Resumo

LASSO-VAZQUEZ, J.M.; ARENAS-DOMINGO, L.; GARCIA RUANO, A.  e  PEREZ CANO, R.. Squamous cell carcinoma infiltrating skull base in 16 years old patient with oculo-cutaneous albinism. Cir. plást. iberolatinoam. [online]. 2013, vol.39, n.4, pp.341-347. ISSN 1989-2055.  https://dx.doi.org/10.4321/S0376-78922013000400004.

We present a 16 year-old girl from Africa, affected by an oculo-cutaneous albinism, who developed a squamous cell carcinoma of the right ear that was treated by radical surgery and reconstructed with a free TRAM flap. Albinism is a genetic metabolic disease that is characterized by an impossibility to produce melanin. It presents two variations: ocular and oculo-cutaneous. In some african countries, patients affected by the disease are socially rejected and they do not receive right medical treatments. For this reason the tumor was infiltrating very deeply, affecting the cranial base when our patient was visited in our hospital. The complex anatomy of the vital structures in this area makes surgical resection of tumors involving the skull base extremely difficult. We review some concepts about the treatment of lateral and temporal skull base tumors.

Palavras-chave : Albinism; Oculo-cutaneous albinism; Squamous cell carcinoma; Ear tumors; Skull base tumors.

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