Materiały źródłowe

• #1 Benign peripheral nerve tumor – Symptoms and causes – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/peripheral-nerve-tumors-benign/symptoms-causes/syc-20368680

  Benign peripheral nerve tumors are tumors that form on peripheral nerves. […] Several different types of tumors may form on the peripheral nerves. While some are caused by genetics, the cause of these tumors is usually not known. […] The cause of benign peripheral nerve tumors usually isn’t known. Some are passed down in families. […] For neurofibromas, having the genetic condition known as neurofibromatosis type 1 (NF1) is a risk factor. NF1 can cause someone to have several neurofibromas. But most people who have neurofibromas don’t have NF1. […] For schwannomas, having the gene that causes schwannomatosis is a risk factor.

• #2 Mayo Clinic Health Library – Benign peripheral nerve tumor | Swiss Medical Network

  https://www.swissmedical.net/fr/healtcare-library/con-20341913

  Benign peripheral nerve tumors are tumors that form on peripheral nerves. The peripheral nerves link the brain and spinal cord to other parts of the body. These nerves control muscles that allow you to walk, blink, swallow, pick things up and do other activities. Benign tumors are not cancerous. […] Several different types of tumors may form on the peripheral nerves. While some are caused by genetics, the cause of these tumors is usually not known. […] The cause of benign peripheral nerve tumors usually isn’t known. Some are passed down in families.

• #3 Benign peripheral nerve tumor | Health Library | Memorial Health System

  https://www.mhsystem.org/health-library/con-20341913/

  Benign peripheral nerve tumors are tumors that form on peripheral nerves. The peripheral nerves link the brain and spinal cord to other parts of the body. These nerves control muscles that allow you to walk, blink, swallow, pick things up and do other activities. Benign tumors are not cancerous. […] Several different types of tumors may form on the peripheral nerves. While some are caused by genetics, the cause of these tumors is usually not known. […] The cause of benign peripheral nerve tumors usually isnt known. Some are passed down in families.

• #4 Schwannoma: What It Is, Causes, Symptoms & Treatment

  https://my.clevelandclinic.org/health/diseases/17877-schwannoma

  A schwannoma is a tumor that develops from Schwann cells in your peripheral nervous system or nerve roots. Theyre almost always benign and slow-growing. […] Schwannomas usually develop randomly in otherwise healthy people. In some cases, a schwannoma is caused by a genetic condition, such as neurofibromatosis 2 (NF2), schwannomatosis or Carney complex. People with these genetic conditions usually have more than one schwannoma. […] The cause of schwannomas isnt known in most cases. Approximately 90% of cases occur sporadically (randomly). […] Genetic disorders such as Carney complex, neurofibromatosis 2 (NF2) and schwannomatosis can cause schwannomas. Genetic studies show that the NF2 gene on chromosome 22 plays an essential role in schwannoma development.

• #5

  https://continentalhospitals.com/diseases/benign-peripheral-nerve-tumor/

  Benign peripheral nerve tumors, also known as neurofibromas, are non-cancerous growths that develop on the peripheral nerves throughout the body. These tumors arise from the cells that make up the protective covering of the nerves called Schwann cells. Neurofibromas can occur in individuals with a condition called neurofibromatosis, which is a genetic disorder characterized by the growth of multiple tumors along the nervous system. However, they can also develop sporadically in people without any underlying genetic predisposition. […] Understanding the causes of a benign peripheral nerve tumor is crucial in order to effectively diagnose and treat this condition. While the exact cause of these tumors is not always clear, there are several factors that have been identified as potential contributors. One potential cause of benign peripheral nerve tumors is genetic mutations. Certain genetic conditions, such as neurofibromatosis type 1 (NF1) and schwannomatosis, have been linked to an increased risk of developing these tumors. These conditions result in abnormalities in the genes responsible for regulating cell growth and division within the peripheral nerves. Additionally, exposure to certain environmental factors may play a role in the development of benign peripheral nerve tumors. For example, individuals who have been exposed to certain chemicals or toxins, such as vinyl chloride or arsenic, may be at an increased risk. Furthermore, it is important to note that while these factors may contribute to the development of benign peripheral nerve tumors, they do not guarantee their occurrence. Many individuals with these risk factors never develop tumors, highlighting the complex nature of this condition.

