Malignant peripheral nerve sheath tumour of the hand

摘要

Dear Sir, The term malignant peripheral nerve sheath tumour (MPNST) refers to any malignant tumour arising from a peripheral nerve showing nerve sheath cell type differentiation. Malignant peripheral nerve sheath tumours are rare, with an incidence of 0.001% in the general population. They often have a history of slow growth over a long period, followed by a period of rapid growth. Malignant transformation from pre-existing plexiform neurofibromas or previously normal peripheral nerve has been reported (Ducatman et al., 1986). They occur with equal frequency in men and women, with peak incidence in the seventh decade of life in the general population but in the third or fourth decade in people with neurofibromatosis. However, they can occur at a much younger age in either group (Ducatman et al., 1986). MPNSTs are extremely rare in the distal part of a limb (Cashen et al., 2004).