An intrathoracic malignant peripheral nerve sheath tumor in a neurofibromatosis type 1 patient / 대한내과학회지

ABSTRACT

A malignant peripheral nerve sheath tumor (MPNST) is a rare soft tissue tumor defined as any malignant tumor arising from or differentiating toward the cells of the peripheral nerve sheath. It is one of the malignant tumors associated with neurofibromatosis type 1 (von Recklinghausen's disease). They occur most commonly on the lower and upper extremities, trunk, head, and neck, while intrathoracic tumors are very rare. We report an intrathoracic MPNST in a 66-year-old female with neurofibromatosis type 1.