Congenital macroglossal angiodysplasia ("Lymphangioendotheliomatosis")

A case of congenital lingual angiodysplasia with macroglossia in a 5-year-o ld girl is presented. A diffusely enlarged tongue was present at birth and continued to grow as the child aged. It was accompanied by impaired speech, difficulty in eating and breathing, and sleep apnea, necessitating surgica l intervention. The fundamental lesion represents a complex vascular malfor mation of the lymphangioma-hemangioma type, involving extensively the deep musculature of the tongue. Multifocal and multicentric cavernous lymphangio ma-like and hemangioma-like areas merge with benign angioendotheliomatous-l ike foci in a background of variable muscle degeneration and marked fibrosi s. Neither a borderline nor an overtly malignant vasoformative neoplasm was present. Because of its distinctively widespread, multicentric intramuscul ar distribution, this lesion may be construed as a diffuse variant of lingu al lymphangioma-hemangioma malformation, closely resembling a previously de scribed case of macroglossal lymphangioendotheliomatosis.