Epithelioid hemangioendothelioma of the mandible in a 15-year-old: A CARE-compliant case report and review of the literature.
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BACKGROUND: Epithelioid hemangioendothelioma (EHE) is an ultra-rare vascular neoplasm of intermediate malignant potential. Involvement of the craniofacial skeleton, particularly the mandible in pediatric patients, is exceptional and poses substantial diagnostic challenges. CASE PRESENTATION: A 15-year-old male presented with a 2-month history of a painless, progressively enlarging swelling of the left mandible. Extraoral examination revealed a hard, non-tender diffuse mass. Computed tomography demonstrated a large, mixed-density destructive lesion (67 × 81.5 × 86.8 mm). Incisional biopsy followed by histopathological examination showed a hypercellular myxovascular stroma containing round to ovoid epithelioid cells with characteristic intracytoplasmic vacuoles ("blister cells") and numerous vascular channels. The patient underwent subtotal mandibulectomy with disarticulation of the left condyle. Postoperative recovery was uneventful, and follow-up at 2 weeks, 6 weeks, and 6 months showed satisfactory healing without recurrence. CONCLUSION: EHE should be considered in the differential diagnosis of destructive jaw lesions in young patients, even when imaging suggests a conventional sarcoma. Definitive diagnosis relies on histopathologic recognition of epithelioid endothelial cells within a myxovascular stroma. Early complete surgical resection remains the standard of care for localized disease.