Neurogenic tumors arise in tissues derived from the embryonic neural crest. Further classification is based on whether the tumor cell originates from nerve sheath, nerve cells (ganglia), paraganglia, or peripheral nerve (Table 138-1). In the thorax, the latter nerve is represented by the intercostal nerve. The chief impediment to understanding thoracic neurogenic tumors is the lack of uniformity in the nomenclature used in the published literature, and thus multiple descriptors exist at each taxonomic level (Table 138-2). This chapter relies on the nomenclature endorsed by the most recent revision of the World Health Organization classification of tumors derived from neural tissue.1
Table 138-1. Classification of Neurogenic Tumors
Table 138-2. Neurogenic Tumor Nomenclature |Favorite Table|Download (.pdf)
Table 138-2. Neurogenic Tumor Nomenclature
Also Known As
Schwannoma, neurilemoma, neurinoma
Granular cell tumor
Malignant peripheral nerve sheath tumor (MPNST)
Malignant schwannoma, neurosarcoma, neurofibrosarcoma, malignant fibroma, malignant tumor of nerve sheath origin (MTNSO)
Primitive neuroectodermal tumor (PNET)
Peripheral neuroectodermal tumor, peripheral neuroepithelial tumor, Askin tumor
Neural crest-derived tissues can be found throughout the body. In the thorax, neurogenic tumors are found most commonly in the posterior mediastinum (63–96%).1–6 In fact, neurogenic tumors account for 75% of all posterior mediastinal neoplasms.3 The epidemiology of neurogenic tumors depends primarily on whether the patient is an adult or a child. Whereas one-third of mediastinal tumors diagnosed and treated in children are neurogenic, the incidence is only 12–14% in adults.7,8 Adults also have a lower rate of malignancy (5–10% in adults compared with 40–60% in children)1,7 (Fig. 138-1). The most common neurogenic tumors in adults arise from the nerve sheath (e.g., neurilemoma and neurofibroma), whereas in pediatric populations the cells of origin are the ganglia (e.g., ganglioneuroma and neuroblastoma)1,9 (Table 138-3).
Malignancy rate according to patient's age. (Reproduced with permission from Takeda S, Miyoshi S, Minami M, Matsuda H: Intrathoracic neurogenic tumors: 50 years' experience in a Japanese institution. Eur J Cardiothorac Surg 26:807–12, 2004.)
Table 138-3. Comparison of Children and Adults for Histologic Type of Neurogenic Tumor |Favorite Table|Download (.pdf)
Table 138-3. Comparison of Children and Adults for Histologic Type of Neurogenic Tumor
Adults, 86 Cases
Children, 60 Cases
Benign/Malignant, 81/5 (5.8%)
Benign/Malignant, 35/25 (41.7%)
18 (M/F: 8/10)
33 (M/F: 17/16)
37 (M/F: 21/16)
28 (M/F: 16/12)
2 (M/F: 0/2)
18 (M/F: 8/10)
5 (M/F: 1/4)
2 (M/F: 1/1)...
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