RESUMO
OBJECTIVE: To determine the epidemiology, histological diagnosis and treatment outcome of oro-facial tumours and tumour-like lesions in Greek children and adolescents. MATERIAL AND METHODS: The medical records of patients with oro-facial lesions who presented at the Department of Oral and Maxillofacial Surgery at "A. & P. Kyriakou" Children's Hospital from 2000 to 2010 were reviewed. Data was analyzed in relation to age, gender, location, histology, treatment choice and outcome. RESULTS: Two hundred and eleven oro-facial lesions were identified. Age ranged from 14 days to 15 years (mean 8 years); the male-to-female ratio was 1.09: 1; 90.05% of the lesions were benign and 9.95% malignant. Vascular anomalies were the most common benign lesion (22.1%) and rhabdomyosarcoma was the most prevalent malignancy (28.57%). One hundred and ten lesions (52.1%) involved soft tissue, most commonly the tongue and 96 cases (45.5%), involved hard tissue, most frequently the mandible; 5 lesions (2.4%) involved both hard and soft tissue. Surgery was performed under general anaesthesia in 198 cases (93.84%). Some malignant lesions were treated with chemotherapy and/or radiotherapy. CONCLUSIONS: Oro-facial tumours and tumour-like lesions are not uncommon in the Greek paediatric population; although most frequently benign, these may cause considerable morbidity. As such early diagnosis and treatment are imperative.
Assuntos
Neoplasias Faciais/epidemiologia , Neoplasias Bucais/epidemiologia , Adolescente , Criança , Pré-Escolar , Feminino , Seguimentos , Grécia/epidemiologia , Humanos , Lactente , Recém-Nascido , Tumores de Vasos Linfáticos/epidemiologia , Masculino , Neoplasias Mandibulares/epidemiologia , Terapia Neoadjuvante/estatística & dados numéricos , Neoplasias de Tecido Vascular/epidemiologia , Tumores Odontogênicos/epidemiologia , Procedimentos Cirúrgicos Bucais/estatística & dados numéricos , Estudos Retrospectivos , Rabdomiossarcoma/epidemiologia , Neoplasias de Tecidos Moles/epidemiologia , Neoplasias da Língua/epidemiologia , Resultado do TratamentoRESUMO
Weaver syndrome is a rare disorder, characterized by accelerated growth, advanced osseous maturation and distinct craniofacial features. Macroglossia and hypothyroidism are seldom mentioned in the literature as clinical findings associated with the syndrome. This paper describes a patient with Weaver syndrome, referred for consultation and treatment of macroglossia, who also suffered from congenital hypothyroidism. This is the first reported case of Weaver syndrome treated with partial glossectomy (tongue reduction). The paper describes the clinical findings of the syndrome, emphasizing the difficulty in identifying it, the indications for partial glossectomy and the authors' recommended operative technique.
