Raras
Buscar doenças, sintomas, genes...
Tumor benigno da bainha dos nervos periféricos
ORPHA:252131DOENÇA RARA

O neurotequeoma (NT) é um tipo de tumor cutâneo benigno raro que geralmente se desenvolve na cabeça e no pescoço. Eles geralmente ocorrem na segunda e na terceira décadas de vida e tendem a afetar as mulheres com mais frequência do que os homens. Foi descrito pela primeira vez por Richard L. Gallager e Elson B. Helwig, que propuseram o termo para refletir a suposta origem da lesão na bainha nervosa. Microscopicamente, as lesões descritas se assemelhavam muito ao tumor “mixoma da bainha nervosa (NSM)”, uma entidade descrita pela primeira vez por Harkin e Reed.

Mantido por Agente Raras·Colaborar como especialista →

Introdução

O que você precisa saber de cara

📋

Tumores benignos da bainha dos nervos periféricos podem causar sintomas neuropáticos agudos, fraqueza muscular, deficiência auditiva e outros achados. Associados a genes como NF2 e LZTR1, podem ocorrer em conjunto com escoliose e lipomas.

Publicações científicas
151 artigos
Último publicado: 2026 Mar 24
🏥
SUS: Sem cobertura SUSScore: 0%
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Sinais e sintomas

O que aparece no corpo e com que frequência cada sintoma acontece

Partes do corpo afetadas

🧠
Neurológico
13 sintomas
🦴
Ossos e articulações
6 sintomas
👂
Ouvidos
5 sintomas
🫃
Digestivo
5 sintomas
😀
Face
3 sintomas
🧬
Pele e cabelo
2 sintomas

+ 33 sintomas em outras categorias

Características mais comuns

Episódios agudos de sintomas neuropáticos
Neoplasia
Escoliose
Fraqueza muscular
Deficiência auditiva
Fasciculações
71sintomas
Sem dados (71)

Os sintomas variam de pessoa para pessoa. Abaixo estão as 71 características clínicas mais associadas, ordenadas por frequência.

Episódios agudos de sintomas neuropáticosAcute episodes of neuropathic symptoms
NeoplasiaNeoplasm
EscolioseScoliosis
Fraqueza muscularMuscle weakness
Deficiência auditivaHearing impairment

Linha do tempo da pesquisa

Publicações por ano — veja quando o interesse científico cresceu
Anos de pesquisa1desde 2026
Total histórico151PubMed
Últimos 10 anos93publicações
Pico202316 papers
Linha do tempo
2026Hoje · 2026🧪 1995Primeiro ensaio clínico📈 2023Ano de pico
Publicações por ano (últimos 10 anos)

Encontrou um erro ou informação desatualizada? Sugira uma correção →

Genética e causas

O que está alterado no DNA e como passa nas famílias

Genes associados

4 genes identificados com associação a esta condição.

COQ6Ubiquinone biosynthesis monooxygenase COQ6, mitochondrialCandidate gene tested inTolerante
FUNÇÃO

FAD-dependent monooxygenase required for two non-consecutive steps during ubiquinone biosynthesis (PubMed:26260787, PubMed:38425362). Required for the C5-ring hydroxylation during ubiquinone biosynthesis by catalyzing the hydroxylation of 4-hydroxy-3-(all-trans-decaprenyl)benzoic acid to 3,4-dihydroxy-5-(all-trans-decaprenyl)benzoic acid (PubMed:26260787, PubMed:38425362). Also acts downstream of COQ4, for the C1-hydroxylation during ubiquinone biosynthesis by catalyzing the hydroxylation of 2-m

LOCALIZAÇÃO

Mitochondrion inner membraneGolgi apparatusCell projection

VIAS BIOLÓGICAS (1)
Ubiquinol biosynthesis
MECANISMO DE DOENÇA

Coenzyme Q10 deficiency, primary, 6

An autosomal recessive disorder characterized by onset in infancy of severe progressive nephrotic syndrome resulting in end-stage renal failure and sensorineural deafness. Renal biopsy usually shows focal segmental glomerulosclerosis.

