primary card... | Recruiting | More Info | ||
| Efficacy and Safety of Regorafenib as Maintenance Therapy After First-line Treatment in Patients With Bone Sarcomas | Randomized, non-comparative, multicentre exploratory phase II study. Two arms concerning patients with bone sarcoma after the first line therapy: in the first arm, patients will be treated with Regorafenib for a maximum of 12 months as maintenance therapy after first line therapy, whereas in the se... | Recruiting | Drug: Treatment by Regorafenib Other: Surveillance | More Info | |
| MASCT-I Combined With Doxorubicin and Ifosfamide for First-line Treatment of Advanced Soft Tissue Sarcoma | This study will evaluate the safety and efficacy of MASCT-I combined with Doxorubicin and Ifosfamide for first-line treatment in patients with advanced soft tissue sarcoma. | Phase 2 | Recruiting | Biological: MASCT-I Drug: Doxorubicin Drug: Ifosfamide | More Info |
References:
Young RJ, Brown NJ, Reed MW, Hughes D, Woll PJ. Angiosarcoma. Lancet Oncol. 2010;11(10):983–991. doi:10.1016/S1470-2045(10)70023-1 Penel N, Bui BN, Bay JO, et al. Phase II trial of weekly paclitaxel for unresectable angiosarcoma: the ANGIOTAX Study. J Clin Oncol. 2008;26(32):5269–5274. doi:10.1200/JCO.2008.17.3146 Fury MG, Antonescu CR, Van Zee KJ, Brennan MF, Maki RG. A 14-year retrospective review of angiosarcoma: clinical characteristics, prognostic factors, and treatment outcomes. Cancer J. 2005;11(3):241–247. doi:10.1097/00130404-200505000-00010 Florou V, Wilky BA. Current and future directions for angiosarcoma therapy. Curr Treat Options Oncol. 2018;19(3):14. doi:10.1007/s11864-018-0530-y Fletcher CDM, Bridge JA, Hogendoorn PCW, Mertens F, eds. WHO Classification of Tumours of Soft Tissue and Bone. 4th ed. Lyon, France: IARC Press; 2013 — Authoritative classification and pathology reference.