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Perineurioma
ORPHA:85102CID-10 · C47.9DOENÇA RARA

Um perineurioma geralmente benigno, não associado a um nervo, que se origina nos tecidos moles.

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Introdução

O que você precisa saber de cara

📋

Um perineurioma geralmente benigno, não associado a um nervo, que se origina nos tecidos moles.

Publicações científicas
508 artigos
Último publicado: 2025 Dec
🏥
SUS: Sem cobertura SUSScore: 0%
CID-10: C47.9
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Sinais e sintomas

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

Linha do tempo da pesquisa

Publicações por ano — veja quando o interesse científico cresceu
Anos de pesquisa1desde 2026
Total histórico508PubMed
Últimos 10 anos200publicações
Pico202028 papers
Linha do tempo
2026Hoje · 2026📈 2020Ano de pico🧪 2024Primeiro ensaio clínico
Publicações por ano (últimos 10 anos)

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Genética e causas

O que está alterado no DNA e como passa nas famílias

Genes associados

2 genes identificados com associação a esta condição.

HTRA1Serine protease HTRA1Candidate gene tested inTolerante
FUNÇÃO

Serine protease with a variety of targets, including extracellular matrix proteins such as fibronectin. HTRA1-generated fibronectin fragments further induce synovial cells to up-regulate MMP1 and MMP3 production. May also degrade proteoglycans, such as aggrecan, decorin and fibromodulin. Through cleavage of proteoglycans, may release soluble FGF-glycosaminoglycan complexes that promote the range and intensity of FGF signals in the extracellular space. Regulates the availability of insulin-like g

LOCALIZAÇÃO

Cell membraneSecretedCytoplasm, cytosol

VIAS BIOLÓGICAS (1)
Degradation of the extracellular matrix
EXPRESSÃO TECIDUAL(Ubíquo)
Aorta
810.7 TPM
Ovário
602.0 TPM
Cervix Ectocervix
551.9 TPM
Fibroblastos
518.4 TPM
Artéria tibial
426.1 TPM
OUTRAS DOENÇAS (5)
cerebral arteriopathy, autosomal dominant, with subcortical infarcts and leukoencephalopathy, type 2CARASIL syndromemalignant perineuriomaHTRA1-related autosomal dominant cerebral small vessel disease
HGNC:9476UniProt:Q92743
SH3PXD2ASH3 and PX domain-containing protein 2ACandidate gene tested inAltamente restrito
FUNÇÃO

Adapter protein involved in invadopodia and podosome formation, extracellular matrix degradation and invasiveness of some cancer cells (PubMed:27789576). Binds matrix metalloproteinases (ADAMs), NADPH oxidases (NOXs) and phosphoinositides. Acts as an organizer protein that allows NOX1- or NOX3-dependent reactive oxygen species (ROS) generation and ROS localization. In association with ADAM12, mediates the neurotoxic effect of amyloid-beta peptide

LOCALIZAÇÃO

CytoplasmCell projection, podosome

VIAS BIOLÓGICAS (2)
Invadopodia formationCDC42 GTPase cycle
EXPRESSÃO TECIDUAL(Ubíquo)
Cervix Ectocervix
133.9 TPM
Nervo tibial
122.7 TPM
Cervix Endocervix
116.5 TPM
Útero
90.9 TPM
Fibroblastos
75.5 TPM
OUTRAS DOENÇAS (2)
malignant triton tumormalignant perineurioma
HGNC:23664UniProt:Q5TCZ1

Variantes genéticas (ClinVar)

160 variantes patogênicas registradas no ClinVar.

🧬 SH3PXD2A: NM_001394015.1(SH3PXD2A):c.229+121A>G ()
🧬 SH3PXD2A: NM_001394015.1(SH3PXD2A):c.803-3C>G ()
🧬 SH3PXD2A: GRCh37/hg19 10q23.1-25.1(chr10:87456174-107789979)x3 ()
🧬 SH3PXD2A: GRCh37/hg19 10q23.33-26.3(chr10:95078198-135427143)x3 ()
🧬 SH3PXD2A: GRCh37/hg19 10p14-q26.3(chr10:11138692-135427143) ()
Ver todas no ClinVar

Vias biológicas (Reactome)

3 vias biológicas associadas aos genes desta condição.

Diagnóstico

Os sinais que médicos procuram e os exames que confirmam

Carregando...

Tratamento e manejo

Remédios, cuidados de apoio e o que precisa acompanhar

Carregando informações de tratamento...

Onde tratar no SUS

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

🇧🇷 Atendimento SUS — Perineurioma

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Pesquisa ativa

Ensaios clínicos abertos e novidades científicas recentes

Pesquisa e ensaios clínicos

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Publicações mais relevantes

Timeline de publicações
227 papers (10 anos)
#1

The PERISCOPE Cohort: A Retrospective Study of Clinicopathological and TRAF7 Genetic Findings in Intraneural Perineurioma.

