Nerve sheath tumor

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Nerve sheath tumors
Nerve sheath tumors.webp
Illustration of typical Schwannoma (a.) and Neurofibroma (b.) configurations around nerves.
Source: Chengazi, H.U., Bhatt, A.A. Pathology of the carotid space. Insights Imaging 10, 21 (2019). https://doi.org/10.1186/s13244-019-0704-z
Specialty Neuro-oncology

A nerve sheath tumor is a type of tumor of the nervous system (nervous system neoplasm) which is made up primarily of the myelin surrounding nerves. Nerve sheath tumors can be benign or malignant, and may affect both the peripheral and central nervous systems. There are three main types of nerve sheath tumors: schwannomas, neurofibromas, and malignant peripheral nerve sheath tumors. [1]

Contents

Classification of nerve sheath tumors

Spinal nerve sheath tumors

Spinal nerve sheath tumors are typically intradural, meaning that they arise inside the dura mater surrounding the spinal cord (thecal sac), but may also be found in other areas of the spine. [2] Spinal nerve sheath tumors generally arise as single lesions. [1] Presence of multiple lesions is associated with genetic conditions including neurofibromatosis type 1, neurofibromatosis type 2, and Schwannomatosis. [1] [3] [4]

Most spinal schwannomas are intradural-extramedullary, growing inside the thecal sac, but outside the spinal cord itself. [5] Intradural-intramedullary schwannomas also develop, but tend to be rare. [5]

Peripheral nerve sheath tumors

A peripheral nerve sheath tumor is a nerve sheath tumor in the peripheral nervous system. Benign peripheral nerve sheath tumors include schwannomas and neurofibromas. A malignant peripheral nerve sheath tumor is a cancerous peripheral nerve sheath tumor, which is frequently resistant to conventional treatments.

Symptoms

Spinal and peripheral nerve sheath tumors may lead to a variety of symptoms depending on tumor type, location, and severity, though they do share some symptoms. [1]

Some common findings for all nerve sheath tumors include:

Many individuals are also asymptomatic. [1] [6]

Mechanism

The primary Schwann cell differentiation and neoplastic proliferations are characteristics of peripheral nerve sheath tumors. For instance, the Schwann cell, which is the major neoplastic cell component of neurofibroma, [7] is cytologically distinguished by the expression of S-100 protein and wavy nuclear outlines. [8] [9] A variety of peripheral nerve cells, including axons, perineurial cells, fibroblasts, and varying inflammatory components such as mast cells and lymphocytes, are also present in neurofibromas. A population of CD34-positive cells with an unknown histogenesis is also found. [10] [11]

Diagnosis

Spinal nerve sheath tumors

Magnetic resonance imaging (MRI) is typically used in spinal nerve sheath tumor diagnosis. [1] Each nerve sheath tumor type has a few different associated features on MRI imaging. Neurofibromas and malignant peripheral nerve sheath tumors can be difficult to distinguish from each other and may require additional testing, including PET scans (18FDG-PET). [1] Image-guided needle biopsies may be performed if there is concern for malignancy. [1]

Peripheral nerve sheath tumors

MRI, tissue, and nerve biopsies are also frequently used in diagnosis of peripheral nerve sheath tumors. [12] Computerized tomography (CT) scans, electromyography, and nerve conduction studies are other options. [12]

Management

Benign spinal nerve sheath tumors

Treatment of spinal nerve sheath tumors typically depends on presence and severity of symptoms. [1] For asymptomatic or incidental schwannomas or neurofibromas, only continued imaging is typically indicated to assess whether tumor growth is occurring. [1] Surgical resection may be an option for tumors causing extensive radicular pain or other symptoms, and for tumors exhibiting aggressive behavior. [1] Plexiform neurofibromas (associated with neurofibromatosis type 1) have a higher risk of transforming into malignant tumors and require individual treatment plans. [1] Surgical resection is the treatment of choice for symptomatic plexiform neurofibromas, though a new drug, selumetinib was approved in 2020, as a systemic therapeutic for inoperable cases in pediatric patients. [1] [13] Stereotactic body radiotherapy (SBRT) is a promising treatment option for benign spinal nerve sheath tumors, though more research is currently needed to determine effectiveness across different tumor types and to establish adequate clinical guidelines. [1]

Malignant spinal nerve sheath tumors

Complete surgical resection is the current treatment of choice for malignant spinal nerve sheath tumors. [1] Post-surgical radiotherapy has shown some promise in improving recurrence-free survival in intermediate and high grade tumors. [1] Chemotherapy for malignant spinal nerve sheath tumors has shown mixed results and is typically only used in patients in which surgery is not an option, or with aggressive or metastatic disease. [1]

