Korean Journal of Medicine  2009;76(2):234-237

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

Jin Yi CHOI 1 ; Jeong Hun KIM ; Ja Sung CHOI ; Jae Ho CHUNG ; Jeong Eun CHOI ; Hwa Eun OH ; Chan Sub PARK

Affiliations

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Keywords

Neurofibromatosis; Intrathoracic; Malignant peripheral nerve sheath tumor

Country

Republic of Korea

Language

Korean

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.