A six-year-old @entity1 is presented with an increasing mass involving the anterior vestibule and the floor of the mouth. Histologic and immunohistochemical examination revealed a poorly differentiated @entity1822 with destruction of the mandible. According to the CWS-96-study the @entity1 underwent preoperative chemotherapy followed by complete resection of the mandibular body with the surrounding soft tissues. Recurrent @entity5 became evident only three months later on. The further treatment consisted of @entity5 resection, oral chemotherapy and irradiation. @entity5 control could not be achieved. The @entity1 died of progressive disease 16 months after diagnosis. @entity1822 is extremely uncommon in childhood, especially with localisation in the oral cavity. Diagnosis is based on histologic examination and immunohistochemistry. In the presented case the lack of smooth muscle actin expression made diagnosis difficult. The preoperative chemotherapy could not achieve reduction of @entity5 size, so that extensive surgery became necessary. Estimation of the prognosis of the @entity1822 in childhood is difficult. High grade @entity5 and involvement of bone seem to be associated with bad clinical outcome. The presented case is confirming that.

[Epitheloid XXXX in childhood].

@entity1 :: ('9606', 'Species') :: ['girl', 'patient'] @entity1822 :: ('MESH:D007890', 'Disease') :: ['oral leiomyosarcomas', 'Leiomyosarcoma', 'epitheloid leiomyosarcoma'] @entity5 :: ('MESH:D009369', 'Disease') :: ['tumor', 'Nevertheless tumor', 'tumours']
@entity1822 :: (MESH:D007890,Disease) :: ['leiomyosarcoma of the mouth']