• #6 Neurofibrosarcoma and Schwannoma

  https://www.webmd.com/cancer/neurofibrosarcoma-and-schwannoma

  Schwannomas are most often noncancerous nerve tumors, while neurofibrosarcomas are cancerous. Doctors dont know what causes either of them, although some cases may be connected to genetics. […] Mostly, doctors dont know what causes nerve sheath tumors. […] Doctors still dont understand what causes about 90% of schwannomas. Most schwannomas occur at random, but certain genetic conditions, such as neurofibromatosis type 2, can significantly increase the risk of developing schwannomas, Westbroek says. A related condition, neurofibromatosis type 1, is associated with a significantly increased risk of developing neurofibrosarcoma. […] As with schwannomas, researchers still have to find out what causes most neurofibrosarcomas. What we do know is that an inherited disorder known as neurofibromatosis type 1 (previously known as von Recklinghausen disease) is „associated with a significantly increased risk of developing neurofibrosarcoma, according to Westbroek. Radiation exposure is also associated with an increased risk of developing neurofibrosarcoma, he says.

• #7 Neurofibroma – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK539707/

  Neurofibromas are the most prevalent benign peripheral nerve sheath tumor. […] A mutation in the NF1 gene causes neurofibromas. […] Approximately 90% of cases occur sporadically, while the remaining cases are associated with neurofibromatosis type 1 or 2. […] In both sporadic and syndromic cases, neurofibromas are a result of a deletion in the NF1 gene.

• #8 Pathology Outlines – Neurofibroma-general

  https://www.pathologyoutlines.com/topic/softtissueneurofibroma.html

  Neurofibroma is a benign peripheral nerve sheath tumor comprised of neuronal and fibrous components. […] According to WHO, etiology is unknown. […] In both sporadic and syndromic cases, neurofibromas are a result of biallelic genetic inactivation of the tumor suppressor gene NF1 with complete loss of function of the NF1 gene product, neurofibromin. […] In sporadic cases, only the lesional cells carry the NF1 mutation. […] In syndromic cases, neurofibromas are the result of a germline mutation in NF1, encoding the tumor suppressor protein neurofibromin, on chromosome 17q11.2.

• #9 Nerve Sheath Tumors: Definition & Types

  https://my.clevelandclinic.org/health/diseases/22526-nerve-sheath-tumors

  Genetic changes play an important role in the development of nerve sheath tumors. Alteration of the NF2 gene is linked to schwannomas, while the NF1 gene is associated with neurofibromas. […] Most often, these genetic changes are sporadic and happen randomly. A small fraction of schwannomas and neurofibromas are caused by a rare, genetic disease that runs in families called neurofibromatosis. […] The greatest risk is for people with NF1 who develop plexiform neurofibroma. Malignant peripheral nerve sheath tumors have a poor prognosis, especially if the tumor is larger than 2 inches. Fewer than half of people with this condition live five years after diagnosis.

• #10

  https://continentalhospitals.com/diseases/benign-peripheral-nerve-tumor/

  One of the primary risk factors for benign peripheral nerve tumors is a genetic predisposition. Research suggests that certain inherited conditions, such as neurofibromatosis type 1 and schwannomatosis, can significantly increase the chances of developing these tumors. Individuals with a family history of these conditions should be particularly vigilant in monitoring their health. Another potential risk factor is exposure to certain environmental toxins or radiation. Studies have indicated a possible link between occupational exposure to chemicals or radiation and the development of benign peripheral nerve tumors. It is important for individuals working in industries where such exposures are common to take appropriate precautions and follow safety guidelines. Age can also play a role in determining the risk of developing these tumors. While they can occur at any age, they are more commonly observed in adults between the ages of 30 and 60. Regular medical check-ups and screenings become increasingly important as individuals reach this age range. It is worth noting that while these risk factors may increase the likelihood of developing benign peripheral nerve tumors, they do not guarantee their occurrence. Many cases occur sporadically without any identifiable cause or predisposing factors. By understanding these risk factors, individuals can take proactive steps towards early detection, proper diagnosis, and timely treatment if necessary. Regular communication with healthcare professionals can provide valuable guidance on managing individual risks and maintaining overall well-being.

