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A new association - multiple endocrine neoplasia type 1 and malignant peripheral nerve sheath tumor
Veronica Preda1, Mark Sywak2, Diana Learoyd1
1Department of Endocrinology, Royal North Shore Hospital St Leonards, Sydney, New South Wales, 2065, Australia ; Sydney Medical School, University of Sydney Sydney, New South Wales, 2006, Australia.
Abstract:
We report a patient with multiple endocrine neoplasia type 1 (MEN-1) and an aggressive malignant peripheral nerve sheath tumor (MPNST) arising from a ganglioneuroma of the adrenal gland. Patients with MEN-1 require careful consideration of other tumor associations, including MPNST, as it can portend a poor prognosis. MEN-1 and MPNST have not been reported. We report a patient with multiple endocrine neoplasia type 1 (MEN-1) and an aggressive malignant peripheral nerve sheath tumor (MPNST) arising from a ganglioneuroma of the adrenal gland. Patients with MEN-1 require careful consideration of other tumor associations, including MPNST, as it can portend a poor prognosis. MEN-1 and MPNST have not been reported.
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