RESUMO
Cowden's disease is a rare genodermatosis that is characterized for multiple cutaneous and visceral hamartoma . Lhermitte-Duclos's disease is a cerebelous lesion that consists in the displasic enlargement of the cerebelous circumvolution. It's incluyed in phacomatosis and usually presents associated to Cowden's disease, tuberous sclerosis and overlap syndromes.A 56 years old man, diagnosed in Dermatology with Cowden's disease ten years ago. In the extension study, he had hamartoma intestinal polip, esophagic glucogenic acanthosis and two solid thyroid nodules. The craneal TC didn't show significant alteration. Ten years after diagnosis a cerebral magnetis resonance was performed for intense cephalea, and it was found a bad-defined mass in right cerebelous hemisphere without contrast captation, compatible with cerebelous glangliocytoma.
Assuntos
Neoplasias Cerebelares/complicações , Ganglioneuroma/complicações , Síndrome do Hamartoma Múltiplo/complicações , Neoplasias Cerebelares/diagnóstico , Feminino , Ganglioneuroma/diagnóstico , Síndrome do Hamartoma Múltiplo/diagnóstico , Humanos , Imageamento por Ressonância Magnética , Masculino , Pessoa de Meia-IdadeRESUMO
La enfermedad de Cowden es una rara genodermatosis que se caracteriza por la aparición de multiples hamartomas tanto a nivel cutaneomucoso como visceral. Es una lesión cerebelosa que consiste en el engrosamiento displásico de las circunvoluciones cerebelosas. Se incluye dentro de las facomatosis y suele presentarse junto a la enfermedad de Cowden, la esclerosis tuberosa o síndromes de solapamiento. Presentamos un paciente de 56 años diagnosticado en la consulta de Dermatología de enfermedad de Cowden hace 10 años. En el estudio de extensión se le diagnostica de poliposis intestinal hamartomatosa, acantosis glucogénica esofágica y se encuentran dos nódulos sólidos tiroideos. A los 10 años del diagnóstico se realiza resonancia magnética nuclear cerebral por aparición de cefalea encontrando una masa mal definida en hemisferio cerebeloso derecho compatible con gangliocitoma displásico cerebeloso
Cowdens disease is a rare genodermatosis that is characterized for multiple cutaneus and visceral hamartoma . Lhermitte-Duclos´s disease is a cerebelous lesion that consists in the displasic enlargement of the cerebelous circumvolution. It´s incluyed in phacomatosis and ussually presents associated to Cowdens disease, tuberous sclerosis and overlap syndromes. A 56 years old man, diagnosed in Dermatology with Cowdens disease ten years ago. In the extension study, he had hamartoma intestinal polip, esophagic glucogenic acanthosis and two solid thyroid nodules. The craneal TC didn´t show significant alteration. Ten years after diagnosis a cerebral magnetis resonance was performanced for intense cephalea, and it was found a bad-defined mass in right cerebelous hemisphere without contrast captation, compatible with cerebelous glangliocytoma
