Macroglossal Lymphangioendotheliomatosis: Report of a Case in a Pregnant Woman

• An unusual case of adult-onset lymphangioendotheliomatous macroglossia occurred in a 37-year-old woman. In addition to a dramatic acceleration of the macroglossia during her third pregnancy, the patient exacerbated the macroglossia by Valsalva's maneuvers and voluntary tongue protrusion. Bed rest and head elevation could improve the macroglossia within 24 hours. Preservation of an adequate airway during a life-threatening state of proliferation and expansion of the lymphangiomatous lesions required surgical intervention. The patient's lesion differed clinically and pathologically from usual glossal lymphangioma, hemangioma, pregnancy gingival tumor, and hemangiomatosis. Pathologically, multifocal, multicentric areas of cavernous hemangiomatouslike, cavernous lymphangiomatouslike, and angioendotheliomatous areas were present within the deep musculature of the tongue, minor salivary tissue, and soft tissue of the floor of the mouth. (Arch Otolaryngol1984;110:600-603)