Malignant Peripheral Nerve Sheath Tumor of the Superior Mediastinal Vagus Nerve in a Neurofibromatosis Type 1 Patient

Background. Patients with neurofibromatosis type 1 (NF1) have a 10% lifetime risk of developing malignant peripheral nerve sheath tumor (MPNST), and the outcome of MPNST, especially it arises in the trunk, is very poor. Case. We report a case of MPNST in a 34-year-old man with NF1. The patient was referred to our hospital with hoarseness, which a chest X-ray film and computed tomography (CT) revealed to be a large tumor in the right upper mediastinum. He underwent surgery via a partial upper sternotomy with right anterior thoracotomy. The tumor, which derived from the right vagus nerve, was 14×8 cm in size, and appeared pseudocapsulated. Severe adhesion to the trachea and the superior vena cava excluded the possibility of a wide margin resection, although the surgical margin was pathologically negative. In this patient, however, tumor recurred locoregionally without distant metastasis, 6 months after the operation. Both radiotherapy and chemotherapy with carboplatin/etoposide were ineffective, and he died 4 months after recurrence. Conclusion. It is important to consider if an inadequate marginal resection of MPNST requires additional adjunctive therapy.