Why the study?
Malignant vascular tumors of the head and neck have heterogeneous presentations that often lead to misdiagnosis and unsuitable treatment, with a lack of standard medical therapy for metastasized disease.
This review summarizes the diagnosis and management of rare malignant vascular tumors of the head and neck, highlighting surgery as the mainstay of treatment and differing responses to medical therapies between tumor types.
Diagnostic vigilance needed for atypical head and neck vascular tumors; case report leaves open questions on prognosis and optimal therapy.
Malignant vascular tumors of the head and neck are rare neoplasms with variable clinical presentation, wide age distribution, and variable clinical courses. The heterogeneous presentation of angiosarcomas and epithelioid hemangioendothelioma often leads to misdiagnosis and unsuitable treatment. While risk factors for angiosarcomas are previous radiation, chronic lymphedema, and exposure to arsenic, thorium oxide, or vinyl chloride, there are only limited and retrospective data available on prognostic factors in EHE. In both angiosarcomas and EHE, surgery is the mainstay of treatment. There is limited evidence regarding the role of radiotherapy in EHE, although EHE is considered relatively radiosensitive. In angiosarcomas, adjuvant radiotherapy is recommended according to retrospective case series. A standard medical therapy for metastasized malignant vascular tumors is lacking. Chemotherapy, which is effective in angiosarcoma, is mostly ineffective in EHE. Targeted therapy, antiangiogenetic drugs and immunotherapy have been studied as new treatment options. The goal of this review is to summarize the current data regarding malignant vascular tumors along with their diagnosis and management.
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Wiegand et al. (2021) studied this question.
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