European journal of neurology2026 Feb

Intraneural perineurioma (INP) is a rare, benign peripheral nerve sheath tumour that typically presents in adolescence or early adulthood as a slowly progressive, motor-predominant mononeuropathy or plexopathy. Although its clinicoradiological and histopathological features are well characterised, the genetic basis remains incompletely defined. We retrospectively analysed 10 patients with histologically confirmed INP diagnosed between February 2015 and December 2024. Demographic and clinical data, MRI/MR neurography findings and histopathology (immunohistochemistry and electron microscopy) were analysed. Targeted Sanger sequencing of TRAF7 exons 17-18 (WD40 domain) was performed. Interphase FISH with an EWSR1 (22q12) probe was performed on archival FFPE tissue in a subset. All patients exhibited progressive motor deficits, with at least one muscle group graded ≤ 2 on the MRC scale. Sensory symptoms were present in 8/10 and pain in 4/10. MRI demonstrated fusiform nerve enlargement and homogeneous gadolinium enhancement in all cases, with T2 hyperintensity in 9/10. A pathogenic TRAF7 p.His521Arg variant was identified in 2/9 evaluable tumours (22.2%). Tendon transfer was performed in 7/10 patients as a reconstructive strategy to improve motor function, resulting in heterogeneous functional outcomes. The MRI triad of fusiform enlargement, T2 hyperintensity and homogeneous enhancement strongly supports INP diagnosis and may obviate biopsy in typical cases. Our hotspot-limited assay detected TRAF7 mutations in only 22.2%, underscoring methodological limitations and probable genetic heterogeneity. Despite an indolent imaging appearance, INP frequently causes severe functional impairment requiring reconstructive surgery. Early recognition, structured functional monitoring and risk-adapted intervention are essential to optimise outcomes.

#2

An Unsuspected Intraneural Perineurioma in a Pediatric Patient: A Case Report.

Cureus2026 Feb

Perineuriomas are rare tumors arising from perineurial cells that form the protective layer surrounding peripheral nerve fascicles. Four types of perineuriomas have been described: (i) intraneural, (ii) soft tissue (extraneural), (iii) sclerosing, and (iv) mucosal. Intraneural perineuriomas are rarely reported nerve sheath tumors that primarily affect the peripheral nerves of the upper and lower extremities. In this report, we present a pediatric case in which the diagnosis of perineurioma was not suspected until lesional tissue was obtained, and the final pathologic diagnosis was made. The patient is a 17-year-old girl who presented with a three-year history of symptoms involving the left upper extremity, including weakness and cramping, which became progressively worse over time. Diagnostic workup included magnetic resonance imaging (MRI), which showed enlargement and contrast enhancement of two of the left brachial plexus nerve trunks, suggestive of an inflammatory or infectious etiology, with schwannoma or neurofibroma also listed as less likely possibilities. An electromyogram (EMG) showed findings concerning for an anterior horn cell process, including amyotrophic lateral sclerosis (ALS). Nerve conduction studies (NCS) demonstrated axonal findings only in motor nerves, and needle EMG demonstrated denervation and fasciculations in multiple muscles. An initial biopsy of the brachial plexus was performed but was non-diagnostic. Ultimately, resection of the involved nerve trunks was performed. The diagnosis of intraneural perineurioma was not suspected preoperatively and was made only after histologic and immunohistochemical examination.

#3

Cutaneous Perineurioma of the Medial Superciliary Arch: An Uncommon Location for a Rare, Benign Spindle Cell Neoplasm.

Cureus2026 Feb

Cutaneous perineuriomas are rare, benign peripheral nerve sheath tumors that are often mistaken for other adnexal or spindle cell lesions, with facial involvement being uncommon. Recognition is important to ensure accurate diagnosis and avoid unnecessary overtreatment. We report a case of a 36-year-old Hispanic woman with a slowly enlarging papule of the left medial superciliary arch, histologically and immunophenotypically confirmed as a cutaneous perineurioma. This case highlights the clinicopathologic features, immunohistochemical profile, and management considerations of cutaneous perineurioma arising in a rare, cosmetically sensitive facial location, adding to the limited body of published cases.

#4

Benign cutaneous plexiform hybrid tumour of perineurioma and cellular neurothekeoma: A rare diagnosis.

Revista espanola de patologia : publicacion oficial de la Sociedad Espanola de Anatomia Patologica y de la Sociedad Espanola de Citologia2026

Benign cutaneous plexiform hybrid tumour of perineurioma and cellular neurothekeoma (BCPHTPCN) is an uncommon mesenchymal tumour first described in 2013, characterised by mixed features of perineurioma and cellular neurothekeoma. Clinically, it presents as a small papule, most frequently located in the perioral region. Histologically, it displays a plexiform pattern composed of epithelioid and spindle cells within a myxoid stroma, together with a hybrid immunohistochemical profile. In this article, we describe the case of an 82-year-old woman presenting with a perioral nodule clinically suspected to be a basal cell carcinoma, whose histological and immunohistochemical findings confirmed the diagnosis of BCPHTPCN. The tumour's features, differential diagnoses, and the need for further studies to better define its clinicopathological spectrum are discussed.

