2003
DOI: 10.1007/bf02637324
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Malignant peripheral nerve sheath tumor in a patient with Werner's syndrome

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“…Mutations of other members of this family are seen in Bloom's syndrome and Rothmund–Thomson's syndrome, all of which are characterized by increased genomic instability and increased cancer predisposition (Sharma et al, 2020). While the association of Werner's syndrome with elevated risk of malignant tumors is widely reported in the literature, it is often associated with thyroid neoplasms, melanoma, and bone and soft tissue sarcomas, and only four cases of MPNST have been reported in the literature so far (Goto et al, 1996; Rosa et al, 2003). MPNST is extremely rare, but devastating tumor that originates in the nerve sheath with a 5‐year survival of 35%–60% (Ducatman et al, 1986; Stucky et al, 2011).…”
Section: Discussionmentioning
confidence: 99%
“…Mutations of other members of this family are seen in Bloom's syndrome and Rothmund–Thomson's syndrome, all of which are characterized by increased genomic instability and increased cancer predisposition (Sharma et al, 2020). While the association of Werner's syndrome with elevated risk of malignant tumors is widely reported in the literature, it is often associated with thyroid neoplasms, melanoma, and bone and soft tissue sarcomas, and only four cases of MPNST have been reported in the literature so far (Goto et al, 1996; Rosa et al, 2003). MPNST is extremely rare, but devastating tumor that originates in the nerve sheath with a 5‐year survival of 35%–60% (Ducatman et al, 1986; Stucky et al, 2011).…”
Section: Discussionmentioning
confidence: 99%