• #11

  https://link.springer.com/article/10.1007/s11606-020-06463-0

  Bells palsy accounts for 6075% of unilateral facial paralysis cases with an annual incidence of 15 to 30 cases per 100,000 people. […] Although rare, malignant tumors can also affect the facial nerve with similar presentation as Bells palsy. Malignant peripheral nerve sheath tumor (MPNST) is an aggressive tumor with an annual incidence of 0.8 cases per 100,000 people. […] MPNST arises sporadically from a peripheral nerve either from a pre-existing benign peripheral nerve sheath tumor as a consequence of radiation treatment or associated with neurofibromatosis type 1 (NF1) syndrome. […] More than 50% of MPNST are associated with NF1 syndrome, and patients with NF1 syndrome have an 8 to 13% lifetime incidence of developing of MPNST. […] The etiology of Bells palsy is still unknown, with the general mechanism involving inflammation and edema of the facial nerve leading to compression, and thus, it is a diagnosis of exclusion based on a thorough history and physical examination.

• #12 Nerve Sheath Tumors: Types, Symptoms, Treatment, Outlook

  https://www.healthline.com/health/nerve-sheath-tumor

  Nerve sheath tumors occur due to mutations (changes) in your genes that result in the overactivation of nerve sheath cells. Researchers arent sure what causes these mutations to occur. They may happen spontaneously in otherwise healthy people for unknown reasons. […] Nerve sheath tumors are sometimes associated with a rare genetic condition called neurofibromatosis. There are three types of neurofibromatosis: NF1, NF2, and schwannomatosis. The risk of a malignant nerve sheath tumor is higher in people with NF1.

• #13 Schwannoma | Other conditions | Cancer Research UK

  https://www.cancerresearchuk.org/about-cancer/other-conditions/schwannoma

  A schwannoma is a tumour of the tissue that covers nerves, called the nerve sheath. They develop from a type of cell called a Schwann cell. Shwannomas are non-cancerous (benign). […] Rarely, schwannomas may change to become malignant peripheral nerve sheath tumours. […] Schwannomas can also develop in the spinal nerves and in the nerves that are outside the brain and spine (peripheral nerves). […] This is a rare condition. It is also known as Neurofibromatosis Type 2. It is caused by a faulty gene. This fault causes the cells to divide uncontrollably. […] It is rare for NF2-related schwannomatosis to develop into a cancer. […] This rare condition is also known as schwannomatosis. Non cancerous schwannomas form on the nerve sheath. In particular on the spinal nerves and the nerves around the body. […] Some people have symptoms, but no genetic change is seen. So far, two genes have been found: LZTR1 and SMARCB1. […] Controlling the pain is very important in this condition. Removing the schwannoma by surgery can also help relieve the pain.

• #14 Hybrid peripheral nerve sheath tumors: report of five cases and detailed review of literature | BMC Cancer | Full Text

  https://bmccancer.biomedcentral.com/articles/10.1186/s12885-017-3350-1

  However, exact rates of recurrence and malignant transformation remain largely unknown owing to extreme rarity of these tumors. […] Rare case reports have described local recurrence. […] At least two recent case reports have described malignant transformation in hybrid PNSTs. […] Hybrid PNSTs are associated with certain tumor syndromes, and more than half of hybrid PNSTs with such associations are multiple. […] Over 70% patients with schwannomatosis have single or multiple hybrid neurofibroma/schwannoma tumors. […] Similarly, over 25% patients with Neurofibromatosis (NF) 2 and about 90% patients with NF1 have single or multiple hybrid neurofibroma/schwannoma tumors. […] Hybrid neurofibroma/perineurioma tumors occur most commonly in association with NF1. […] Even today the exact pathogenetic basis of dual (or even triple) differentiation in hybrid PNSTs is poorly understood and whether such hybrid differentiation results from a clonal genetic alteration or from a localized change in the microenvironment is not known.