VIAS REACTOME (1)
OUTRAS DOENÇAS (2)
familial steroid-resistant nephrotic syndrome with sensorineural deafnessschwannomatosis
HGNC:20233UniProt:Q9Y2Z9
NF2MerlinCandidate gene tested inAltamente restrito
FUNÇÃO

Probable regulator of the Hippo/SWH (Sav/Wts/Hpo) signaling pathway, a signaling pathway that plays a pivotal role in tumor suppression by restricting proliferation and promoting apoptosis. Along with WWC1 can synergistically induce the phosphorylation of LATS1 and LATS2 and can probably function in the regulation of the Hippo/SWH (Sav/Wts/Hpo) signaling pathway. May act as a membrane stabilizing protein. May inhibit PI3 kinase by binding to AGAP2 and impairing its stimulating activity. Suppress

LOCALIZAÇÃO

Cell projection, filopodium membraneCell projection, ruffle membraneNucleusCytoplasm, perinuclear regionCytoplasmic granuleCytoplasm, cytoskeleton

VIAS BIOLÓGICAS (2)
Regulation of actin dynamics for phagocytic cup formationRHO GTPases activate PAKs
MECANISMO DE DOENÇA

Schwannomatosis, vestibular

An autosomal dominant neoplasia syndrome characterized by the development of multiple benign nerve sheath tumors called schwannomas, particularly affecting the vestibular nerve. Affected individuals usually present with bilateral vestibular schwannomas but can have schwannomas on other cranial, spinal, and peripheral/cutaneous nerves. Meningiomas are common, whereas 20 to 35% of affected individuals develop intramedullary spinal cord tumors called ependymomas. The condition is also characterized by several ophthalmic features such as lenticular opacities, retinal hamartoma, epiretinal membranes.

EXPRESSÃO TECIDUAL(Ubíquo)
Cerebelo
30.4 TPM
Cérebro - Hemisfério cerebelar
27.6 TPM
Córtex cerebral
25.9 TPM
Fibroblastos
25.9 TPM
Brain Frontal Cortex BA9
24.3 TPM
OUTRAS DOENÇAS (5)
NF2-related schwannomatosisfamilial meningiomamosaic NF2-related schwannomatosismeningioma
HGNC:7773UniProt:P35240
LZTR1Leucine-zipper-like transcriptional regulator 1Candidate gene tested inTolerante
FUNÇÃO

Substrate-specific adapter of a BCR (BTB-CUL3-RBX1) E3 ubiquitin-protein ligase complex that mediates ubiquitination of Ras (K-Ras/KRAS, N-Ras/NRAS and H-Ras/HRAS) (PubMed:30442762, PubMed:30442766, PubMed:30481304). Is a negative regulator of RAS-MAPK signaling that acts by controlling Ras levels and decreasing Ras association with membranes (PubMed:30442762, PubMed:30442766, PubMed:30481304)

LOCALIZAÇÃO

Endomembrane systemRecycling endosomeGolgi apparatus

MECANISMO DE DOENÇA

Glioma

Gliomas are benign or malignant central nervous system neoplasms derived from glial cells. They comprise astrocytomas and glioblastoma multiforme that are derived from astrocytes, oligodendrogliomas derived from oligodendrocytes and ependymomas derived from ependymocytes.

EXPRESSÃO TECIDUAL(Ubíquo)
Pituitária
76.5 TPM
Nervo tibial
68.8 TPM
Útero
67.8 TPM
Cervix Ectocervix
65.1 TPM
Cervix Endocervix
61.7 TPM
OUTRAS DOENÇAS (9)
Noonan syndrome 10RASopathyNoonan syndrome 2cafe au lait spots, multiple
HGNC:6742UniProt:Q8N653
SMARCB1SWI/SNF-related matrix-associated actin-dependent regulator of chromatin subfamily B member 1Candidate gene tested inAltamente restrito
FUNÇÃO

Core component of the BAF (hSWI/SNF) complex. This ATP-dependent chromatin-remodeling complex plays important roles in cell proliferation and differentiation, in cellular antiviral activities and inhibition of tumor formation. The BAF complex is able to create a stable, altered form of chromatin that constrains fewer negative supercoils than normal. This change in supercoiling would be due to the conversion of up to one-half of the nucleosomes on polynucleosomal arrays into asymmetric structures

