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Vol.50 No.2 contents | Japanese/English |
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- Case Report -
Malignant Peripheral Nerve Sheath Tumor of the Superior Mediastinal Vagus Nerve in a Neurofibromatosis Type 1 Patient
Kikuo Shigemitsu1, Gen Ito2, Akira Shiraki2, Takashi Abe2, Morihide Ando2, Joh Shindo21Department of Chest Surgery, 2Department of Pneumology, Ogaki Municipal Hospital, Japan
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.
key words: Mediastinal tumor, Malignant peripheral nerve sheath tumor, Vagus nerve, Neurofibromatosis type 1
Received: October 30, 2009
Accepted: January 5, 2010
JJLC 50 (2): 157-161, 2010