• #15 Hybrid peripheral nerve sheath tumors: report of five cases and detailed review of literature | BMC Cancer | Full Text

  https://bmccancer.biomedcentral.com/articles/10.1186/s12885-017-3350-1

  Hybrid peripheral nerve sheath tumors (PNSTs) have been recognized recently and were first included in the 4th edition of World Health Organization (WHO) Classification of Tumors of Soft tissue and Bone, published in 2013. […] We have discussed the types and etiology, epidemiology and sites of localization, gross and microscopic appearances and immunohistochemical features of hybrid PNSTs and association of these tumors with tumor syndromes. […] The most common types are combinations of Schwannoma/perineurioma, which usually occur sporadically, and neurofibroma/schwannoma, which are typically associated with neurofibromatosis (NF) type 1 or 2 or with schwannomatosis. […] Those associated with NF1 carry a risk of malignant transformation to malignant peripheral nerve sheath tumors (MPNSTs).

• #16 Peripheral nerve tumors // Middlesex Health

  https://middlesexhealth.org/learning-center/diseases-and-conditions/peripheral-nerve-tumors

  It’s not clear why most peripheral nerve tumors develop. Some are linked to known inherited syndromes, such as neurofibromatosis (types 1 and 2) and schwannomatosis. Others may be caused by changes in a gene. […] Peripheral nerve tumors are more common in people who have: […] Neurofibromatosis (types 1 and 2) and schwannomatosis. In these disorders, tumors develop on or near the nerves throughout the body. There are often multiple tumors. They can lead to a variety of symptoms depending on where they are in the body. These tumors are usually not cancerous. […] A history of radiation treatment. A person who was exposed to radiation is at higher risk of developing peripheral nerve tumors years later.

• #17 Nerve Sheath Tumors | The National Canine Cancer Foundation

  https://wearethecure.org/learn-more-about-canine-cancer/canine-cancer-library/nerve-sheath-tumors/

  Although the etiology is unknown, they are believed to develop in areas around former injury. […] Normally schwan cells from which these tumors originate help in the restoration of tissues and cells damaged during injury. It is thought that during the process of repair, tumorogenesis takes place. However, there is no published information supporting the fact. […] Reports suggest that benign peripheral nerve sheath tumors have an excellent prognosis.

• #18 Tumor of the Nerves in Dogs – Symptoms, Causes, Diagnosis, Treatment, Recovery, Management, Cost

  https://wagwalking.com/condition/tumor-of-nerves

  Tumors of the nerves are abnormal growths that originate from peripheral nerves or along the nerve sheath (myelin surrounding the nerves). […] While the exact cause for tumors in the nerves in dogs has not yet been pinpointed, documentation shows that the following factors have been suspected: Previous exposure to radiation may bring about a tumor, A hereditary mutation may be an explanation for a tumor, Tumors have been seen to develop around a preceding injury.

• #19 Benign peripheral nerve tumor // Middlesex Health

  https://middlesexhealth.org/learning-center/diseases-and-conditions/benign-peripheral-nerve-tumor

  Several different types of tumors may form on the peripheral nerves. The cause of these tumors is usually unknown. Some have a genetic cause. […] The cause of benign peripheral nerve tumors usually isn’t known. Some are passed down in families. […] The most common type of benign peripheral nerve tumor is a schwannoma. A schwannoma typically starts in a single bundle inside the main nerve, shifting the rest of the nerve. […] Another common type is a neurofibroma. A neurofibroma also often forms inside the nerve. Sometimes it grows from several nerve bundles. […] Perineuriomas are rare and can grow from inside or outside the nerve. Tumors outside a nerve can cause problems when they press on the nerve. Other benign tumors that form outside nerves are soft lumps of slow-growing fat cells called lipomas and ganglion cysts.