LOCALIZAÇÃO

Nucleus

VIAS BIOLÓGICAS (8)
RUNX1 interacts with co-factors whose precise effect on RUNX1 targets is not knownRMTs methylate histone argininesFormation of neuronal progenitor and neuronal BAF (npBAF and nBAF)Formation of the embryonic stem cell BAF (esBAF) complexFormation of the polybromo-BAF (pBAF) complex
MECANISMO DE DOENÇA

Rhabdoid tumor predisposition syndrome 1

A familial cancer syndrome predisposing to renal or extrarenal malignant rhabdoid tumors and to a variety of tumors of the central nervous system, including choroid plexus carcinoma, medulloblastoma, and central primitive neuroectodermal tumors. Rhabdoid tumors are the most aggressive and lethal malignancies occurring in early childhood.

EXPRESSÃO TECIDUAL(Ubíquo)
Linfócitos
136.4 TPM
Testículo
110.5 TPM
Cérebro - Hemisfério cerebelar
98.7 TPM
Cerebelo
93.5 TPM
Ovário
93.2 TPM
OUTRAS DOENÇAS (9)
rhabdoid tumor predisposition syndrome 1intellectual disability, autosomal dominant 15familial multiple meningiomaschwannomatosis
HGNC:11103UniProt:Q12824

Variantes genéticas (ClinVar)

2,254 variantes patogênicas registradas no ClinVar.

🧬 COQ6: NM_182476.3(COQ6):c.1330C>A (p.Leu444Ile) ()
🧬 COQ6: NM_182476.3(COQ6):c.8C>A (p.Ala3Asp) ()
🧬 COQ6: NM_182476.3(COQ6):c.481+171C>T ()
🧬 COQ6: NM_182476.3(COQ6):c.164-277G>A ()
🧬 COQ6: NM_182476.3(COQ6):c.1343G>A (p.Gly448Asp) ()
Ver todas no ClinVar

Diagnóstico

Os sinais que médicos procuram e os exames que confirmam

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Tratamento e manejo

Remédios, cuidados de apoio e o que precisa acompanhar

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Onde tratar no SUS

Hospitais de referência no Brasil e o protocolo oficial do SUS (PCDT)

🇧🇷 Atendimento SUS — Tumor benigno da bainha dos nervos periféricos

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Ensaios clínicos abertos e novidades científicas recentes

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Publicações mais relevantes

Timeline de publicações
94 papers (10 anos)
#1

Benign Peripheral Nerve Sheath Tumors: Recurrence Rates and Pain Outcomes Following Excision.

Plastic and reconstructive surgery2026 Mar 24

Benign peripheral nerve sheath tumors (bPNSTs) present a surgical challenge due to their intimate relationship with functional neurons. This study aims to examine the anatomic distribution of bPNSTs and analyze recurrence rates and factors predictive of recurrence following surgical excision. A retrospective analysis of patients who underwent resection of bPNST was conducted. The anatomic distribution was assessed, along with recurrence rates and pain outcomes. Exploratory bivariate analyses were conducted to assess factors associated with recurrence. A total of 228 patients were included (follow-up: 1.02 years (IQR: 0.55-2.12)). BPNSTs were most commonly located in the upper extremity (45.2%) and lower extremity (44.3%), with fewer cases in the head and neck (6.6%), trunk (3.5%), and pelvis (0.4%). Schwannomas comprised 76.3% of cases, while neurofibromas accounted for 22.4%. The overall recurrence rate following excision was 7.0%, with neurofibromas exhibiting a higher recurrence rate (15.7% vs 4.6% for schwannomas, p=0.049). Patients with neurofibromatosis type 1 (NF1) demonstrated significantly higher recurrence rates (19.4% vs 5.1%, p=0.004). Recurrence was more frequent in patients with postoperative motor deficits (26.3% vs 5.3%, p<0.001), patients with psychiatric comorbidities (13.5% vs 3.9%, p=0.008), and younger patients (median age 38.5 (IQR:28.2-44.8) vs 50.2 years (IQR:39.3-60.3), p=0.001). Overall improvement was reported in 77.1% patients. BPNSTs primarily affect the extremities, with schwannomas being the predominant histological subtype. Neurofibromas, NF1, postoperative motor deficits, psychiatric comorbidity, and younger age were associated with increased recurrence risk. Understanding these distribution patterns and recurrence factors is important for surgical planning and patient counseling.