Benign peripheral nerve sheath tumors

As with spinal nerve sheath tumors, continuous monitoring for asymptomatic, incidental, or slow growing tumors is generally the standard of care for peripheral nerve sheath tumors. [12] Management of symptomatic tumors is also similar to spinal nerve sheath tumors. [12] A complete surgical resection of peripheral tumors is typically done whenever indicated, and if possible. [12] Gamma knife radiosurgery, a subset of SBRT, is an option for peripheral nerve sheath tumors growing near the brain. [12]

Related Research Articles

<span class="mw-page-title-main">Brain tumor</span> Neoplasm in the brain

A brain tumor occurs when abnormal cells form within the brain multiplies. There are two main types of tumors: malignant (cancerous) tumors and benign (non-cancerous) tumors. These can be further classified as primary tumors, which start within the brain, and secondary tumors, which most commonly have spread from tumors located outside the brain, known as brain metastasis tumors. All types of brain tumors may produce symptoms that vary depending on the size of the tumor and the part of the brain that is involved. Where symptoms exist, they may include headaches, seizures, problems with vision, vomiting and mental changes. Other symptoms may include difficulty walking, speaking, with sensations, or unconsciousness.

<span class="mw-page-title-main">Neurofibromatosis</span> Three genetic disorders involving benign tumors of the nervous system

Neurofibromatosis (NF) refers to a group of three distinct genetic conditions in which tumors grow in the nervous system. The tumors are non-cancerous (benign) and often involve the skin or surrounding bone. Although symptoms are often mild, each condition presents differently. Neurofibromatosis type I (NF1) is typically characterized by café au lait spots, neurofibromas, scoliosis, and headaches. Neurofibromatosis type II (NF2), on the other hand, may present with early-onset hearing loss, cataracts, tinnitus, difficulty walking or maintaining balance, and muscle atrophy. The third type is called schwannomatosis and often presents in early adulthood with widespread pain, numbness, or tingling due to nerve compression.

<span class="mw-page-title-main">Nervous tissue</span> Main component of the nervous system

Nervous tissue, also called neural tissue, is the main tissue component of the nervous system. The nervous system regulates and controls body functions and activity. It consists of two parts: the central nervous system (CNS) comprising the brain and spinal cord, and the peripheral nervous system (PNS) comprising the branching peripheral nerves. It is composed of neurons, also known as nerve cells, which receive and transmit impulses to and from it, and neuroglia, also known as glial cells or glia, which assist the propagation of the nerve impulse as well as provide nutrients to the neurons.

Spinal tumors are neoplasms located in either the vertebral column or the spinal cord. There are three main types of spinal tumors classified based on their location: extradural and intradural. Extradural tumors are located outside the dura mater lining and are most commonly metastatic. Intradural tumors are located inside the dura mater lining and are further subdivided into intramedullary and extramedullary tumors. Intradural-intramedullary tumors are located within the dura and spinal cord parenchyma, while intradural-extramedullary tumors are located within the dura but outside the spinal cord parenchyma. The most common presenting symptom of spinal tumors is nocturnal back pain. Other common symptoms include muscle weakness, sensory loss, and difficulty walking. Loss of bowel and bladder control may occur during the later stages of the disease.

<span class="mw-page-title-main">Neurofibromatosis type I</span> Type of neurofibromatosis disease

Neurofibromatosis type I (NF-1), or von Recklinghausen syndrome, is a complex multi-system human disorder caused by the mutation of neurofibromin 1 (NF-1). NF-1 is a gene on chromosome 17 that is responsible for production of a protein (neurofibromin) which is needed for normal function in many human cell types. NF-1 causes tumors along the nervous system that can grow anywhere on the body. NF-1 is one of the most common genetic disorders and is not limited to any person's race or sex. NF-1 is an autosomal dominant disorder, which means that mutation or deletion of one copy of the NF-1 gene is sufficient for the development of NF-1, although presentation varies widely and is often different even between relatives affected by NF-1.

<span class="mw-page-title-main">Malignant peripheral nerve sheath tumor</span> Cancer of the connective tissue surrounding peripheral nerves

A malignant peripheral nerve sheath tumor (MPNST) is a form of cancer of the connective tissue surrounding peripheral nerves. Given its origin and behavior it is classified as a sarcoma. About half the cases are diagnosed in people with neurofibromatosis; the lifetime risk for an MPNST in patients with neurofibromatosis type 1 is 8–13%. MPNST with rhabdomyoblastomatous component are called malignant triton tumors.

