¨A síndrome de Gorlin-Goltz (SGG) ou síndrome do nevo basocelular, é uma desordem rara autossômica dominante. Apesar de hereditária, há casos de mutação espontânea. Caracteriza-se pela predisposição cancerígena e várias manifestações clínicas. A SGG associa-se a achados clínicos classificados em critérios maiores: CBCs mínimo dois ou um em menores de 20 anos, tumor odontogênico ceratocístico, pits palmoplantares, calcificação ectópica intracraniana, história familiar de SGG; e menores: anomalisas craniofaciais, macrocefalia, fenda labial ou palatina, bossa frontal, hipertelorismo, anomalias esqueléticas, fibroma ovariano, meduloblastoma. Define SGG com a presença de dois critérios maiores ou um maior e dois menores. O tratamento é multidisciplinar dependendo das manifestações clínicas do pacientes e requer constante vigilância a novos achados clínicos.¨
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by Dr Paulo Fernando Leite
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