#2

Posterior Interosseous Nerve Schwannoma Presenting as a Painless Forearm Mass: A Case Report.

Cureus2026 Feb

Schwannomas are benign peripheral nerve sheath tumors that typically exhibit slow growth and may present diagnostic challenges when arising from uncommon sites. We report the case of a 65-year-old female who presented with a six-year history of a gradually enlarging, painless swelling over the extensor aspect of the left forearm. The lesion initially appeared as a small nodule and progressively enlarged to approximately 4 × 3 cm, with intermittent tingling and occasional numbness but no significant functional impairment. Clinical examination revealed a firm, well-defined mass with a positive Tinel's sign. Ultrasonography and contrast-enhanced MRI demonstrated a well-circumscribed, encapsulated lesion along the course of the posterior interosseous nerve (PIN) in the intermuscular plane, suggestive of a benign peripheral nerve sheath tumor. The patient underwent planned microsurgical excision under general anesthesia. Intraoperatively, the tumor was found to arise eccentrically from the PIN and was meticulously dissected from the surrounding nerve fascicles, allowing complete excision while preserving nerve continuity. Histopathological examination confirmed the diagnosis of schwannoma, demonstrating characteristic Antoni A and Antoni B areas, Verocay bodies, and thick-walled hyalinized vessels. The postoperative course was uneventful, with preservation of motor and sensory function and no evidence of recurrence on follow-up. This report underscores the importance of thorough clinical evaluation and appropriate imaging in the diagnosis of peripheral nerve tumors of the forearm and highlights the role of meticulous microsurgical technique in achieving complete tumor excision with excellent functional outcomes.

#3

A rare case report of solitary neurofibroma arising in the male breast.

International journal of surgery case reports2026 Mar

Primary tumors of the chest wall are fairly uncommon and typically involve the mammary glands. Additional breast lesions usually involve the soft tissues, with neurofibromas comprising a significantly low amount of cases that skew toward female patients. A neurofibroma of the male breast is an exceedingly rare occurrence; however, most cases are associated with Neurofibromatosis Type 1 (NF1). This report aims to present the case of a solitary neurofibroma in the breast of a male patient in the absence of a diagnosis of Neurofibromatosis, a situation with only three prior cases documented in the literature. In addition, we review the literature and management approach for this rare occurrence. A 41-year-old male with a PMHx of medication-controlled HTN and migraines noticed a small firm mass in his left breast years ago that recently started causing discomfort with deep palpation despite no reported significant changes in size or development of additional symptoms. Preliminary diagnostic studies (diagnostic mammogram, targeted ultrasound, ultrasound-guided biopsy) illustrated a circumscribed, spiculated oval mass 3 cm from the nipple-areolar complex at the 9-o'clock position, with a biopsy report indicative of a neurofibroma. The patient underwent elective surgical excision of the lesion, and the surgical pathology report additionally confirmed the diagnosis of a neurofibroma. Neurofibromas are benign peripheral nerve sheath tumors that originate from the endoneurium, the connective tissue of nerve sheaths. Solitary neurofibroma of the male breast in the absence of a diagnosis of neurofibromatosis is a substantially rare occurrence, with only three prior confirmed cases documented. Current literature suggests surgical excision as the mainstay of management, regardless of their low risk of malignant transformation. To our knowledge, this is only the fourth case of its kind and the only one to provide gross imagery and two separate confirmatory pathology reports. Implications of the findings include continued preferred management via surgical excision, remaining awareness of rare tumors, and additional evidence for the possible prevalence of rare male breast tumors that may have been previously not considered.

#4

A rare case of plexiform neurofibroma.

Radiology case reports2026 Apr

Plexiform neurofibroma is a rare benign peripheral nerve sheath tumor that occurs almost exclusively in patients with neurofibromatosis type I (NF1). This report presents a 10-year-old patient with multiple enlarging masses on the left and posterior neck for 4 years, accompanied by pain and restricted neck movement. Radiographic evaluation revealed a soft tissue mass without bony abnormalities, while Magnetic Resonance Imaging (MRI) demonstrated multiple confluent, ill-defined, infiltrative lesions involving bilateral cervical regions and extending into the anterior mediastinum. The lesions showed hypointense signals on T1-weighted and hyperintense target sign appearances on T2-weighted images, with encasement of neurovascular structures and spinal cord compression. Histopathological findings confirmed the diagnosis of plexiform neurofibroma without evidence of malignancy. This case emphasizes the diagnostic value of MRI in delineating lesion extent and neurogenic origin, as well as the importance of histopathology in confirming the benign nature of the tumor and excluding malignant transformation.

