Article Abstract

Malignant peripheral nerve sheath tumor of the chest wall associated with neurofibromatosis: a case report

Authors: Chin-Chieh Hsu, Tsai-Wang Huang, Jane-Yi Hsu, Nieh Shin, Hung Chang


Primary chest wall tumors are relatively rare and represent approximately 5% of all thoracic neoplasms. Malignant peripheral nerve sheath tumor (MPNST) is highly aggressive and occurs in the second or third decade of patients with neurofibromatosis type 1 (NF-1). The estimated incidence of MPNST in patients with NF-1 is 2-5% (general population, 0.001%). This neoplasm usually affects the extremities and rarely the thoracic cavity. We present a case of MPNST of the chest wall in a patient with NF-1 who developed local recurrence 5 months after complete surgical resection and postoperative adjuvant radiotherapy.