[Maxillary epithelioid hemangioendothelioma: case report and review of the literature]. / L'hémangioendothéliome épithélioïde du maxillaire : à propos d'un cas et revue de la littérature.

ABSTRACT

INTRODUCTION: Epitheloid hemangioendothelioma is a rare vascular tumor with a borderline malignancy and a potential risk for recurrence. It usually affects the soft tissue of extremities, the liver, and the lungs. The cervicofacial localization is rare. We report a very rare maxillary localization. CASE REPORT An 8-year-old girl with no personal or family history was sent to us by her dentist for a painless ulceration of the right maxilla having developed months before. There was no cervicofacial lymphadenopathy. The orthopantomography was normal. CT scan revealed palatine osteolysis under the ulceration. Biopsy proved the diagnosis of an epitheloid hemangioendothelioma. Screening for metastases was negative. The final treatment was partial maxilectomy 11 months later. No sign of locoregional recurrence was observed. DISCUSSION: Twenty-six cases of oral epitheloid hemangioendothelioma have been reported. The localizations are mostly gingival and lingual. The gingival localization is more frequent in children. The small number of reported cases does not allow determining the risk of locoregional recurrence or metastasis. There is no consensus on treatment. Like other authors, we suggest performing a wide local excision and careful follow-up.