#5

Hybrid Nerve Sheath Tumor Detected by Endoscopic Full-Thickness Resection for a Gastric Subepithelial Lesion: A Case Report.

DEN open2026 Apr

Hybrid nerve sheath tumors (HNSTs) are exceedingly rare in the gastrointestinal tract, particularly in the stomach. We describe a case of an enlarging gastric subepithelial lesion (SEL) that was accurately diagnosed and curatively treated by endoscopic full-thickness resection (EFTR). A 50-year-old woman presented with a 10 mm SEL on the posterior wall of the upper gastric curvature. Endoscopic ultrasound (EUS) revealed a low-hypoechoic lesion primarily originating from the third layer with focal, indistinct borders with the muscularis propria. Initial boring biopsy suggested a granular cell tumor based on morphology and SOX10/S100 positivity. Six months later, the lesion had enlarged to 15 mm, and EFTR under general anesthesia with laparoscopic backup was selected to obtain a full-thickness specimen. En bloc resection was successfully achieved, and the defect was completely closed with clips. Histopathological and immunohistochemical examinations revealed biphasic Schwann and perineurial differentiation, confirming a hybrid schwannoma/perineurioma. The postoperative course was uneventful, and no recurrence was observed during the 22-month follow-up. This case highlights the diagnostic value of EFTR for rare neurogenic SELs in which superficial biopsy may be inconclusive.

Publicações recentes

Ver todas no PubMed

📚 EuropePMC329 artigos no totalmostrando 197

2026

An Unsuspected Intraneural Perineurioma in a Pediatric Patient: A Case Report.

Cureus
2026

Cutaneous Perineurioma of the Medial Superciliary Arch: An Uncommon Location for a Rare, Benign Spindle Cell Neoplasm.

Cureus
2026

The PERISCOPE Cohort: A Retrospective Study of Clinicopathological and TRAF7 Genetic Findings in Intraneural Perineurioma.

European journal of neurology
2026

Benign cutaneous plexiform hybrid tumour of perineurioma and cellular neurothekeoma: A rare diagnosis.

Revista espanola de patologia : publicacion oficial de la Sociedad Espanola de Anatomia Patologica y de la Sociedad Espanola de Citologia
2026

Hybrid Nerve Sheath Tumor Detected by Endoscopic Full-Thickness Resection for a Gastric Subepithelial Lesion: A Case Report.

DEN open
2025

Sclerosing perineurioma of the anterior chest wall in an active Caucasian toddler: a case report.

Translational pediatrics
2026

Orbital soft-tissue perineurioma.

Journal francais d'ophtalmologie
2025

Distinct Imaging Features of Peripheral Nerve Sheath Tumours in NF2-Related Schwannomatosis: A Case Report.

Case reports in neurological medicine
2025

Extraneural Soft Tissue Perineurioma of the Oral Cavity: A Rare Case with Medico-Legal Implications and Literature Review.

Life (Basel, Switzerland)
2025

Beyond Hybrid Morphology: A Large Series of Fusion-Driven Benign Peripheral Nerve Sheath Tumors Including 5 Tumors With Novel Fusions.

Modern pathology : an official journal of the United States and Canadian Academy of Pathology, Inc
2025

Utility of ultrasound in the diagnosis and management of a radial nerve perineurioma in a pediatric patient.

Surgical neurology international
2025

Perineuriomatous Nevus: A New Case Presentation With a Brief Review of the Literature.

The American Journal of dermatopathology
2025

The Role of Ultrasound in the Diagnosis of Intraneural Perineurioma.

Muscle & nerve
2025

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

Case reports in gastrointestinal medicine
2025

Hybrid Benign Peripheral Nerve Sheath Tumors: A Comprehensive Literature Review with Emphasis on Their Clinical, Morphological and Genetic Features.

Diagnostics (Basel, Switzerland)
2025

A Case of Perineurioma Recurring in the Mandible.

Journal of plastic and reconstructive surgery
2025

Hybrid granular cell tumor/perineurioma: a report of two rare cases with PIK3CA mutations.

Virchows Archiv : an international journal of pathology
2025

Epithelial-Stromal Polyp With Features of Perineurioma Lacking Covering Serrated Crypts Associated With a Sessile Serrated Lesion With High-Grade Dysplasia of the Colon: A Case Report.

Cureus
2025

Neural or Neural-Related Colorectal Lesion Incidence Varies by Site, and Multifocal Cases Are Often Syndromic: Insights From a Series of 593 Patients.

Archives of pathology & laboratory medicine
2025

Intraneural Pseudoperineuriomatous Proliferations and Traumatic Neuromas: A Retrospective Multicenter Study of Clinicopathological Characteristics.

Head and neck pathology
2025

Hybrid nerve sheath tumor of the spinal canal and neurofibromatosis-2, where the twain shall meet-a case report and review of literature.

Journal of medical case reports
2025

A Case of Hybrid Cellular Neurothekeoma and Perineurioma With a Folliculin Gene Mutation.

