Kaposiform Hemangioendothelioma of the Oral Cavity in an Adult Woman: A Case Report

Kaposiform Hemangioendothelioma (KHE) is a rare, locally aggressive vascular tumor with histological similarities to Kaposi’s Sarcoma. It often presents in infancy or early childhood, rarely affecting adults. The most common sites include the skin, retroperitoneum, and less frequently, the head, neck, or mediastinum. KHE is seldom self-regressing, and distant metastases are rare, though 70% of cases may develop Kasabach-Merritt Syndrome (KMS), a severe coagulopathy marked by thrombocytopenia and high mortality due to hemorrhage. This report discusses a case of a 39-year-old woman referred for an exophytic lesion in the left retromolar region, with a diagnosis of Systemic Lupus Erythematosus. A biopsy confirmed KHE, and surgical resection was decided upon. Histological findings revealed characteristic features such as spindle cell clusters, numerous vascular fissure spaces, and eosinophilic cytoplasm. The patient underwent further surgical treatment due to the aggressive nature of KHE and the risk of KMS. This case highlights the importance of early diagnosis and appropriate surgical management for KHE to avoid complications.