#5

Symptomatic Schwannomatosis of the Brachial Plexus: A Case Report and Literature Review.

Annals of plastic surgery2026 Feb 01

Brachial plexus schwannomatosis, a rare disease that is characterized by multiple schwannomas within the brachial plexus, remains difficult to treat due to the risk of postoperative neurological deficits. We report a case presenting with a left supraclavicular mass, radiating pain, and difficulty with neck extension. The magnetic resonance imaging results revealed 2 well-circumscribed masses, and schwannomatosis was highly suspected, excluding NF2 features. The patient underwent tumor enucleation via using the supraclavicular approach with microsurgical dissection, preserving both sensory and motor function. At 2 years of follow-up, the patient remained asymptomatic without recurrence. In comparison to solitary schwannoma, 2 distinct schwannomas may involve greater complexity and a heightened risk of motor deficits. Furthermore, this study presents a detailed clinical examination and conducts a literature review to assess previous cases, focusing on treatment approaches and prognostic outcomes. We believe that our findings can provide valuable insights for further research and a better understanding of schwannomatosis in the brachial plexus.

Publicações recentes

Ver todas no PubMed

📚 EuropePMC20 artigos no totalmostrando 93

2026

Benign Peripheral Nerve Sheath Tumors: Recurrence Rates and Pain Outcomes Following Excision.

Plastic and reconstructive surgery
2026

Posterior Interosseous Nerve Schwannoma Presenting as a Painless Forearm Mass: A Case Report.

Cureus
2026

A rare case report of solitary neurofibroma arising in the male breast.

International journal of surgery case reports
2026

A rare case of plexiform neurofibroma.

Radiology case reports
2026

Symptomatic Schwannomatosis of the Brachial Plexus: A Case Report and Literature Review.

Annals of plastic surgery
2025

Plexiform Schwannoma of the Middle Finger: A Rare Benign Mesenchymal Tumor Presenting as a Chronic Digital Swelling.

Cureus
2025

Multinodular/Plexiform Schwannoma of the Ankle: A Case Report and Literature Review.

Cancer diagnosis &amp; prognosis
2026

Multidisciplinary Management of Plexiform Neurofibromas in Pediatric Patients With Neurofibromatosis 1: Insights From Advisory Board-Guided Clinical Experience in Japan.

The Journal of dermatology
2025

Schwannoma Presenting as Infectious Fluid Collection.

Journal of primary care &amp; community health
2025

A Solitary Diffuse Neurofibroma in a Young Adult Female: An Unusual Presentation in the Scapular Region.

Cureus
2025

Adrenal Schwannoma: A Rare but Important Consideration in Adrenal Tumors.

Cureus
2025

Unveiling the Rare: A Case Report of Rectal Schwannoma in a Neurofibromatosis Type 1 Patient.

Cureus
2025

Gastrointestinal Tract Perineuriomas and Benign Fibroblastic Polyps: Case Report and Comprehensive Systematic Review.

Case reports in gastrointestinal medicine
2025

Microcystic/Reticular Schwannoma of the Skin: A Report of a Rare Case and Brief Literature Review.

Cureus
2025

Nonsyndromic Extraosseous Palate Neurofibroma of a 30-Year-Old Woman: A Case Report.

Case reports in dentistry
2024

Primary pleural schwannoma: a rare case report.

Journal of medicine and life
2024

Axillary Peripheral Nerve Schwannoma: A Rare Occurrence in the Torso.

Cureus
2024

Extraneural Soft Tissue Perineurioma: A Report of a Rare Case of Peripheral Nerve Sheath Tumor.

Cureus
2025

Intraosseous benign peripheral nerve sheath tumor of the jaws: report of 4 new cases and a comprehensive literature review.

