Sindrome de gorlin (sindrome de carcinoma basocelular nevoide). Presentacion de dos casos y revision de la literatura
Por:
Meneses Garcia A., Hernandez V.J., Miranda Linares J., Acosta Mondragon H., Blanco M.A., Suarez Roa M.D.L., Ruiz-Godoy Rivera L.M.
Publicada:
1 ene 1998
Resumen:
The nevoid basal cell carcinoma syndrome was described four decades ago by Gorlin and Goltz and characterized by multiple basal cell carcinomas in or not exposure areas to sun. Besides the patients who course with this disorder have jaw cysts, skeletal anomalies (bifid ribs), palmo-plantar pits, ectopic calcifications. This syndrome is associated with another alterations on the face, skin, musculoskeletal anomalies, genitourinary abnormalities, neurologic manifestations, ophthalmologic abnormalities and some neoplasms including Hodgkin and no-Hodgkin's lymphoma, medulloblastoma, fibrosarcoma and ovarian fibromas, melanomas and fetal rhabdomyoma. It is established for the diagnosis of Gorlin Syndrome two main features considered major criteria or one major and two minor abnormalities. Some studies have demonstrated that this syndrome is an autosomal dominant disorder on chromosome 9 which is a relevant early event in tumorigenesis. This present serie shows two cases of nevoid basal cell carcinoma syndrome, including a full familiar study finding another eight more family members with the Gorlin Syndrome.
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