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)...
Log In to View More
If you don't have a subscription, please view our individual subscription options below to find out how you can gain access to this content.
Want remote access to your institution's subscription?
Sign in to your MyAccess profile while you are actively authenticated on this site via your institution (you will be able to verify this by looking at the top right corner of the screen - if you see your institution's name, you are authenticated). Once logged in to your MyAccess profile, you will be able to access your institution's subscription for 90 days from any location. You must be logged in while authenticated at least once every 90 days to maintain this remote access.
If your institution subscribes to this resource, and you don't have a MyAccess profile, please contact your library's reference desk for information on how to gain access to this resource from off-campus.
AccessSurgery Full Site: One-Year Subscription
Connect to the full suite of AccessSurgery content and resources including more than 160 instructional videos, 16,000+ high-quality images, interactive board review, 20+ textbooks, and more.
Pay Per View: Timed Access to all of AccessSurgery
24 Hour Subscription $34.95
48 Hour Subscription $54.95
Pop-up div Successfully Displayed
This div only appears when the trigger link is hovered over.
Otherwise it is hidden from view.