Multiple oral fibropapillomatosis as an initial manifestation of Cowden Syndrome. Case report.

Cowden syndrome is a rare hereditary disease included within hamartoma-type gastrointestinal polyposis. It is characterised by associated mucocutaneous anomalies and by the extraordinary tendency to develop malignant neoplasia, mainly in the breast and thyroid. Early diagnosis of the syndrome and adequate tumoral screening in patients with mucocutaneous papillomatosis make it possible to make an earlier diagnosis of associated pathologies which have great morbidity when detected late. We present the case of a patient diagnosed with Cowden syndrome after consultation for labial papillomatous lesions of long evolution who was subsequently treated for breast and kidney cancer in initial stages. The correct diagnosis of a banal pathology of oral mucosa made it possible for us to take early action against the neoplastic pathology associated with this disease.

Fibropapilomatosis oral múltiple como manifestación inicial de Síndrome de Cowden. Caso clínico / Multiple oral fibropapillomatosis as an initial manifestation of Cowden Syndrome. Case report

El síndrome de Cowden es una infrecuente enfermedad hereditaria englobada dentro de las poliposis gastrointestinales de tipo hamartomatoso. Se caracteriza por asociar anomalías cutaneomucosas y por la extraordinaria tendencia a desarrollar neoplasias malignas, principalmente de mama y tiroides. La importancia de un diagnóstico precoz del síndrome y de un adecuado screening tumoral en pacientes con lesiones papilomatosas cutaneomucosas, nos va a permitir adelantarnos en el diagnóstico de patologías con enorme morbimortalidad asociada en caso de una detección tardía. Presentamos el caso de una paciente diagnosticada de síndrome de Cowden tras consultar por lesiones papilomatosas labiales de largo tiempo de evolución y tratada posteriormente de cáncer de mama y riñón en estadios iniciales. El correcto diagnóstico de una patología banal de mucosa oral, nos ha permitido actuar de manera muy precoz frente a la patología neoplásica asociada a dicha enfermedad

Cowden syndrome is a rare hereditary disease included within hamartoma-type gastrointestinal polyposis. It is characterisedby associated mucocutaneous anomalies and by the extraordinary tendency to develop malignant neoplasia, mainly in the breast and thyroid. Early diagnosis of the syndrome and adequate tumoral screening in patients with mucocutaneous papillomatosis make it possible to make an earlier diagnosis of associated pathologies which have great morbidity when detected late. We present the case of a patient diagnosed with Cowden syndrome after consultation for labial papillomatous lesions of long evolution who was subsequently treated for breast and kidney cancer in initial stages.The correct diagnosis of a banal pathology of oral mucosa made it possible for us to take early action against the neoplasticpathology associated with this disease