Malignant peripheral nerve sheath tumor in a patient with Werner's syndrome

被引:0
作者
M. A. Rosa
G. Falcone
V. De Santis
O. Azzaro
机构
[1] Orthopaedics Department, University of Messina, I-98100 Messina, Via Consolare Valeria
关键词
MPNST; Soft tissue sarcomas; Tumor; Werner;
D O I
10.1007/s10195-003-0018-1
中图分类号
学科分类号
摘要
Werner's syndrome is a rare autosomal recessive disorder characterized by features of premature aging. It is attributed to an autosomal recessive mutation on chromosome 8p12 where the Werner gene is located. This gene encodes a protein homologous to the RecQ-type DNA helicase, an enzyme that unwinds double-stranded DNA into single-stranded DNA. The association of Werner's syndrome with malignant tumors is widely reported in the literature. Both bone and soft tissue sarcomas have been described. We report a patient with Werner's syndrome who developed a malignant peripheral nerve sheath tumor of the left popliteal fossa.
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页码:95 / 99
页数:4
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