Oral surgery, oral medicine, oral pathology and oral radiology
2024

Schwannoma of the Lower Limb: A Case Report.

Cureus
2024

Successful Treatment of Peripheral Nerve Sheath Tumor-related Pain with Perineural Steroid Injection: a case report.

Interventional pain medicine
2024

Plexiform Neurofibroma: A Case Report.

Cureus
2024

Nerve Sheath Myxoma in Pregnancy: A Case Report.

Diseases (Basel, Switzerland)
2024

Common Benign Nerve Sheath Tumor at an Uncommon Location - Gastric Schwannoma.

Cureus
2024

Intravascular schwannoma as an extremely unusual cause of vein obstruction: a case report.

Journal of pathology and translational medicine
2025

Clinical course and predictors of outcome following surgical treatment of benign peripheral nerve sheath tumors, a single center retrospective study.

The International journal of neuroscience
2024

Excision of Solitary Non-syndromic Oral Plexiform Neurofibroma Utilizing a Diode Laser: A Case Report.

Cureus
2024

Intramuscular Hybrid Nerve Sheath Tumor of the Thigh: Case Report and Literature Review.

In vivo (Athens, Greece)
2024

Investigations on ultrasonography in the diagnosis of nodular localized cutaneous neurofibroma.

Journal of clinical ultrasound : JCU
2024

A rare case of synovial sarcoma mimicking benign peripheral nerve-sheath tumor.

Asian journal of surgery
2024

A Case of Superficial Papular Neuroma: A Rare Neural Neoplasm.

The American Journal of dermatopathology
2023

Gnathic Schwannomas: A Report of Two Cases and Systematic Review of the Literature.

Head and neck pathology
2023

Unusual Clinical Presentation of a Benign Peripheral Nerve Sheath Tumor of the Radial Nerve.

Eplasty
2023

Plexiform neurofibroma (Hamartoma) of the median nerve: A two-case report.

Hand surgery &amp; rehabilitation
2023

Benign peripheral nerve sheath tumors: an interdisciplinary diagnostic and therapeutic challenge.

Neurosurgical review
2023

Diagnosis of vestibular schwannoma following an 11-year interval of head and neck manifestations: case report.

Quintessence international (Berlin, Germany : 1985)
2023

Pleural Schwannoma Presenting As Musculoskeletal Type Pain: A Case Report.

Cureus
2023

Mediastinal Teratoma Mimicking Neurofibroma in CT-Guided Biopsy in a Patient With Neurofibromatosis Type 1.

Cureus
2023

DISEASE PROCESSES IDENTIFIED IN CAPTIVE ARABIAN SAND CATS (FELIS MARGARITA HARRISONI).

Journal of zoo and wildlife medicine : official publication of the American Association of Zoo Veterinarians
2023

Atypical neurofibromas reveal distinct epigenetic features with proximity to benign peripheral nerve sheath tumor entities.

Neuro-oncology
2023

Microreticular perineurioma: A new morphological subtype of a rare entity.

Journal of cutaneous pathology
2023

Percutaneous Full Endoscopic Management of Spinal Foraminal Schwannomas: Case Series.

Operative neurosurgery (Hagerstown, Md.)
2022

A Rare Case of a Large Sinonasal Neurofibroma.

Cureus
2023

Plexal intraneural perineurioma: an analysis of the clinicoradiologic presentation of this rare variant.

Acta neurochirurgica
2023

Intraoral neurofibroma: A case report with emphasis on immunohistochemical markers.

Journal of cancer research and therapeutics
2023

Diagnostic Significance of Ki67 Staining Pattern in Benign Peripheral Nerve Sheath Tumors: An Additional Diagnostic Tool.

International journal of surgical pathology
2022

A Rare Case of Malignant Peripheral Nerve Sheath Tumor of Larynx.

Indian journal of otolaryngology and head and neck surgery : official publication of the Association of Otolaryngologists of India
2023

An unusual cause of posterior interosseous nerve palsy and contribution of ultrasonography to electromyography in a patient with neurofibroma.

Turkish journal of physical medicine and rehabilitation
2022

Hybrid peripheral nerve sheath tumor of parapharyngeal space having features of neurofibroma and schwannoma in an 8-year-old child - A rare entity.

