Regarding Neurogenic Malignant Tumors in the Mediastinum

Abstract

This letter reports on the rare presentation of a malignant neurogenic tumor in the phrenic nerve.

Key words

mediastinum (incl. mediastinoscopy) - mediastinal tumor - nerves - cranial or peripheral - mediastinum - neurogenic tumor - phrenic nerve

References

• 1 Huang T W, Yang M H, Cheng Y L, Tsai W C, Lee S C. Vagus nerve schwannoma in the middle mediastinum.  Thorac Cardiovasc Surg. 2010;  58 (5) 312-314
• 2 Park J H, Choi K H, Lee H B, Rhee Y K, Lee Y C, Chung M J. Intrathoracic malignant peripheral nerve sheath tumor in von Recklinghausen's disease.  Korean J Intern Med. 2001;  16 (3) 201-204
• 3 Shinozaki T, Ishii K, Tsubaki K, Ota Y, Adachi H. A case of malignant peripheral nerve sheath tumor requiring thoracotomy.  Nippon Jibiinkoka Gakkai Kaiho. 2003;  106 (12) 1139-1142
• 4 Kawachi R, Takei H, Furuyashiki G, Koshi-Ishi Y, Goya T. A malignant peripheral nerve sheath tumor of the mediastinum in a patient with neurofibromatosis type 1: report of a case.  Surg Today. 2008;  38 (10) 945-947
• 5 Hassoun P M, Celli B R. Bilateral diaphragm paralysis secondary to central von Recklinghausen's disease.  Chest. 2000;  117 (4) 1196-1200
• 6 Wasa J, Nishida Y, Tsukushi S et al. MRI features in the differentiation of malignant peripheral nerve sheath tumors and neurofibromas.  AJR Am J Roentgenol. 2010;  194 (6) 1568-1574
• 7 Leroy K, Dumas V, Martin-Garcia N et al. Malignant peripheral nerve sheath tumors associated with neurofibromatosis type 1: a clinicopathologic and molecular study of 17 patients.  Arch Dermatol. 2001;  137 (7) 908-913

Dear Author,

We would like to express our appreciation of the author's management of a malignant peripheral nerve sheath tumor (MPSNT) of the phrenic nerve in the mediastinum. We would also like to thank the author for his response to our report on a middle mediastinal vagal nerve schwannoma. Mediastinal nerve sheath tumors are rare. Unlike vagal nerve schwannomas, the incidence of MPSNT of the phrenic nerve is very low, and it is often associated with neurofibromatosis type 1 (NF1, von Recklinghausen's disease). Schwannomas are generally benign and slow-growing. Malignant schwannomas have also been reported, but they are rarer. Accurate diagnosis can only be made by thoracic exploration and excision. The diagnosis of MPNST is a pathological diagnosis of a malignant tumor originating from or differentiating toward cells of the peripheral nerve sheath with anaplastic features in the form of high cellularity, cellular and nuclear pleomorphism, a high mitotic rate and necrosis. The management of these tumors arising from intrathoracic vagus nerves consists primarily of surgical excision to prevent mass effect and the loss of host nerve function. Local recurrence is common after resection of the primary tumor. The prognosis for MPNST is poor. Aggressive surgical intervention and adjuvant radiotherapy may prolong the patient's overall survival.

Tsai-Wang Huang
Department of Thoracic Surgery
Tri-Service General Hospital
325, Cheng-Kung Road 2nd section
Taipei 114
Taiwan, R. O. C.
[e-mail]