Journal of cutaneous pathology
2024

Hybrid peripheral nerve sheath tumours - A Review.

Journal of oral and maxillofacial pathology : JOMFP
2025

Glucose Transporter 1 (GLUT1) Positivity in Ganglion Cysts: A Diagnostic Caveat Due to Overlap With Perineuriomas and Other Fibromyxoid Lesions.

Cureus
2025

Acral Cutaneous Intraneural Pseudoperineurioma.

The American Journal of dermatopathology
2025

Hybrid Epithelioid Schwannoma/Neurofibroma: A Report of 3 Cases.

The American Journal of dermatopathology
2025

Endobronchial ultrasound-guided cryobiopsy of a rare epicardial tumour.

Pulmonology
2025

Male breast perineurioma: a rare presentation.

BMJ case reports
2024

A Rare Case of Perineurioma of the Posterior Interosseous Nerve: A Case Report.

Cureus
2024

Gastrointestinal stromal tumor mimicking perineurioma: A case report.

CytoJournal
2024

Gastric perineurioma: a rare entity with different clinical presentations. A case report.

Journal of gastrointestinal and liver diseases : JGLD
2024

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

Cureus
2025

Perineuriomatous Melanocytic Nevi: A Case Series of Four Cases.

Journal of cutaneous pathology
2024

Pseudolipoblastic Perineuroma: A Rare Histologi̇c Subtype.

The American Journal of dermatopathology
2025

Rare Hybrid Perineurioma and Granular Cell Tumor: A Pediatric Case.

Pediatric and developmental pathology : the official journal of the Society for Pediatric Pathology and the Paediatric Pathology Society
2024

EMA-Positive Superficial ALK-Rearranged Myxoid Spindle Cell Neoplasm Masquerading as Perineurioma/Hybrid Nerve Sheath Tumor.

The American Journal of dermatopathology
2024

Collection of Rare Peripheral Nerve Tumors-Insights from the German Registry.

Cancers
2024

Sclerosing Perineurioma of the Orbit.

Ophthalmic plastic and reconstructive surgery
2024

Mesenchymal neoplasms of the tongue: A clinicopathologic study of 93 cases.

Human pathology
2024

Feasibility study of ResNet-50 in the distinction of intraoral neural tumors using histopathological images.

Journal of oral pathology & medicine : official publication of the International Association of Oral Pathologists and the American Academy of Oral Pathology
2024

Surgical treatment of perineurioma involving both the intramedullary and extramedullary nerve roots in the cervical spine.

Asian journal of surgery
2024

Malignant perineurioma derived from the retroperitoneum with an aggressive clinical course: a case report.

Surgical case reports
2024

Small intestinal obstruction caused by perineurioma: A case report and review of literature.

Asian journal of surgery
2024

A 22-year-old man with a posterior left shoulder mass.

Skeletal radiology
2024

Cutaneous Syncytial Myoepithelioma: An Uncommon and Distinct Variant of Cutaneous Epithelioid Neoplasm.

The American Journal of dermatopathology
2024

Utility of LEF1 to differentiate desmoid fibromatosis from its histologic mimics.

Virchows Archiv : an international journal of pathology
2024

Treatment of childhood intraneural perineurioma: A case report and literature review.

Heliyon
2024

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

In vivo (Athens, Greece)
2024

Gastric Perineurioma: A Rare Entity with Molecular Analysis and Literature Review.

International journal of surgical pathology
2023

Case report: Intraneural perineurioma in dogs: a case series and brief literature review.

Frontiers in veterinary science
2024

Hybrid peripheral nerve sheath tumours: MRI features with pathological correlation in 24 cases.

The British journal of radiology
2024

Cytological features of cranial and paraspinal nerve Tumours.

Cytopathology : official journal of the British Society for Clinical Cytology
2024

CYP1A1 immunohistochemistry is highly specific for angiofibroma of soft tissue among morphological mimics.

Histopathology
2025

Hybrid Schwannoma/Neurofibroma of the Neck: A Case Report.

Ear, nose, & throat journal
2023

What is new in intraneural perineurioma?

Acta neurochirurgica
2023

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

Neurosurgical review
2023

Perineurioma Diagnosed After Endoscopic Treatment of Suspected Gastric Adenocarcinoma of the Fundic Gland.

ACG case reports journal
2024

Hybrid Schwannoma-Perineurioma of the Orbit.

Ophthalmic plastic and reconstructive surgery
2023

Rare peripheral nerve tumor of the median nerve.

Hand surgery & rehabilitation
2023

Gene fusions in superficial mesenchymal neoplasms: Emerging entities and useful diagnostic adjuncts.

Seminars in diagnostic pathology
2023

Superficial mesenchymal tumours expressing epithelial markers on immunohistochemistry: Diagnostic clues and pitfalls.

Seminars in diagnostic pathology
2023

Diagnosis and Treatment of Peripheral and Cranial Nerve Tumors with Expert Recommendations: An EUropean Network for RAre CANcers (EURACAN) Initiative.