Journal of oral and maxillofacial pathology : JOMFP
2022

Morphological Relation of Peripheral Nerve Sheath Tumors and Nerve Fascicles: Prospective Study and Classification.

Journal of clinical medicine
2022

Nivolumab induced pulmonary sarcoid-like granulomas with a concurrent pleural schwannoma: A pathologic-radiologic correlation of a rare condition mimicking metastatic melanoma.

Annals of diagnostic pathology
2021

Difficulty Breathing or Just a Case of the Nerves? Incidental Finding of Primary Pleural Schwannoma in a COVID-19 Survivor.

Cureus
2021

Prevalence of oral neurofibroma in Central Indian population: A retrospective study of 20 years.

Journal of oral and maxillofacial pathology : JOMFP
2023

Extraneural Sclerosing Perineurioma of the Tongue.

Ear, nose, &amp; throat journal
2021

Artificial Intelligence for Classification of Soft-Tissue Masses at US.

Radiology. Artificial intelligence
2021

Analysis of the effect of intraoperative neuromonitoring during resection of benign nerve sheath tumors on gross-total resection and neurological complications.

Journal of neurosurgery
2021

Human cutaneous neurofibroma matrisome revealed by single-cell RNA sequencing.

Acta neuropathologica communications
2021

A Bayesian approach for diagnostic accuracy of malignant peripheral nerve sheath tumors: a systematic review and meta-analysis.

Neuro-oncology
2020

Multimodal Imaging Characteristics and Diagnostic Approach to Ancient Schwannoma in a Pediatric Patient.

Cureus
2020

Case report of seminal vesical schwannoma treated with conservative strategy.

Medicine
2020

A Case of Dendritic Cell Neurofibroma With Pseudorosettes.

The American Journal of dermatopathology
2020

Unexpected high frequency of neurofibroma in the celiac ganglion of German cattle.

Veterinary research
2020

Benign peripheral nerve sheath tumor of digit versus major-nerve: Comparison of MRI findings.

PloS one
2020

A case report of solitary neurofibroma of the Vas deferens.

Urology case reports
2019

Pediatric peripheral nerve tumors: clinical and surgical aspects.

Child's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery
2019

Small breast Schwannoma in an old female; a case report.

Breast disease
2019

Epithelioid schwannoma: imaging findings on radiographs, MRI, and ultrasound.

Skeletal radiology
2020

Go for the Gold: A "Plane" and Simple Technique for Resecting Benign Peripheral Nerve Sheath Tumors.

Operative neurosurgery (Hagerstown, Md.)
2019

Perineurioma of the Tongue: A Case Report and Review of the Literature.

Journal of oral and maxillofacial surgery : official journal of the American Association of Oral and Maxillofacial Surgeons
2018

Perineurioma of the colon: an uncommon tumor with an unusual location. Report of a case and review of the literature.

Pathologica
2018

Evaluation of diagnostic accuracy and a practical algorithmic approach for the diagnosis of renal masses by FNA.

Cancer cytopathology
2018

Analysis of Peripheral Nerve Schwannoma Pseudocapsule.

World neurosurgery
2018

Cutaneous microcystic/reticular schwannoma: case report and literature review of an exceedingly rare entity with an unusual presentation.

Romanian journal of morphology and embryology = Revue roumaine de morphologie et embryologie
2017

Benign Cutaneous Peripheral Nerve Sheath Tumor with Hybrid Features: Report of Two Cases with Schwannoma/Perineurioma and Schwannoma/Neurofibroma Components.

Dermatopathology (Basel, Switzerland)
2017

"Pure" Suprasellar Schwannoma Presented with Communicating Hydrocephalus: A Case Report.

NMC case report journal
2017

Soft Tissue Perineurioma in a Child With Neurofibromatosis Type 1: A Case Report and Review of the Literature.

Pediatric and developmental pathology : the official journal of the Society for Pediatric Pathology and the Paediatric Pathology Society
2017

Synovial sarcoma mimicking benign peripheral nerve sheath tumor.

Skeletal radiology
2018

Management of peripheral nerve sheath tumors: 17 years of experience at Toronto Western Hospital.

Journal of neurosurgery
2017

Solitary neurofibroma of eyelid masquerading as chalazion.

