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10 slides generated from the cancer page, with a quiz from the open benchmark and speaker notes that cite the sources. Arrow keys move between slides; Print gives one slide per page.
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.
| Setting | Approach | Guideline |
|---|---|---|
| Localised angiosarcoma | Wide excision with radiotherapy; margins are often positive because of field spread in the scalp, and neoadjuvant paclitaxel is used to downstage. | NCCN Category 2A |
| Advanced angiosarcoma | Weekly paclitaxel (ANGIOTAX) or doxorubicin-based chemotherapy; gemcitabine-docetaxel, pazopanib; checkpoint inhibitors for cutaneous head and neck disease (DART cohort) or in trials. | not mapped |
| Epithelioid haemangioendothelioma | Active surveillance if asymptomatic and stable; sirolimus for progressive or symptomatic disease; surgery or liver transplant for isolated hepatic disease. | not mapped |
| Kaposiform haemangioendothelioma with Kasabach-Merritt | Sirolimus, with steroids in the acute phase; vincristine as an alternative; platelet transfusion avoided unless bleeding because it feeds the consumptive process. | not mapped |