• #20 Benign peripheral nerve tumor

  http://ask-ahd.ahdubai.com/con-20341913

  Several types of nerve tumors occur. Though their cause is usually unknown, some are hereditary. […] The cause of benign peripheral nerve tumors usually isn’t known. Some are inherited. The most common type is schwannoma. It typically starts in a single bundle inside the main nerve, displacing the rest of the nerve. […] Another common type is neurofibroma. These tumors often form inside the nerve. Sometimes they arise from several nerve bundles. […] Perineuriomas are rare and can grow from inside or outside the nerve. Tumors outside a nerve can cause problems when they press on the nerve. Other benign tumors that form outside nerves are soft lumps of slow-growing fat cells called lipomas and ganglion cysts.

• #21 Peripheral nerve sheath tumor | Radiology Reference Article | Radiopaedia.org

  https://radiopaedia.org/articles/peripheral-nerve-sheath-tumour-2?lang=us

  Peripheral nerve sheath tumors (PNSTs) are a group of primary neurogenic tumors that arise from nerve sheaths outside of the central nervous system. The vast majority are benign, however, malignant transformation is seen particularly in large tumors and those associated with neurofibromatosis type 1 (NF1). […] Symptoms of benign peripheral nerve sheath tumors are non-specific and include sensory and/or motor deficits, isolated pain, painful mass (i.e. positive Tinel sign), asymptomatic mass or may be incidental. […] Pre-surgical biopsy of peripheral nerve sheath tumors (usually an ultrasound-guided core biopsy) is typically reserved for lesions with indeterminate or malignant clinical and/or radiological features; the risk of biopsy (e.g. new/worsening neuropathic pain or sensory/motor impairment) outweighs the benefit of biopsy in suspected benign peripheral nerve sheath tumors.

• #22 Decades Old: A Paraspinal Tumor with Intrathoracic Extension and Pathologic Features of Neurofibroma and Schwannoma | ACS

  https://www.facs.org/for-medical-professionals/news-publications/journals/case-reviews/issues/v4n3/20-zipple-paraspinal-tumor/

  Benign PNSTs are rare tumors that can involve any peripheral nerve. […] When arising from nerve roots, they are classified as paraspinal PNSTs. […] These are frequently asymptomatic and can be large before symptoms occur. […] In fact, paraspinal PNSTs frequently invade the posterior mediastinum and may rarely involve other mediastinal compartments, the parenchyma of the lung, or extend intrathoracically, as with our patient. […] Care must be taken to differentiate schwannoma and neurofibromas from malignant varieties of PNST. […] Schwannomas comprise approximately 65% of PNSTs, while malignant PNSTs compromise only 5% of PNSTs. […] Clinical suspicion should be raised in patients presenting with multiple neurofibromas or schwannomas, given their association with inherited genetic disorders (like neurofibromatosis type 1, neurofibromatosis type 2, and schwannomatosis).

• #23

  https://www.orthobullets.com/pathology/8053/neurilemmoma

  Neurilemmoma, also known as Schwannoma, are benign, encapsulated nerve sheath tumors composed of Schwann cells that occur on the surface of peripheral nerves. […] Etiology: often associated with mutations affecting NF2 gene. […] Malignant transformation: extremely rare.

• #24 PERIPHERAL NERVE TUMORS – Dr Prem Pillay

  https://singaporebrain.org/en/nerves/peripheral-nerve-tumors/

  Neurofibromas also arise from Schwann cells, typically featuring other non-neoplastic cellular components (e.g., fibroblasts, perineurial cells). A distinct subtype is the plexiform neurofibroma, often linked to neurofibromatosis type 1 (NF1). Plexiform neurofibromas can transform into malignant peripheral nerve sheath tumors (MPNSTs) and therefore warrant close monitoring. […] MPNSTs are aggressive soft-tissue sarcomas accounting for about 10% or slightly more of all peripheral nerve sheath tumors. They may arise sporadically or in association with NF1 or prior radiation therapy. MPNSTs often present with a high risk of local recurrence and metastasis, with the prognosis highly dependent on complete surgical resection and the tumors stage at diagnosis. […] In asymptomatic, slowly growing, small benign peripheral nerve tumors (usually a few mm in size) a watch-and-wait approach (also called monitoring) may be considered if they do not threaten function. Periodic MRI or other imaging helps track any changes in size or symptoms, and treatment can be initiated if the tumor grows or becomes symptomatic.