<span class="mw-page-title-main">Neurofibromatosis type II</span> Type of neurofibromatosis disease

Neurofibromatosis type II is a genetic condition that may be inherited or may arise spontaneously, and causes benign tumors of the brain, spinal cord, and peripheral nerves. The types of tumors frequently associated with NF2 include vestibular schwannomas, meningiomas, and ependymomas. The main manifestation of the condition is the development of bilateral benign brain tumors in the nerve sheath of the cranial nerve VIII, which is the "auditory-vestibular nerve" that transmits sensory information from the inner ear to the brain. Besides, other benign brain and spinal tumors occur. Symptoms depend on the presence, localisation and growth of the tumor(s). Many people with this condition also experience vision problems. Neurofibromatosis type II is caused by mutations of the "Merlin" gene, which seems to influence the form and movement of cells. The principal treatments consist of neurosurgical removal of the tumors and surgical treatment of the eye lesions. Historically the underlying disorder has not had any therapy due to the cell function caused by the genetic mutation.

<span class="mw-page-title-main">Neurofibroma</span> Benign nerve-sheath tumor in the peripheral nervous system

A neurofibroma is a benign nerve-sheath tumor in the peripheral nervous system. In 90% of cases, they are found as stand-alone tumors, while the remainder are found in persons with neurofibromatosis type I (NF1), an autosomal-dominant genetically inherited disease. They can result in a range of symptoms from physical disfiguration and pain to cognitive disability.

Phakomatoses, also known as neurocutaneous syndromes, are a group of multisystemic diseases that most prominently affect structures primarily derived from the ectoderm such as the central nervous system, skin and eyes. The majority of phakomatoses are single-gene disorders that may be inherited in an autosomal dominant, autosomal recessive or X-linked pattern. Presentations may vary dramatically between patients with the same particular syndrome due to mosaicism, variable expressivity, and penetrance.

<span class="mw-page-title-main">Ganglioneuroma</span> Benign tumor of the autonomic nervous system

Ganglioneuroma is a rare and benign tumor of the autonomic nerve fibers arising from neural crest sympathogonia. However, ganglioneuromas themselves are fully differentiated neuronal tumors that do not contain immature elements.

<span class="mw-page-title-main">Schwannomatosis</span> Rare genetic disorder

Schwannomatosis is an extremely rare genetic disorder closely related to the more-common disorder neurofibromatosis (NF). Originally described in Japanese patients, it consists of multiple cutaneous schwannomas, central nervous system tumors, and other neurological complications, excluding hallmark signs of NF. The exact frequency of schwannomatosis cases is unknown, although some populations have noted frequencies as few as 1 case per 1.7 million people.

<span class="mw-page-title-main">Schwannoma</span> Benign nerve-sheath tumor composed of Schwann cells

A schwannoma is a usually benign nerve sheath tumor composed of Schwann cells, which normally produce the insulating myelin sheath covering peripheral nerves.

<span class="mw-page-title-main">Granular cell tumor</span> Medical condition

Granular cell tumor is a tumor that can develop on any skin or mucosal surface, but occurs on the tongue 40% of the time.

Malignant triton tumor (MTT) is a relatively rare, aggressive tumor made up of both malignant schwannoma cells and malignant rhabdomyoblasts. It is classified as a malignant peripheral nerve sheath tumor with rhabdomyosarcomatous differentiation.

The cerebellopontine angle syndrome is a distinct neurological syndrome of deficits that can arise due to the closeness of the cerebellopontine angle to specific cranial nerves. Indications include unilateral hearing loss (85%), speech impediments, disequilibrium, tremors or other loss of motor control. The cerebellopontine angle cistern is a subarachnoid cistern formed by the cerebellopontine angle that lies between the cerebellum and the pons. It is filled with cerebrospinal fluid and is a common site for the growth of acoustic neuromas or schwannomas.

<span class="mw-page-title-main">Selumetinib</span> Chemical compound

Selumetinib (INN), sold under the brand name Koselugo, is a medication for the treatment of children, two years of age and older, with neurofibromatosis type I (NF-1), a genetic disorder of the nervous system causing tumors to grow on nerves. It is taken by mouth.

Within medical ophthalmology, Intraocular schwannoma, also termed uveal schwannoma, is a type of schwannoma found in the eye. These tumors are almost always benign in nature and while malignant forms have been documented in other areas of the body, this has not been reported in the uveal region. Composed of Schwann cells, these masses are generally slow growing and can be found in the peripheral nerve tract, often around the head and neck.

