Solitary Primary Intraosseous Angiosarcoma of the Mandible

Angiosarcoma is a malignant vascular tumor derived from the mesenchymal cells which have undergone an angioblastic differentiation. About half of the angiosarcomas are reported to localize in the skin and soft tissues of the head and neck, i.e., found predominantly to arise from the scalp and face. Angiosarcomas rarely involve the skeletal system, wherein they generally have a tendency for multicentricity. Primary bony angiosarcoma accounts for about 6% of all the angiosarcomas and about 1.4% of all the primary bony sarcomas. A majority of angiosarcomas are reported to occur in the elderly males; its occurrence in the pediatric population is extraordinarily rare. We report a rare case of a solitary primary intraosseous mandibular angiosarcoma in a 7-year-old male child. The unique clinical presentation, the histopathological features along with the management challenges of this fascinating yet aggressive tumor is discussed.