• #25 Peripheral Nerve Sheath Tumors | Neurological Surgery

  https://neurosurgery.weillcornell.org/condition/peripheral-nerve-sheath-tumors

  Individuals may be predisposed to developing peripheral nerve sheath tumors if they have a family history of them. […] Even in those without a family history, these tumors can form due to certain genetic mutations that result in an overactivation of nerve sheath cells. […] Those who have had radiation therapy to treat cancer may be at risk for MPNSTs between 10 and 20 years after treatment.

• #26 Benign peripheral nerve tumor | ABC Medical Center

  https://centromedicoabc.com/en/padecimientos/benign-peripheral-nerve-tumor/

  A benign tumor of the peripheral nerves can develop in any part of the body. Although it is not cancerous, it can damage the peripheral nerve and cause loss of function in the affected area. […] The cause of a benign peripheral nerve tumor is unknown, but it may be related to genetic alteration or hereditary syndromes such as neurofibromatosis type 1 and type 2, and schwannomatosis. People who receive or received radiation therapy treatments for cancer have a greater predisposition to developing a peripheral nerve tumor.

• #27 Nerve Sheath Tumor in Dogs – Symptoms, Causes, Diagnosis, Treatment, Recovery, Management, Cost

  https://wagwalking.com/condition/nerve-sheath-tumor

  Nerve Sheath Tumor in Dogs is an abnormal growth or mass of the skin and soft tissue. The tumor is comprised of Schwann cells that are found in connective tissue known as the myelin, or cover, that surrounds the nerve. […] There is no publicly disclosed cause at this time. […] The average age of onset for most breeds is 9. […] Both types need to be evaluated and removed surgically to decrease discomfort and the likelihood of the tumor to develop further or increase in size. […] The malignant tumors are characterized by having the potential to become invasive and grow rapidly.

• #28 Nervous system: Peripheral nerve sheath tumors

  https://atlasgeneticsoncology.org/solid-tumor/5094/nervous-system-peripheral-nerve-sheath-tumors

  The etiology of PNSTs is usually unknown. However, several hereditary disorders are known to predispose to benign and malignant PNSTs, notably neurofibromatosis type 1 (NF1) and neurofibromatosis type 2 (NF2), both of which are inherited in an autosomal dominant fashion. […] The basis for MPNST occurring in the setting of NF1 is presumed to be biallelic inactivation of the NF1 gene. Indeed, allelic imbalance at the NF1 locus in 17q is commonly (20-50% of the cases) detected in sporadic as well as NF1-associated MPNSTs.

• #29 Peripheral Nerve Tumors of the Extremities | Neupsy Key

  https://neupsykey.com/peripheral-nerve-tumors-of-the-extremities/

  Schwannomas (neurilemmomas) are benign tumors that arise from the nerve sheath. They are typically solitary and slow growing. A schwannoma can arise from any peripheral nerve with a Schwann cell, including distal portions of cranial nerves. Sporadic schwannomas can occur at any age but are most common in the third to sixth decades of life. […] The tumors that develop may be conventional globular schwannomas or plexiform and arise throughout the body or may be limited to a single segment or nerve. It is currently unknown whether the diagnosis of schwannomatosis alters the risk of malignant transformation of a schwannoma. […] Mutations in the NF2 gene at the 22q12.2 locus are found in sporadic schwannomas and those in patients with NF2. The schwannomas that develop in patients with schwannomatosis show a multitude of truncations in the NF2 gene, and multiple tumors from an individual patient can each have unique mutations.

