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Epithelioid hemangioendothelioma (EHE) is a rare tumor, first characterized by Sharon Weiss and Franz Enzinger in 1982 [1] that both clinically and histologically is intermediate between angiosarcoma and hemangioma. However, a distinct, disease-defining genetic alteration recently described for EHE indicates that it is an entirely separate ...
Epithelioid hemangioendothelioma is an uncommon vascular tumor of intermediate malignancy that was first described by Steven Billings, Andrew Folpe, and Sharon Weiss in 2003. [1] These tumors are so named because their histologic appearance resembles a proliferation of epithelioid cells, with polygonal shape and eosinophilic cytoplasm.
Differential diagnosis includes vascular malformation, angioma, pyogenic granuloma, angiosarcoma, epithelioid hemangioendothelioma, Kaposi’s sarcoma, and other more rare vascular tumors. Three forms of intravascular papillary endothelial hyperplasia exist: the primary or pure form, the secondary or mixed form, and the extravascular form.
Kaposiform hemangioendothelioma. Kaposiform hemangioendotheliomas (KHEs) are borderline, locally destructive vascular tumors. [4] They are named after their resemblance to the lesions of Kaposi's sarcoma. [13] KHEs are described as locally destructive because they can infiltrate underlying muscle and fat. [4]
Angiolymphoid hyperplasia with eosinophilia (also known as: [1] "Epithelioid hemangioma," "Histiocytoid hemangioma," "Inflammatory angiomatous nodule," "Intravenous atypical vascular proliferation," "Papular angioplasia," "Inflammatory arteriovenous hemangioma," and "Pseudopyogenic granuloma") usually presents with pink to red-brown, dome-shaped, dermal papules or nodules of the head or neck ...
Depending on the grade of the sarcoma, it is treated with surgery, [8] chemotherapy, and/or radiotherapy.Though surgery is the current standard of care for hemangiopericytomas, metastasis and tumor recurrence occur in more than 30% of patients, in particular recurrence in the pelvis and retroperitoneum [3] and metastasis in bone and lungs. [9]
Epithelioid may refer to: Epithelioid cell, a cell that resembles epithelial cells; Epithelioid sarcoma, a soft tissue tumour; Epithelioid hemangioendothelioma, a vascular tumour occurring in the lining of blood vessels; Epithelioid blue nevus, a melanocytic nevus; Epithelioid sarcoma-like hemangioendothelioma, a group of vascular neoplasms
The epithelioid inflammatory myofibroblastic sarcoma subtype of IMT shows sheets of epithelioid to round cells within a myxoid (i.e. appears blue or purple compared to the normal red appearance of connective tissue when appropriately H&E stained and examined under the microscope), collagenous, or mixed myxoid-collagenous matrix, <5% spindle ...