Malignant epithelioid hemangioendothelioma (MEH), also known as high-risk epithelioid hemangioendothelioma, is a low- to intermediate-grade vascular malignancy originally described as a vascular neoplasm of endothelial origin. This very rare vascular neoplasm has been described mainly in soft tissue, but also in various organs and locations, including the liver, lung, brain, colon, lymph nodes, peritoneum, spleen, bone, skin, heart, soft tissues, and vascular system. Several cases have been described in the head and neck, including the submandibular gland, parotid gland, nasal cavity, parapharyngeal space, maxilla, maxillary sinus, occipital bone, oral cavity, thyroid gland, neck, scalp, larynx, and mandible. This case report is the first description of MEH presenting as an exophytic lower-lip lesion.
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