• #30 Malignant peripheral nerve sheath tumors

  https://www.mymlc.com/health-information/diseases-and-conditions/m/malignant-peripheral-nerve-sheath-tumors/?section=Risk%20factors

  It’s not clear what causes most malignant peripheral nerve sheath tumors. […] Doctors know that these cancers begin when a cell in the protective lining around a nerve develops an error (mutation) in its DNA. The mutation tells the cell to multiply rapidly and to continue living when other cells would normally die. The accumulating cells form a tumor that can grow to invade nearby tissue or spread to other areas of the body. […] Factors that increase the risk of malignant peripheral nerve sheath tumors include: Previous radiation therapy for cancer. A malignant peripheral nerve sheath tumor may develop in the area treated with radiation 10 to 20 years after treatment. Noncancerous nerve tumors. Malignant peripheral nerve sheath tumors can develop from noncancerous (benign) nerve tumors, such as neurofibroma. An inherited condition that increases risk of nerve tumors. Malignant peripheral nerve sheath tumors occur more frequently in people with neurofibromatosis 1.

• #31 A giant posterior mediastinal malignant peripheral nerve sheath tumor and benign neurofibroma in body surface: a case report | BMC Surgery | Full Text

  https://bmcsurg.biomedcentral.com/articles/10.1186/s12893-021-01122-5

  Neurofibromatosis comprises neurofibromatosis type 1 (NF1) and type 2 (NF2). Major tumor type of NF1 are neurofibroma recognized as benign peripheral nerve tumor, malignant peripheral nerve sheath tumor (MPNST), and glioma. […] Despite neurofibroma and nerve sheath tumor are both Schwann cell-derived tumors, their tumor characteristics and clinical manifestations are significantly different. […] The appearance of neurofibroma should draw particular attention to the possibility of developing MPNST. More careful imaging examinations should be carried out, and pathological examination could diagnose it. […] As is known, neurofibromas are benign peripheral nerve sheath tumors arising from Schwann cell progenitors, of which cutaneous neurofibromas (cNF) are the most common type. […] The significant difference between Ki67 expression in both tumor (1% of neurofibroma vs. 30% of nerve sheath tumor) indicated the dramatic cell proliferation.

• #32 A giant posterior mediastinal malignant peripheral nerve sheath tumor and benign neurofibroma in body surface: a case report | BMC Surgery | Full Text

  https://bmcsurg.biomedcentral.com/articles/10.1186/s12893-021-01122-5

  Atypical neurofibroma (ANF), with pathologically increased variable cellularity, cytological atypia and fascicular growth patterns, was regarded as precursor lesions for MPNST. […] Taken together, the appearance of neurofibroma should draw particular attention to the possibility of developing MPNST. Once NF1 is diagnosed, more careful imaging examinations should be carried out and needle biopsy is a more confirmative approach if possible.

• #33 Benign peripheral nerve sheath tumors: an interdisciplinary diagnostic and therapeutic challenge

  https://pmc.ncbi.nlm.nih.gov/articles/PMC10439084/

  A benign peripheral nerve sheath tumor (bPNST) is a rare lesion associated with peripheral nerval structures. […] For the first time, our data shows the quantity and impact of incorrect initial diagnosis in bPNST causing a delay in causative treatment or resulting in unnecessary or potentially harmful treatment. […] A benign peripheral nerve sheath tumor (bPNST) is a rare lesion of neuroectodermal origin, directly associated with peripheral nerve structures. […] We aimed to evaluate the rate of primary misdiagnosis and mistreatment in patients suffering from deep-seated bPNSTs. Furthermore, we aimed to identify causative factors for mistreatment. […] An initial incorrect diagnosis was made in 44.7% of patients. […] We were able to identify four main causes resulting in misdiagnosis of bPNST.

• #34

  https://link.springer.com/article/10.1007/s10143-023-02107-z

  A benign peripheral nerve sheath tumor (bPNST) is a rare lesion associated with peripheral nerval structures. […] Therefore, we aimed to evaluate the rate of primary misdiagnosis and mistreatment in patients suffering from deep-seated bPNSTs. Furthermore, we aimed to identify causative factors for mistreatment. […] A misdiagnosis occurred, if the final diagnosis was inconsistent with the diagnosis which was set up by the doctor who treated the patient at first. […] We were able to identify four main causes resulting in misdiagnosis of bPNST. Firstly, variation of different symptoms and especially failure to recognize and interpret specific symptoms (for example, a positive Hoffmann Tinels sign) may lead to misdiagnosis concerning a non-pathological MRI of the spine. […] Secondly, symptom presentation of bPNST might be similar or mimic symptoms of more common diseases.

