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Vascular tumours range from angiosarcoma, an aggressive cancer of blood vessel lining cells, to the slow-growing EHE and the infant tumour KHE. Angiosarcoma responds to paclitaxel and, in the sun-damaged scalp form, to immunotherapy; EHE and KHE depend on growth signals that the mTOR blocker sirolimus quiets, and EHE without symptoms is watched.
Malignant and intermediate vascular tumours share an endothelial origin but differ sharply in behaviour. Angiosarcoma is a high-grade sarcoma arising in sun-damaged skin of the scalp and face of older adults, in the breast after radiotherapy (with MYC amplification), in lymphoedematous limbs (Stewart-Treves) or in viscera; ultraviolet-signature cutaneous tumours carry a high mutation burden. Epithelioid haemangioendothelioma (EHE) is defined by the WWTR1-CAMTA1 fusion (or YAP1-TFE3 in a minority) that constitutively activates the Hippo pathway effector TAZ; it is multifocal in liver, lung and bone and may stay stable for years. Kaposiform haemangioendothelioma (KHE) is an infantile tumour with lymphatic features that can trigger the Kasabach-Merritt phenomenon, a consumptive coagulopathy. Infantile haemangioma, though benign, is on the same NCI page and is treated with propranolol.
Angiosarcoma is treated with wide resection and radiotherapy when localised; paclitaxel (ANGIOTAX) is the preferred first-line chemotherapy, with doxorubicin and gemcitabine-based regimens as alternatives, and propranolol has been added in some series. Checkpoint inhibitors produce responses particularly in cutaneous head and neck angiosarcoma (DART SWOG S1609 cohort, ipilimumab plus nivolumab), consistent with its UV-driven mutation load. EHE is managed by surveillance when asymptomatic, sirolimus when progressive (mTOR inhibition targets the tumour's dependency on PI3K-mTOR signalling downstream of TAZ), and transplant is considered for isolated hepatic disease. KHE with Kasabach-Merritt is treated with sirolimus, which has replaced vincristine and steroids as first line in many centres.
Mechanistic frontiers are TEAD inhibitors that block the TAZ-TEAD transcriptional complex in EHE, and immunotherapy combinations for angiosarcoma.
Angiosarcoma accounts for about 1 to 2 percent of soft tissue sarcomas; EHE and KHE are rarer still, with KHE mostly in infants.
Bone sarcomas favour the fast-growing ends of long bones (osteosarcoma) or the shaft (Ewing), soft tissue sarcomas the deep muscle compartments; spread is through the blood to the lungs, rarely via lymph nodes.
Lymph node spread is rare (except epithelioid, synovial, clear cell and rhabdomyosarcoma); sarcomas go through the blood to the lungs.
Same organ: Adamantinoma of bone, Dedifferentiated chordoma, Poorly differentiated chordoma (SMARCB1-deficient), Desmoplastic small round cell tumour, Leiomyosarcoma, Liposarcoma, Synovial sarcoma, Sarcomas (soft tissue, bone, GIST), Osteosarcoma, Ewing sarcoma, Rhabdomyosarcoma, Chordoma, Desmoid tumour, Tenosynovial giant cell tumour (TGCT), Epithelioid sarcoma, Chondrosarcoma, Angiosarcoma, Undifferentiated pleomorphic sarcoma (UPS), Myxofibrosarcoma, Alveolar soft part sarcoma, Perivascular epithelioid cell tumour (PEComa), Epithelioid haemangioendothelioma, Malignant peripheral nerve sheath tumour (MPNST), Retroperitoneal sarcoma, Soft tissue sarcoma of the extremity (localised and advanced)
Wide excision with radiotherapy; margins are often positive because of field spread in the scalp, and neoadjuvant paclitaxel is used to downstage.
Weekly paclitaxel (ANGIOTAX) or doxorubicin-based chemotherapy; gemcitabine-docetaxel, pazopanib; checkpoint inhibitors for cutaneous head and neck disease (DART cohort) or in trials.
Active surveillance if asymptomatic and stable; sirolimus for progressive or symptomatic disease; surgery or liver transplant for isolated hepatic disease.
Sirolimus, with steroids in the acute phase; vincristine as an alternative; platelet transfusion avoided unless bleeding because it feeds the consumptive process.
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2 trials on record are attached to one of the types below rather than to Vascular tumours (angiosarcoma, epithelioid haemangioendothelioma, kaposiform haemangioendothelioma) itself. They are grouped by the type that holds them, so someone still working out which type they have can see the whole field from here.
Query for this cancer: (TITLE:"Vascular tumours" OR ABSTRACT:"Vascular tumours" OR TITLE:"angiosarcoma, epithelioid haemangioendothelioma, kaposiform haemangioendothelioma" OR ABSTRACT:"angiosarcoma, epithelioid haemangioendothelioma, kaposiform haemangioendothelioma" OR TITLE:"Childhood vascular tumours" OR ABSTRACT:"Childhood vascular tumours" OR TITLE:"Angiosarcoma" OR ABSTRACT:"Angiosarcoma" OR TITLE:"EHE" OR ABSTRACT:"EHE" OR TITLE:"KHE" OR ABSTRACT:"KHE") AND (treatment OR therapy OR trial OR survival OR diagnosis). Results are unfiltered search hits about Vascular tumours (angiosarcoma, epithelioid haemangioendothelioma, kaposiform haemangioendothelioma), not a curated reading list.
Léauté-Labrèze and colleagues, NEJM: serendipitous discovery.
Penel and colleagues, JCO, phase 2.
Tanas and colleagues, Sci Transl Med; Errani and colleagues.
Florou and colleagues; later confirmed in the DART SWOG S1609 angiosarcoma cohort (2021).
Surveillance first, sirolimus for progression (ESMO Open).
The targets of this cancer's medicines and the ones linked to it directly.
Cases by country, the UK and NHS pathway and other country lenses, the expert centres with trials on record, and the centres named on this cancer's subtypes.
One section per setting: the options named, what each is for, the trials behind them, the recorded trade-offs and the questions to ask.
Bleeding that does not stop by itself, bleeding from more than one site, or new bruising in several places or one large area.
Persistent headache with extreme tiredness, nausea, dizziness on standing or low blood pressure. Vomiting, severe weakness or collapse is adrenal crisis.
Sudden severe abdominal pain, a hard or very tender abdomen, or abdominal pain with vomiting and fever. Boxed warning for gastrointestinal perforation on bevacizumab.
Fainting, near-fainting, or an irregular or racing heartbeat; several kinase inhibitors prolong the QT interval and the labels require ECG and electrolyte monitoring.
Cumulative dose: risk rises steeply above 400-550 mg/m² (see the anthracycline calculator).
Take on an empty stomach (1 hour before or 2 hours after food).
See all on the product pages:DoxorubicinIpilimumabNivolumabPaclitaxel / nab-paclitaxelPazopanibVincristine·Printable cards in the navigator
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Everything in development, the medicines held by this cancer's subtypes, the open problems and what is being done about them, the roadmaps, and what changed on this record.
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