Cancers
2023

Myxoid perineurioma: an entity with many mimics.

Dermatology online journal
2023

Perineurioma of the sciatic nerve with reticular, microcystic, and pseudolipoblastic features: illustrative case.

Journal of neurosurgery. Case lessons
2023

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

Journal of cutaneous pathology
2023

Epithelioid perineurioma of the vulva: A case report.

The Journal of dermatology
2023

GAB1::ABL1 fusions define a distinctive soft tissue neoplasm, with variable perineurial differentiation, and a predilection for children and young adults.

Genes, chromosomes & cancer
2022

Assessing the role of sodium fluorescein in peripheral nerve sheath tumors and mimicking lesions surgery: An update after 142 cases.

Frontiers in oncology
2023

Ultrasonography of peripheral nerve tumours: a case series.

Radiology and oncology
2023

Benign peripheral nerve tumors: Analysis of 89 FNA cases emphasizing diagnostic accuracy and their morphologic spectrum.

Cancer cytopathology
2023

Colonic perineurioma of the transverse colon: presentation of a rare neoplasm in an atypical location.

Revista espanola de enfermedades digestivas
2023

Surgical Management of Peripheral Nerve Pathology in Patients With Neurofibromatosis Type 2.

Neurosurgery
2023

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

Acta neurochirurgica
2023

Hybrid tumors with perineurioma components: a systematic review of the literature and illustrative case.

Acta neurochirurgica
2023

Letter to Editor Regarding "Hybrid Neurofibroma/Schwannoma of the Oral Cavity: A Rare Case Report and Literature Review" by Leite et al.

International journal of surgical pathology
2022

A Rare Case of Bladder Perineurioma.

Cureus
2022

Sclerosing Perineurioma: a Rare Cause of Thumb Mass.

Journal of general internal medicine
2022

Recurrent VGLL3 fusions define a distinctive subset of spindle cell rhabdomyosarcoma with an indolent clinical course and striking predilection for the head and neck.

Genes, chromosomes & cancer
2023

RREB1::MRTFB fusion-positive extra-glossal mesenchymal neoplasms: A series of five cases expanding their anatomic distribution and highlighting significant morphological and phenotypic diversity.

Genes, chromosomes & cancer
2022

Practical Approach to Histological Diagnosis of Peripheral Nerve Sheath Tumors: An Update.

Diagnostics (Basel, Switzerland)
2022

Benign Cutaneous Plexiform Hybrid Tumor Composed of Agminated Perineurioma and Cellular Neurothekeoma Components in a 9-Month-Old Boy.

Actas dermo-sifiliograficas
2023

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

International journal of surgical pathology
2022

Histopathological and Immunohistochemical Evaluation of Canine Nerve Sheath Tumors and Proposal for an Updated Classification.

Veterinary sciences
2021

Sclerosing perineurioma: a rare benign tumor in a pediatric patient.

Dermatology online journal
2022

Acral perineurioma: A case report of a rare neoplasm successfully treated with autologous skin graft reconstructive surgery.

Dermatology reports
2022

A Rare case of Ulnar Nerve Intraneural Perineurioma in an elderly gentleman.

JPRAS open
2022

Unilateral Cavovarus Foot From Sciatic Nerve Perineurioma.

Neurology India
2022

Clinicopathologic and Molecular Study of Hybrid Nerve Sheath Tumors Reveals Their Common Association With Fusions Involving VGLL3.

The American journal of surgical pathology
2024

Pediatric Soft Tissue Perineurioma in the Head and Neck.

Ear, nose, & throat journal
2022

Clinical, pathologic, and molecular analyses of superficial low-grade fibromyxoid sarcoma in two young patients: A rare and deceptive mimic of benignancy.

Journal of cutaneous pathology
2022

Port-Wine Stain and Monomelic Weakness Associated With Perineurioma: A Neuromuscular Image.

Journal of clinical neuromuscular disease
2022

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

Journal of clinical medicine
2021

[Sclerosing perineurioma of the left hand middle finger: report of a case].

Zhonghua bing li xue za zhi = Chinese journal of pathology
2021

Soft tissue perineurioma involving the kidney: a report of two cases with an emphasis on differential diagnosis.

Diagnostic pathology
2021

Nerve conduction assessment and magnetic resonance imaging for the diagnosis of localized hypertrophic neuropathy of the sciatic nerve and the lumbo-sacral plexus.

Clinical neurology and neurosurgery
2021

Update on Peripheral Nerve Sheath Tumors in Upper Extremity: A Descriptive Review.

The journal of hand surgery Asian-Pacific volume
2022

Mesenchymal PLAG1 Tumor With PCMTD1-PLAG1 Fusion in an Infant: A New Type of "Plagoma".

The American Journal of dermatopathology
2021

Clinical Features, Natural History, and Outcomes of Intraneural Perineuriomas: A Systematic Review of the Literature.

World neurosurgery
2021

A Solitary Sigmoid Perineurioma in an Otherwise Healthy 30-Year-Old Male.