• #35 Benign peripheral nerve sheath tumors: an interdisciplinary diagnostic and therapeutic challenge

  https://pmc.ncbi.nlm.nih.gov/articles/PMC10439084/

  Our data show the prolonged time for a symptomatic patient to receive a definite diagnosis and adequate therapy in many cases. […] However, chronic pain and the missing explanation of individual symptoms can lead to a significant psychological burden. […] This resulted in severe neurological deficits that had to be treated with nerve interposition devices during revision surgery. […] Treatment by an experienced nerve surgeon showed significantly lower rates of mistreatment compared with treatment by other specialists.

• #36

  https://link.springer.com/article/10.1007/s10143-023-02107-z

  Thirdly, bPNST may occur in every region of the body. […] Lastly, in the German medical system, general practitioners are the first consultants of the patients and are essential for organizing further therapy at a specialized center. […] Our data show the prolonged time for a symptomatic patient to receive a definite diagnosis and adequate therapy in many cases. […] However, chronic pain and the missing explanation of individual symptoms can lead to a significant psychological burden. […] Another reason for mistreatment is the result of a priorly wrong diagnosis. […] The misinterpretation of a symptom and/or the radiological imaging may lead to unnecessary or inappropriate surgical treatment unrelated to the actual disease or to a more radical surgical treatment. […] This resulted in severe neurological deficits that had to be treated with nerve interposition devices during revision surgery. […] Treatment by an experienced nerve surgeon showed significantly lower rates of mistreatment compared with treatment by other specialists.

• #37 PERIPHERAL NERVE TUMORS – Dr Prem Pillay

  https://singaporebrain.org/en/nerves/peripheral-nerve-tumors/

  Peripheral nerve tumors are a heterogeneous group of neoplasms that arise from or affect nerves outside the brain and spinal cord. According to the 2021 WHO classification of the Central Nervous System (CNS), peripheral nerve sheath tumors (PNSTs) comprise a set of unique entities each with specific clinical, anatomical, histological, and molecular features. Broadly, PNSTs can be subdivided into benign and malignant forms, often reflecting differences in their behavior, prognosis, and recommended treatments. […] Schwannomas originate from Schwann cells, which form the myelin sheath of peripheral nerves. Most schwannomas are benign and can appear anywhere along peripheral nerves. Variants include plexiform schwannoma, which can arise in superficial or deep sites and may be challenging to distinguish from more aggressive lesions if they show atypical features. Multiple schwannomas may be associated with NF1 (Neurofibromatosis). Patients with a peripheral nerve schwannoma may need to have screening of other nerves with MRI of the whole spine and Brain with gadolinium contrast to rule out multiple nerve tumors at other locations.

• #38 PERIPHERAL NERVE TUMORS – Dr Prem Pillay

  https://singaporebrain.org/en/nerves/peripheral-nerve-tumors/

  Research into molecular pathways involved in PNSTs, especially MPNSTs, has propelled the exploration of targeted agents. Emerging evidence suggests that immunomodulatory approaches or other molecularly guided treatments may benefit a subset of aggressive MPNSTs, though these remain experimental. Clinical trials continue to assess efficacy. […] Peripheral nerve tumors, encompassing benign entities such as schwannomas and neurofibromas and malignant variants like MPNSTs, represent a diverse group requiring careful workup and multidisciplinary treatment. Accurate diagnosis via imaging and biopsy, alongside precision surgical techniques, can often lead to remission in benign lesions and offer the best chance for disease control in malignant ones.

• #39 Hybrid peripheral nerve sheath tumors: report of five cases and detailed review of literature | BMC Cancer | Full Text

  https://bmccancer.biomedcentral.com/articles/10.1186/s12885-017-3350-1

  The status of hybrid PNSTs as distinct entities has been recognized by the WHO and included in the 4th edition of the WHO classification of tumors of soft tissue and bone. […] However, further studies are required to determine the status of hybrid PNSTs as distinct entities and to determine the exact pathogenetic basis of hybrid differentiation in PNSTs.

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