Multidisciplinary surgical management of Cowden syndrome: report of a case
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Patini, Romeo; Staderini, Edoardo; Gallenzi, Patrizia
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Aquest document és un/a article, creat/da en: 2016
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Cowdenâ s Syndrome (CS) is a rare congenital autosomal dominant disorder that affects around 1/200000 patients
with an incomplete penetrance and variable expressivity, characterized by alterations in a tumor suppressor gene.
A 14-year-old Caucasian male patient came to the attention of the authors complaining of palm nodules, gingival
bleeding and painful pedunculated lesions on the lips and on the labial side of anterior sextants. After genetic investigation
the final diagnosis of a Cowden Syndrome was made. The lesions were surgically removed under general
anesthesia and no clinical signs of recurrence were found three months after surgical excision. Considering the
severe symptoms of the syndrome and the strong tendency to malignant development of the associated lesions all
clinicians should focus their efforts to the early diagnosis and, when possible, multidisciplinary treatment. |
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