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Revista Portuguesa de Pneumologia
Print version ISSN 0873-2159
Abstract
MARCAL, N Serrano et al. Malignant peripheral nerve sheath tumors: A case report. Rev Port Pneumol [online]. 2010, vol.16, n.3, pp.483-492. ISSN 0873-2159.
Malignant peripheral nerve sheath tumors comprehend a rare group of soft tissue sarcomas that tend to occur in patients with neurofibromatosis type 1 or several years after radiotherapy treatments. Its thoracic localization is a very unusual entity. The typical symptoms are due to nerve roots compression which can persist for several months or years before diagnosis. Due to very few patients with this type of tumor its therapeutic approach is still a matter of permanent debate, being surgery the main treatment. This tumor has a bad prognosis because of high local recurrence and metastasis. The clinical case we describe serves as a glimpse for discussion.
Keywords : Malignant tumour; peripheral nerve sheath.