Objectives: To present an uncommon cause for a submandibular mass and review of the literature.
Design: Case Report
Setting: Tertiary Government Hospital
Results: A 25-year-old lady presented with a painless chronic submandibular swelling. Ultrasound identified a solid mass following which an uncomplicated core biopsy was performed obtaining an accurate pre-operative histopathological diagnosis. Pre-operative arterial embolization of this vascular mass led to a relatively bloodless wide local excision. Radiological imaging for distant metastases was negative.
Conclusion: Epitheloid Hemangioendothelioma is an uncommon cause for a submandibular mass. A malignant vascular soft tissue tumor with morphologic characteristics similar to carcinomas, melanomas and epitheloid sarcomas, it has a high rate of metastasis and morbidity when it affects the soft tissues and viscera. Immunohistochemistry provides clues to differentiation and recommended treatment consists of a surgical wide local excision with regional lymph node resection. As there are no established standard therapeutic protocols for this disease due to its rarity, an individual case-by-case approach and follow-up needs to be undertaken.