International medical case reports journal
2017

Microcystic/Reticular Schwannoma of the Frontal Lobe: An Unusual Occurrence.

Case reports in pathology
2017

Benign Peripheral Nerve Sheath Tumor in a Distance Runner.

The Journal of orthopaedic and sports physical therapy
2016

Intraoral Pseudo-Onion Bulb Intraneural Proliferations in a Patient with Hemimandibular Hyperplasia: A Case Report and Review of the Literature.

Head and neck pathology
2016

Benign Peripheral Nerve Sheath Tumor in a Wild Toco Toucan ( Ramphastos toco ).

Journal of avian medicine and surgery
2017

Malignant peripheral nerve sheath tumor in the paraspinal region mimicking a benign peripheral nerve sheath tumor: a case report.

European spine journal : official publication of the European Spine Society, the European Spinal Deformity Society, and the European Section of the Cervical Spine Research Society
2016

Malignant Peripheral Nerve Sheath Tumor.

Surgical oncology clinics of North America
2016

Periprostatic Perineurioma, Description of a Case With Unusual Localization.

Urology case reports
2016

Radiological findings of malignant peripheral nerve sheath tumor: reports of six cases and review of literature.

World journal of surgical oncology
2016

Fine-needle aspiration of soft tissue perineurioma: A comparative analysis of cytomorphology and immunohistochemistry with benign and malignant mimics.

Cancer cytopathology
2016

Perineurioma of scalp in an infant: A case report with short review of literature.

Asian journal of neurosurgery
2016

Epithelioid angiosarcoma arising in schwannoma of the kidney: report of the first case and review of the literature.

World journal of surgical oncology
2015

Malignant Peripheral Nerve Sheath Tumor: MRI and CT Findings.

Case reports in radiology
2015

Solitary Neurofibroma Of The Spermatic Cord: A Case Report.

Journal of radiology case reports
2015

An unusual oral manifestation of type 1 neurofibromatosis: A case report and review of literature.

Journal of natural science, biology, and medicine

Associações

Organizações que acompanham esta doença — pra ter apoio e orientação

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Comunidades

Grupos ativos de quem convive com esta doença aqui no Raras

Ainda não existe comunidade no Raras para Tumor benigno da bainha dos nervos periféricos

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Doenças relacionadas

Doenças com sintomas parecidos — ajudam quem ainda está buscando diagnóstico

Referências e fontes

Bases de dados externas citadas neste artigo

Publicações científicas

Artigos indexados no PubMed ligados a esta doença no grafo RarasNet — título, periódico e PMID direto da fonte, sem intermediação de IA.

  1. Benign Peripheral Nerve Sheath Tumors: Recurrence Rates and Pain Outcomes Following Excision.
    Plastic and reconstructive surgery· 2026· PMID 41874286mais citado
  2. Posterior Interosseous Nerve Schwannoma Presenting as a Painless Forearm Mass: A Case Report.
    Cureus· 2026· PMID 41869218mais citado
  3. A rare case report of solitary neurofibroma arising in the male breast.
    International journal of surgery case reports· 2026· PMID 41836985mais citado
  4. A rare case of plexiform neurofibroma.
    Radiology case reports· 2026· PMID 41625572mais citado
  5. Symptomatic Schwannomatosis of the Brachial Plexus: A Case Report and Literature Review.
    Annals of plastic surgery· 2026· PMID 41576255mais citado

Bases de dados e fontes oficiais

Identificadores e referências canônicas usadas para montar este verbete.

  1. ORPHA:252131(Orphanet)
  2. MONDO:0016752(MONDO)
  3. Variantes catalogadas(ClinVar)
  4. Busca completa no PubMed(PubMed)
  5. Q55786405(Wikidata)

Dados compilados pelo RarasNet a partir de fontes abertas (Orphanet, OMIM, MONDO, PubMed/EuropePMC, ClinicalTrials.gov, DATASUS, PCDT/MS). Este conteúdo é informativo e não substitui avaliação médica.

Conteúdo mantido por Agente Raras · Médicos e pesquisadores podem colaborar

Tumor benigno da bainha dos nervos periféricos
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Tumor benigno da bainha dos nervos periféricos

ORPHA:252131 · MONDO:0016752
MedGen
UMLS
C5577926
EuropePMC
Wikidata
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