Cureus
2023

Trigeminal hybrid nerve sheath tumor - a case report and literature review.

British journal of neurosurgery
2023

Extraneural Sclerosing Perineurioma of the Tongue.

Ear, nose, & throat journal
2022

Perineurioma: A Rare Entity of Peripheral Nerve Sheath Tumors.

Journal of neurological surgery. Part A, Central European neurosurgery
2021

Benign cutaneous plexiform hybrid tumor of perineurioma and cellular neurothekeoma on the leg.

The Journal of dermatology
2021

Eyelid-Cheek Junction Soft-Tissue Perineurioma: Case Report and Controversies.

Ophthalmic plastic and reconstructive surgery
2021

Perineuriomatous nevi: A series of eight cases highlighting unifying pathologic features to avoid misdiagnosis.

Journal of cutaneous pathology
2021

[Intra-oral Perineurioma, a case report].

Nederlands tijdschrift voor tandheelkunde
2021

Hybrid schwannoma-perineurioma frequently harbors VGLL3 rearrangement.

Modern pathology : an official journal of the United States and Canadian Academy of Pathology, Inc
2021

Sporadic Malignant Perineurioma: A Rare Diagnosis Among Malignant Peripheral Nerve Sheath Tumors.

World neurosurgery
2021

Intraneural perineurioma of the median nerve at the elbow: A clinical case.

Hand surgery & rehabilitation
2021

Cap-assisted Endoscopic Mucosal Resection of Rectal Perineurioma Mimicking a Neuroendocrine Tumor.

The Korean journal of gastroenterology = Taehan Sohwagi Hakhoe chi
2021

Diagnostic Pathology of Tumors of Peripheral Nerve.

Neurosurgery
2020

From Dyspepsia to Diagnosis: A Rare Gastric Subepithelial Lesion Definitively Diagnosed via Endoscopic Submucosal Dissection and Immunohistochemistry.

The Permanente journal
2021

Intrinsic peripheral nerve and root tumor and pseudotumoral lesions at a tertiary care pediatric hospital.

Child's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery
2021

Hybrid Schwannoma/Perineurioma: Morphologic Variations and Genetic Profiles.

Applied immunohistochemistry & molecular morphology : AIMM
2021

Perineurioma versus meningioma. A multi-institutional immunohistochemical and ultrastructural studY.

Ultrastructural pathology
2021

Epithelial-Stromal Polyps of the Colon Are Not Perineuriomas.

American journal of clinical pathology
2020

Peripheral nerve tumors of the hand: Clinical features, diagnosis, and treatment.

World journal of clinical cases
2020

Peripheral Nerve Sheath Tumors of Head and Neck: Imaging-Based Review of World Health Organization Classification.

Journal of computer assisted tomography
2020

Orbital hybrid peripheral nerve sheath tumors.

Taiwan journal of ophthalmology
2020

[Informative value of ultrasound in the diagnosis of intraneural perineurioma].

Zhurnal voprosy neirokhirurgii imeni N. N. Burdenko
2021

Orbital hybrid schwannoma/perineurioma: A case presentation.

Pathology international
2020

Primary intra-osseous Hybrid Schwannoma-Perineurioma in the mandible.

Journal of clinical and experimental dentistry
2020

[Intraneural perineurioma of the radial nerve in the spiral canal as the cause of a 6-year progressive paresis of hand and finger extension].

Der Nervenarzt
2020

Intraneural perineurioma of the mandible: case series of a rare entity.

Oral surgery, oral medicine, oral pathology and oral radiology
2020

Recurrent Fusion of the GRB2 Associated Binding Protein 1 (GAB1) Gene With ABL Proto-oncogene 1 (ABL1) in Benign Pediatric Soft Tissue Tumors.

Cancer genomics & proteomics
2020

Facial Nerve Intraneural Perineurioma Masquerading as a Schwannoma.

JAMA otolaryngology-- head & neck surgery
2021

Intraneural perineurioma with thenar atrophy presenting as congenital absence of thenar muscles.

Clinical anatomy (New York, N.Y.)
2020

Supraclavicular Solitary Hybrid Schwannoma/Neurofibroma: A Case Report.

Cureus
2020

Association of intraneural perineurioma with neurofibromatosis type 2.

Acta neurochirurgica
2020

Limb Undergrowth in Intraneural Perineuriomas: An Under-Recognized Association.

World neurosurgery
2020

Onychomatricoma Presenting as a Dystrophic Right Great Toenail: Case Report and Review.

Cureus
2020

Endoscopic Mucosal Resection of a Gastric Perineurioma.

ACG case reports journal
2020

Intramucosal true perineurioma of the sigmoid: endoscopic and pathological features of an infrequent lesion.

Endoscopy
2020

Intraneural perineurioma in neurofibromatosis type 2 with molecular analysis.

Clinical neuropathology
2020

Penile pacinian neurofibroma.

Revista espanola de patologia : publicacion oficial de la Sociedad Espanola de Anatomia Patologica y de la Sociedad Espanola de Citologia
2020

Decision Making in Retroperitoneal Nerve Sheath and Nerve-Associated Tumors: A Modular Approach.

