VERY-RARE CANCERSarcomaWHO 5th Edition Classification

Angiosarcoma

Very Rare Malignancy (1–5 cases per 1,000,000 / year) • Clinical Staging, Genomic Targets & Vetted Specialists

A malignant tumour of the cells lining blood and lymphatic vessels. It occurs in several distinct settings: on the scalp and face of older adults, in the breast after radiotherapy, in long-standing lymphoedema after breast cancer surgery (Stewart-Treves syndrome), and in deep viscera such as the liver, heart and spleen.

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Emergent Referral AdvisoryImmediate Action

Certain rare malignancies progress rapidly or carry acute risk of airway obstruction, acute spinal compression, hydrocephalus, or biopsy-seeding. Do not perform needle biopsy or surgery outside of an NCI-designated specialty sarcoma or neuro-oncology unit without multidisciplinary tumor board review.

Report immediately to an emergency department or tertiary oncologist upon:

  • Any non-healing bruise-like scalp lesion in an older adult - biopsy
Who It Affects

Older adults for cutaneous disease; any age for visceral or secondary forms

Annual Incidence

Approximately 1-2% of soft-tissue sarcomas; roughly 3 per million per year

Clinical Evidence Review

Last audited: 2026-08-13 against NCCN, ESMO, and WHO 5th ed. guidelines.

Clinical Presentation & Hallmark Symptoms

Presenting signs most frequently observed across clinical case series

A bruise-like patch on the scalp or face that will not resolve
A rapidly enlarging, sometimes bleeding, skin lesion
New skin change in a previously irradiated field
Swelling, pain or a mass in a chronically lymphoedematous limb
Anaemia, abdominal pain or spontaneous bleeding with visceral disease

Genomic Profiling & Defining Molecular Lesions

Critical diagnostic fusions, somatic mutations, and therapeutic targets

Rare malignancies frequently depend on distinct oncogenic drivers rather than conventional environmental carcinogens. Comprehensive Next-Generation Sequencing (NGS comprehensive panel) and FISH/IHC are mandatory to establish the true diagnosis and screen for basket trial agents.

MYC amplification (radiation/lymphoedema-associated)KDRPLCG1PTPRBTP53

Standard-of-Care Treatment Protocol

Frontline and multimodal strategies established under international consensus guidelines

1

Wide surgical excision - margins are frequently difficult on the scalp

Administered in specialized high-volume oncology programs with subspecialty pathology and organ-preservation protocols.

2

Radiotherapy, often large-field, for cutaneous scalp disease

Administered in specialized high-volume oncology programs with subspecialty pathology and organ-preservation protocols.

3

Weekly paclitaxel - the most consistently active systemic agent

Administered in specialized high-volume oncology programs with subspecialty pathology and organ-preservation protocols.

4

Doxorubicin-based chemotherapy

Administered in specialized high-volume oncology programs with subspecialty pathology and organ-preservation protocols.

5

Antiangiogenic agents (pazopanib, bevacizumab) and propranolol in selected settings

Administered in specialized high-volume oncology programs with subspecialty pathology and organ-preservation protocols.

6

Immunotherapy in UV-signature cutaneous head and neck disease

Administered in specialized high-volume oncology programs with subspecialty pathology and organ-preservation protocols.

Clinical Trial Advisory: For rare and ultra-rare malignancies, enrollment in an active clinical trial or expanded-access program is widely considered the preferred standard of care by ASCO and NCCN panels.

Prognosis & Disease Trajectory

Objective clinical outlook without false reassurance

Generally poor, with five-year survival around 30-40% and worse for visceral, cardiac and radiation-associated disease. Small, completely resected cutaneous lesions do considerably better.

Note: Statistics reflect cohort averages. Individual outcomes depend heavily on performance status, resectability, biomarker expression, and timely access to specialized tertiary care.

Active Research, Biomarkers & Clinical Trials

Novel investigational agents, phase I/II trials, and international rare disease consortia

Checkpoint inhibitors show real activity in the high-mutational-burden UV-driven cutaneous subtype; MYC-amplified radiation-associated disease is being studied separately as a distinct entity.

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Expert Clinicians

Verified Specialists for Angiosarcoma

Browse All Oncologists

Dr. Sandra D'Angelo

Medical Oncologist

15+ yrs exp

MSKCC

New York, USA

AngiosarcomaImmunotherapy CombinationsClinical Trials
Vetted DirectoryConsult Specialist

Dr. Toh Wei Peng

Surgical Oncologist

20+ yrs exp

National Cancer Centre

Singapore, Singapore

Surgical OncologyHead/Neck AngiosarcomaMultimodal Therapy
Vetted DirectoryConsult Specialist

Prof. Henrik Falch

Surgical Oncologist

20+ yrs exp

University Hospital Copenhagen

Copenhagen, Denmark

Cutaneous AngiosarcomaSurgeryIntralesional Therapy
Vetted DirectoryConsult Specialist

Dr. Navin Khattry

Medical Oncologist

15+ yrs exp

ACTREC

Mumbai, India

Rare SarcomasAngiosarcomaMetronomic Regimens
Vetted DirectoryConsult Specialist
Hospital Network

Designated Cancer Centers with Dedicated Programs

Search All 72 NCI Centers

Memorial Sloan Kettering Cancer Center

NY

NCI-Designated Comprehensive Cancer Center

World-leading rare tumor board & pediatric solid tumor protocols

Proton TherapyCAR-T Cell TherapyRobotic Surgery
1,000+ Active Clinical TrialsView Center Profile

University of Texas MD Anderson Cancer Center

TX

NCI-Designated Comprehensive Cancer Center

Largest specialized rare cancer and sarcoma multidisciplinary program

Proton TherapyCAR-T Cell TherapyCyberKnife SBRT
1,200+ Active Clinical TrialsView Center Profile

Dana-Farber / Harvard Cancer Center

MA

NCI-Designated Comprehensive Cancer Center

Pioneering genomic molecular tumor boards & rare histologies

Proton TherapyCAR-T Cell TherapyPhase I Unit
1,100+ Active Clinical TrialsView Center Profile

Mayo Clinic Comprehensive Cancer Center

MN

NCI-Designated Comprehensive Cancer Center

High-volume surgical oncology & rare endocrine/neuroendocrine expertise

Proton TherapyCAR-T Cell TherapyCyberKnife SBRT
600+ Active Clinical TrialsView Center Profile

Frequently Asked Clinical Questions

Authoritative guidance on diagnosis, tumor boards, genomic markers, and care options

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