- Case report
- Open Access
Malignant schwannoma of the upper mediastinum originating from the vagus nerve
© Shoji et al; licensee BioMed Central Ltd. 2005
Received: 03 June 2005
Accepted: 06 October 2005
Published: 06 October 2005
Malignant schwannoma of the upper mediastinum originating from the vagus nerve is extremely rare.
A 46-year-old female was admitted for a left cervical mass which was associated with both hoarseness and Horner's syndrome. Chest computed tomography showed a mass extending from the left upper mediastinum to the left supraclavicular area. A fine needle aspiration cytological examination suggested primary lung cancer stage IIIB large cell carcinoma. After administering induction chemo-radiotherapy, a complete surgical resection was performed. The tumor was found to involve both the left vagus nerve and the left sympathetic nerve. Histological examination of the resected specimen revealed the tumor to be malignant schwannoma.
Despite incorrect preoperative diagnosis, the multimodality treatment administered in this case, including induction chemo-radiotherapy and surgery, proved to be effective.
According to a collected series of 2399 cases of mediastinal tumors reported in the literature , 496 cases (20.7%) were of neurogenic tumors, and most of them occurred in the posterior mediastinum. Neurogenic tumors can be divided into two groups depending on their origin: those that arise from the nerve sheath and those that arise from nerve cells. The majority of the tumors of nerve sheath origin in adults are either benign schwannomas or neurofibromas, and they usually arise from either an intercostal nerve or a sympathetic nerve. Intrathoracic schwannoma originating from the vagus nerve, is extremely rare.
Tumors of vagus nerve origin are observed in about 2% of all neurogenic tumors of the mediastinum , however, no instace of malignant schwannoma was reported in this review. To our knowledge, only a few such cases have been previously reported [5–7]. As a result, malignant schwannoma originating from the vagus nerve is therefore considered to be extremely rare.
Malignant peripheral nerve sheath tumors (MPNST) including malignant schwannoma are the malignant variants of schwannomas and neurofibromas. Although the 5-year survival rates have been reported to be up to 75 % in MPNST's patients, MPNST often advance locally and can also occasionally metastasize to the lung or other organs . Therefore, in addition to a complete surgical resection, adjuvant therapy is usually advocated. However, in an adjuvant setting, the efficacy of chemotherapy or radiotherapy appears to provide little additional benefit [9, 10]. We previously reported concurrent chemo-radiotherapy with UFT plus cisplatin as an induction treatment followed by a surgical resection for patients with marginally resectable stage IIIB NSCLC to be both a feasible and promising treatment . Since we preoperatively considered the disease to be marginally resectable stage IIIB NSCLC, we performed concurrent induction chemo-radiotherapy followed by surgery. As a result, this multimodality treatment proved to be effective and the patient is now doing well without any recurrence.
We initially misdiagnosed this patient's disease to be non-small cell lung cancer. The reason for this was partly due to the cytological findings which indicated undifferentiated carcinoma. In general, an exact diagnosis cannot always be made based on the findings of aspiration cytology alone. The second reason for a misdiagnosis in this case was due to the patient's symptoms which included hoarseness and Horner's syndrome. Apical lung cancer involving both the vagus and the sympathetic nerve is occasionally observed. However, to the best of our knowledge, the present case is considered to be the first case demonstrating malignant schwannoma of the vagus nerve involving the sympathetic nerve.
Malignant schwannoma of the upper mediastinum arising from the vagus nerve is rare. The multimodality treatment administered in this case, including induction chemo-radiotherapy and surgery, proved to be effective.
We thank Mr. Brian Quinn for critical comments on the manuscript.
Written consent was obtained from the patient for the publication of this case.
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