Neurosurgery
2020

Modern treatment of perineuriomas: a case-series and systematic review.

BMC neurology
2020

A Rare Perineurioma/Granular Cell Tumor Hybrid Peripheral Nerve Sheath Tumor.

The American Journal of dermatopathology
2020

Perineurioma -a rare cause of proptosis.

Orbit (Amsterdam, Netherlands)
2019

Novel case of hybrid perineurioma-neurofibroma of the orbit.

Canadian journal of ophthalmology. Journal canadien d'ophtalmologie
2020

The differential diagnosis between sclerotic fibroma and sclerosing perineurioma: An unresolved challenge.

Journal of cutaneous pathology
2020

What's new in nerve sheath tumors.

Virchows Archiv : an international journal of pathology
2020

Spindle cell proliferations of the sigmoid colon, rectum and anus: a review with emphasis on perineurioma.

Histopathology
2020

Sodium Fluorescein as Intraoperative Visualization Tool During Peripheral Nerve Biopsies.

World neurosurgery
2019

[Hybrid schwannoma/perineurioma: a clinicopathological analysis of 35 cases].

Zhonghua bing li xue za zhi = Chinese journal of pathology
2019

Colonic Perineurioma's Malignant Proximity to Serrated Colonic Polyps.

Cureus
2020

Intraneural granular cell tumor: Histologic spectrum and histogenetic implication.

Journal of cutaneous pathology
2020

Extraneural perineurioma: CT and MRI imaging characteristics.

Skeletal radiology
2019

Primary intraosseous hybrid epithelioid schwannoma/perineurioma in the proximal tibia: a case report of benign hybrid neoplasm with local hypercellularity.

Diagnostic pathology
2019

Intramucosal fat is uncommon in large bowel polyps but raises three differential diagnoses.

Journal of clinical pathology
2019

Gastric hybrid schwannoma/perineurioma.

Cirugia espanola
2019

Atypical Endoscopic Findings of Rectal Perineurioma.

Internal medicine (Tokyo, Japan)
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

Intraneural perineurioma: a retrospective study of 19 patients.

The Pan African medical journal
2018

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

Pathologica
2019

Mucosal Schwann Cell Hamartoma of the Rectosigmoid Junction: A Rare Lesion Mimicking Mucosal Prolapse Syndrome and Other Neural Lesions.

International journal of surgical pathology
2018

A wolf in sheep's clothing: a benign perineurioma-like lesion that is not what it seems.

Singapore medical journal
2019

Clinical and Radiological Follow-up of Intraneural Perineuriomas.

Neurosurgery
2018

Recurrent Genomic Alterations in Soft Tissue Perineuriomas.

The American journal of surgical pathology
2018

Perineurioma of the sigmoid colon.

BMJ case reports
2018

Intraosseous intraneural perineurioma derived from the inferior alveolar nerve with an abnormality of chromosome 22 and expression of the BCR-ABL fusion gene: report of a case and review of recent literature.

World journal of surgical oncology
2018

Low-fat, plexiform spindle cell lipoma of the lip expressing S100 protein: a neural tumor simulator.

Romanian journal of morphology and embryology = Revue roumaine de morphologie et embryologie
2018

Serrated epithelial colorectal polyps (hyperplastic polyps, sessile serrated adenomas) with perineurial stroma: Clinicopathological and molecular analysis of a new series.

Annals of diagnostic pathology
2018

Large polypoid perineurioma of the transverse colon without crypt serration (NPS).

International journal of clinical and experimental pathology
2018

Left Supraclavicular Swelling: Sclerosing Perineurioma.

The Eurasian journal of medicine
2018

Reconstruction of the Shoulder Joint with a Custom-Made Ceramic Implant After a Total Scapulectomy: A Case Report.

JBJS case connector
2018

Multiple perineuriomatous melanocytic nevi.

Journal of cutaneous pathology
2018

Colonic perineurioma (benign fibroblastic polyp): case report and review of the literature.

Diagnostic pathology
2017

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

Dermatopathology (Basel, Switzerland)
2018

A Perineurioma Treated With Mohs Micrographic Surgery.

Dermatologic surgery : official publication for American Society for Dermatologic Surgery [et al.]
2018

Low-grade fibromyxoid sarcoma arising within the median nerve.

Neuropathology : official journal of the Japanese Society of Neuropathology
2017

Anesthesia for a parturient with intraneural perineurioma: A case report.

Medicine
2018

Clinicoradiological features of intraneural perineuriomas obviate the need for tissue diagnosis.

Journal of neurosurgery
2018

A Sclerosing Perineurioma With Collagen Rosette Formation: Benign Mimic of Low-Grade Fibromyxoid Sarcoma.

International journal of surgical pathology
2018

Intraneural perineuriomas: diagnostic value of magnetic resonance neurography.

Journal of the peripheral nervous system : JPNS
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

Hybrid peripheral nerve sheath tumors: A review.

