NEUROGENIC TUMORS OF THE NECK

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The peripheral nervous system consists of the cranial and spinal nerves, the sympathetic and parasympathetic divisions of the autonomic nervous system, and the peripheral ganglia. Peripheral nervous system tumors arise from neural crest derivatives including Schwann cells, perineurial cells, and fibroblasts and include neurofibroma; schwannoma (neurilemmoma and neurinoma); and malignant peripheral nerve sheath tumor (MPNST) (neurogenic sarcoma, anaplastic neurofibroma, and malignant schwannoma). Many peripheral nerve sheath tumors express Schwann cell characteristics, including the presence of S-100 antigen.15 An increased incidence of peripheral nervous system tumors is associated with the heritable neurofibromatosis (NF) syndromes. Although two distinct types of NF (types 1 and 2, see later) have been well described, some authors have proposed up to nine different varieties.20

Neoplasms that arise from primordial neural crest cells include neuroblastoma, ganglioneuroblastoma, and ganglioneuroma. Neuroblastoma is the least differentiated of these tumors and contains small primitive-appearing cells known as neuroblasts. With increasing differentiation, a proportion of larger ganglion cells and Schwann cells may be found (ganglioneuroblastoma) and the term ganglioneuroma is designated when neuroblasts are no longer seen.32 Paragangliomas are tumors of paraganglionic tissue that are composed of neuroendocrine tissues. These are dealt with elsewhere in this issue. The different types of neurogenic tumors are listed next:

  • Neurogenic Tumors

  • Schwannoma

  • Neurofibroma

    • Solitary

    • Neurofibroma in NF1

    • Plexiform neurofibroma

    • Diffuse

  • MPNST

  • Neural Tumors

  • Neuroblastoma

  • Ganglioneuroblastoma

  • Ganglioneuroma

Neuroma is a term usually used to denote an exaggerated response to neuronal injury in which a tangle of regenerating axons, fibrous tissue, and Schwann cells form at the site of a severed nerve. Multiple mucosal and cutaneous neuromas with hypertrophy of peripheral nerves occur in multiple endocrine neoplasia type IIB.29 Marfanoid body habitus, pheochromocytomas, and medullary carcinoma of the thyroid gland also characterize this condition.

Section snippets

Schwannoma

Schwannomas are benign tumors arising from Schwann cells surrounding peripheral nerves. These neoplasms account for approximately 5% of all benign soft tissue tumors.25 Although most schwannomas are sporadic, they are seen in approximately 90% of individuals with NF type 2 (NF2).38 Schwannomas involving the head and neck in NF2 are most commonly intracranial, however, and usually involve the vestibular nerves. Twenty-five percent to 45% of extracranial schwannomas occur in the head and neck

PRIMITIVE NEURAL TUMORS

Primitive neural tumors are derived from primordial neural crest cells that migrate from the mantle layer of the developing spinal cord to the sympathetic ganglia, adrenal medulla, and other sites. These have a common denominator cell and are thought to represent different stages of maturation with the neuroblastoma representing the poorly differentiated.19 The three classic histopathologic patterns of neuroblastoma, ganglioneuroblastoma, and ganglioneuroma reflect a spectrum of maturation and

SUMMARY

Neurogenic tumors of the neck occur in children and adults. Important parameters to aid in the differential diagnosis are age at presentation, location, and a history of NF or multiple endocrine neoplasia.

Schwannoma is the most common solitary neurogenic tumor in the neck and is usually seen in patients between 20 and 50 years of age. The plexiform neurofibroma and multiple localized neurofibromas are characteristic of NF1. MPNSTs are uncommon aggressive lesions that can arise de novo in

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    Address reprint requests to Alfred L. Weber, MD, Department of Radiology, Massachusetts Eye and Ear Infirmary, 243 Charles Street, Boston, MA 02117, e-mail: [email protected]

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