Tumor maligno da bainha dos nervos periféricos

Metarrestin (ML-246) in Subjects With Metastatic Solid Tumors

NCT04222413

B7-H3-Specific Chimeric Antigen Receptor Autologous T-Cell Therapy for Pediatric Patients With Solid Tumors (3CAR)

NCT04897321

Multi-Institutional Registry for Malignant Peripheral Nerve Sheath Tumors

NCT03141021

SPEARHEAD-3 Pediatric Study

Fase II

NCT05642455

A Study of ASTX727 in People With Malignant Peripheral Nerve Sheath Tumors (MPNST)

NCT04872543

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PHASE1, PHASE2

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EARLY_PHASE1

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Publicações mais relevantes

Timeline de publicações

1.109 papers (10 anos)

Mostrando amostra de 200 publicações de um total de 1.109

#1

Giant malignant peripheral nerve sheath tumor: Illustrative case and surgical technique.

Surgical neurology international2026

Malignant peripheral nerve sheath tumors (MPNSTs) are one of the most difficult malignant mesenchymal neoplasms to treat. They can arise from a preexisting peripheral nerve sheath or in neurofibromatosis type 1 (NF1) patients. MPNSTs in the head-and-neck region represent approximately 2-6% of all head-and-neck sarcomas and account for 12-19% of all MPNSTs. It has an incidence of 0.001% in the general population, and prognosis is typically poor; only between 20% and 50% of patients survive 5 years after diagnosis. The complete surgical resection is recommended whenever possible. The authors report the case of a 31-year-old woman with a history of plexiform neurofibroma, who presented with recurrence with disease progression to MPNST, without association with NF1 disease. The aim of tumor resection is symptomatic relief. These tumors have a high risk of severe complications and even mortality due to various causes. The benefits - including the emotional ones - can be so significant that, despite their malignant lineage, resection and regrowth control remain worthwhile for as long as possible. Four- or six-hand surgery for these tumors can reduce bleeding, operative time, and complications.

#2

Misdiagnosed tuberculous myelitis turned out to be spinal malignant peripheral nerve sheath tumor: a case report.

Neurological sciences : official journal of the Italian Neurological Society and of the Italian Society of Clinical Neurophysiology2026 Jan 12

Malignant peripheral nerve sheath tumors (MPNSTs) are rare, aggressive soft tissue sarcomas. Spinal involvement is uncommon, representing only 2-3% of all cases, and is typically associated with poor prognosis. Early diagnosis is challenging due to non-specific clinical and imaging findings. A 34-year-old woman presented with progressive lower limb weakness and numbness. Cerebrospinal fluid analysis revealed elevated protein and decreased glucose and chloride levels. MRI showed spinal meningeal enhancement, initially suggestive of tuberculous myelitis. Despite prolonged anti-tuberculous treatment, her condition worsened. Repeat imaging revealed an intradural extramedullary mass compressing the spinal cord. Surgical biopsy confirmed MPNST based on spindle-shaped tumor cells and complete loss of H3K27me3 expression. This case illustrates the diagnostic challenges of spinal MPNSTs, which may mimic tuberculous myelitis. When clinical features and imaging are inconclusive and response to treatment is poor, early histopathological examination remains essential for accurate diagnosis and timely management.

#3

[Malignant peripheral nerve sheath tumor presenting with Pancoast syndrome in a ‍patient with neurofibromatosis type 1].

Rinsho shinkeigaku = Clinical neurology2026 Jan 22

A 36-year-old woman with a history of neurofibromatosis type 1 (NF1) presented with acute onset of left-hand weakness, numbness, and pain. Neurological examination revealed Horner's syndrome, distal weakness in the C8-T1 myotomes, and sensory disturbances in the corresponding dermatomes. The constellation of these signs was consistent with Pancoast syndrome, involving both the sympathetic trunk and the lower brachial plexus. Nerve conduction studies demonstrated reduced distal compound muscle action potentials and reduced or absent sensory nerve action potentials from multiple nerves in the C8-T1 territories, supporting involvement of the lower trunk. Chest X-ray revealed a large apical mass, and cervical MRI showed that the mass was compressing the brachial plexus at that level, accounting for her symptoms. Histopathological examination of a specimen obtained from the apical mass confirmed malignant peripheral nerve sheath tumor (MPNST). The patient underwent multimodal treatment including partial resection, embolization, radiotherapy, and chemotherapy, which controlled tumor growth and allowed her to maintain functional independence over a two-year follow-up. This case highlights the importance of early recognition of MPNST in NF1 patients presenting with new neurological symptoms. It also underscores the value of careful neurological examination and electrophysiological studies in accurately localizing the responsible lesion.