Ceskoslovenska patologie
2017

Hybrid peripheral nerve sheath tumors: report of five cases and detailed review of literature.

BMC cancer
2017

Gastric perineurioma: clinicopathological characteristics.

Pathology
2017

Primary Extracranial Meningioma: A Rare Location.

Head and neck pathology
2017

Spontaneous spinal plexiform perineurioma in a juvenile chicken ( Gallus domesticus).

Journal of veterinary diagnostic investigation : official publication of the American Association of Veterinary Laboratory Diagnosticians, Inc
2017

Whorling cellular perineurioma: A previously undescribed variant closely mimicking monophasic fibrous synovial sarcoma.

Annals of diagnostic pathology
2017

Intranodal meningothelial proliferation in a patient with Cowden syndrome: a case report.

Human pathology
2017

Teaching NeuroImages: Sonographic detection of intraneural perineurioma in therapy-refractory carpal tunnel syndrome.

Neurology
2017

Targeted fascicular biopsy of the brachial plexus: rationale and operative technique.

Neurosurgical focus
2017

Can Intraneural Perineuriomas Occur Intradurally? An Anatomic Perspective.

Neurosurgery
2017

Sclerosing Dermatofibrosarcoma Protuberans Shows Significant Overlap With Sclerotic Fibroma in Both Routine and Immunohistochemical Analysis: A Potential Diagnostic Pitfall.

The American Journal of dermatopathology
2017

Extra-acral cutaneous sclerosing perineurioma with CD34 fingerprint pattern.

Journal of cutaneous pathology
2017

Oral perineurioma: clinicopathologic features from two cases and review of literature.

Oral surgery, oral medicine, oral pathology and oral radiology
2017

Genomic analysis reveals frequent TRAF7 mutations in intraneural perineuriomas.

Annals of neurology
2017

Reticular Perineurioma of the Hand: Diagnosis and Treatment of a Rare Case of Hand Mass.

The Journal of hand surgery
2017

Protein gene product 9.5 (PGP9.5) expression in benign cutaneous mesenchymal, histiocytic, and melanocytic lesions: comparison with cellular neurothekeoma.

Pathology
2017

Broadening the Anatomic Landscape of Sclerosing Perineurioma: A Series of 5 Cases in Nonacral Sites.

The American Journal of dermatopathology
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

Pseudolipoblastic perineurioma: an unusual morphological variant of perineurioma that may simulate liposarcoma.

Human pathology
2016

Intratemporal Intraneural Perineurioma of the Facial Nerve.

Otology & neurotology : official publication of the American Otological Society, American Neurotology Society [and] European Academy of Otology and Neurotology
2016

Case report: Intraneural perineurioma of the sciatic nerve in an adolescent - strategies for revealing the diagnosis.

Clinical case reports
Ver todos os 329 no EuropePMC

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Doenças relacionadas

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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. The PERISCOPE Cohort: A Retrospective Study of Clinicopathological and TRAF7 Genetic Findings in Intraneural Perineurioma.
    European journal of neurology· 2026· PMID 41657107mais citado
  2. An Unsuspected Intraneural Perineurioma in a Pediatric Patient: A Case Report.
    Cureus· 2026· PMID 41822653mais citado
  3. Cutaneous Perineurioma of the Medial Superciliary Arch: An Uncommon Location for a Rare, Benign Spindle Cell Neoplasm.
    Cureus· 2026· PMID 41798498mais citado
  4. Benign cutaneous plexiform hybrid tumour of perineurioma and cellular neurothekeoma: A rare diagnosis.
    Revista espanola de patologia : publicacion oficial de la Sociedad Espanola de Anatomia Patologica y de la Sociedad Espanola de Citologia· 2026· PMID 41547237mais citado
  5. Hybrid Nerve Sheath Tumor Detected by Endoscopic Full-Thickness Resection for a Gastric Subepithelial Lesion: A Case Report.
    DEN open· 2026· PMID 41536645mais citado
  6. Gastric perineurioma detected in routine endoscopy biopsy practice: case report and review of clinico-pathological findings in the literature.
    Pathologica· 2025· PMID 41954346recente
  7. Novel perineurioma-like soft tissue tumour with SETD1A::FOSB fusion.
    Pathology· 2026· PMID 41927362recente
  8. Perineurioma.
    Mayo Clin Proc· 2026· PMID 41920111recente

Bases de dados e fontes oficiais

Identificadores e referências canônicas usadas para montar este verbete.

  1. ORPHA:85102(Orphanet)
  2. MONDO:0019404(MONDO)
  3. GARD:12698(GARD (NIH))
  4. Variantes catalogadas(ClinVar)
  5. Busca completa no PubMed(PubMed)
  6. Q1314058(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

Perineurioma
Compêndio · Raras BR

Perineurioma

ORPHA:85102 · MONDO:0019404
CID-10
C47.9 · Neoplasia maligna dos nervos periféricos e sistema nervoso autônomo, não especificados
MedGen
UMLS
C0751691
EuropePMC
Wikidata
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