An intraneural perineurioma is a rare benign tumor within the sheath of a single nerve that grows but typically does not recur or metastasize. These lesions are only composed of perineurial cells, cloned from a single cell. They are distinct from schwannoma and neurofibroma.

<span class="mw-page-title-main">Peripheral nerve tumor</span> Medical condition

Peripheral nerve tumors, also called tumors of peripheral nerves or tumors of the peripheral nervous system, are a diverse category with a range of morphological characteristics and biological potential. They are categorized as either benign or malignant peripheral nerve sheath tumors.

References

  1. 1 2 3 4 5 6 7 8 9 10 11 12 13 14 15 16 17 18 Gui C, Canthiya L, Zadeh G, Suppiah S (October 2024). "Current state of spinal nerve sheath tumor management and future advances". Neuro-Oncology Advances. 6 (Suppl 3): iii83–iii93. doi:10.1093/noajnl/vdae067. PMC   11485951 . PMID   39430389.
  2. ↑ Major NM, Anderson MW, Helms CA, Kaplan PA, Dussault R (2020-01-01). "Chapter 13 - Spine". In Major NM, Anderson MW, Helms CA, Kaplan PA (eds.). Musculoskeletal MRI (Third ed.). St. Louis (MO): Elsevier. pp. 295–346. ISBN   978-0-323-41560-6 . Retrieved 2024-12-12.
  3. ↑ Conti P, Pansini G, Mouchaty H, Capuano C, Conti R (January 2004). "Spinal neurinomas: retrospective analysis and long-term outcome of 179 consecutively operated cases and review of the literature". Surgical Neurology. 61 (1): 34–43, discussion 44. doi:10.1016/S0090-3019(03)00537-8. PMID   14706374.
  4. ↑ Jinnai T, Koyama T (March 2005). "Clinical characteristics of spinal nerve sheath tumors: analysis of 149 cases". Neurosurgery. 56 (3): 510–515. doi:10.1227/01.NEU.0000153752.59565.BB. PMID   15730576.
  5. 1 2 Swiatek VM, Stein KP, Cukaz HB, Rashidi A, Skalej M, Mawrin C, et al. (August 2021). "Spinal intramedullary schwannomas-report of a case and extensive review of the literature". Neurosurgical Review. 44 (4): 1833–1852. doi:10.1007/s10143-020-01357-5. PMC   8338859 . PMID   32935226.
  6. 1 2 3 4 5 6 7 "Nerve Sheath Tumor". Archived from the original on 2024-04-19. Retrieved 2024-12-18.
  7. ↑ Perry A, Roth KA, Banerjee R, Fuller CE, Gutmann DH (July 2001). "NF1 deletions in S-100 protein-positive and negative cells of sporadic and neurofibromatosis 1 (NF1)-associated plexiform neurofibromas and malignant peripheral nerve sheath tumors". The American Journal of Pathology. 159 (1): 57–61. doi:10.1016/S0002-9440(10)61673-2. PMC   1850421 . PMID   11438454.
  8. ↑ Chaubal A, Paetau A, Zoltick P, Miettinen M (1994). "CD34 immunoreactivity in nervous system tumors". Acta Neuropathologica. 88 (5): 454–458. doi:10.1007/BF00389498. PMID   7531384.
  9. ↑ Weiss SW, Langloss JM, Enzinger FM (September 1983). "Value of S-100 protein in the diagnosis of soft tissue tumors with particular reference to benign and malignant Schwann cell tumors". Laboratory Investigation; A Journal of Technical Methods and Pathology. 49 (3): 299–308. PMID   6310227.
  10. ↑ Carroll SL (March 2012). "Molecular mechanisms promoting the pathogenesis of Schwann cell neoplasms". Acta Neuropathologica. 123 (3): 321–348. doi:10.1007/s00401-011-0928-6. PMC   3288530 . PMID   22160322.
  11. ↑ Weiss SW, Nickoloff BJ (October 1993). "CD-34 is expressed by a distinctive cell population in peripheral nerve, nerve sheath tumors, and related lesions". The American Journal of Surgical Pathology. 17 (10): 1039–1045. doi:10.1097/00000478-199310000-00009. PMID   7690524.
  12. 1 2 3 4 5 6 "Peripheral nerve tumors - Diagnosis and treatment - Mayo Clinic". www.mayoclinic.org. Retrieved 2024-12-18.
  13. ↑ Research Cf (2024-08-09). "FDA approves selumetinib for neurofibromatosis type 1 with symptomatic, inoperable plexiform neurofibromas". FDA.
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