#4

Epidemiology and prognosis of malignant peripheral nerve sheath tumor (MPNST) in Japan: A population-based analysis using the national cancer registry.

Journal of orthopaedic science : official journal of the Japanese Orthopaedic Association2026 Mar

The national epidemiology of malignant peripheral nerve sheath tumor (MPNST) remains underexplored. We analyzed Japanese domestic statistics for MPNST using the National Cancer Registry (NCR), a population-based database launched in 2016. We analyzed 837 MPNST cases diagnosed between 2016 and 2019, comparing them with 22,685 non-MPNST soft tissue sarcomas. We assessed demographics, treatment, and survival using Kaplan-Meier and Cox proportional hazards models. The age-adjusted incidence of MPNST in Japan was 0.13 per 100,000. Unlike other sarcomas, MPNST showed a balanced sex ratio and a relatively higher proportion of adolescent and young adult (AYA) patients (18.5 % vs. 10.7 %, p < 0.001). Most MPNSTs (87.4 %) originated from the skin and soft tissues. The 3-year survival rate for MPNST patients (53.0 %) was lower than that for other sarcomas (64.3 %, p < 0.001). Surgery was associated with longer survival (hazard ratio [HR]: 2.09; p < 0.001), while no clear benefit was observed for chemotherapy or radiotherapy. Radiotherapy was more frequently used in MPNST patients (21.1 % vs. 15.6 %, p < 0.001), likely reflecting a selection bias towards non-surgical cases. This is the first study to analyze the national epidemiology and prognosis of MPNST in a socio-economically homogeneous, single ethnic group. MPNST primarily affected the skin and soft tissues, with a higher incidence in the AYA group. Surgery was associated with better outcomes, whereas chemotherapy and radiotherapy implied poorer prognosis owing to selection bias for unresectable cases. The NCR provides a valuable model for research on rare diseases in homogeneous populations.

#5

Novel genomic risk stratification model for primary high-grade malignant peripheral nerve sheath tumor (MPNST).

The Journal of pathology2026 Mar 20

Risk stratification across the three main clinical subsets of malignant peripheral nerve sheath tumor (MPNST), neurofibromatosis type I (NF1-related), sporadic, and prior radiation therapy (RT), is based mainly on clinicopathologic parameters, such as size, grade, stage, and NF1 status. Moreover, no prior study investigated the additional impact of genomic alterations in the prognosis of high-grade MPNST using clinically validated DNA targeted next-generation sequencing (NGS) panels. Our goal was to integrate clinicopathologic and genomic parameters using an elastic-net penalized Cox proportional hazards machine learning model using OncoCast for risk prediction. Herein we perform comprehensive mutational and copy number profiling using Memorial Sloan Kettering-Integrated Mutation Profiling of Actionable Cancer Targets (MSK-IMPACT) on 81 primary localized high-grade MPNSTs (51% NF1-related, 38% sporadic, 11% RT-associated). The most common genomic alterations included NF1 (51% germline, 59% somatic), CDKN2A/B (62%), PRC2 components (SUZ12, EED) (53%), and TP53 (25%). Variables selected by OncoCast as significantly associated with survival were used to construct a three-tier risk stratification model for progression-free survival (PFS) and disease-specific survival (DSS). For PFS, patients with chr16 deletion were classified as high-risk, those with concurrent germline and somatic NF1 alterations as low-risk, and the remainder was assigned to the intermediate-risk group. For DSS, cases with fraction genome altered (FGA) > 50% were defined as high-risk, those with PRC2 abnormalities, CDKN2A deletion, TERT promoter mutation, or chr16 deletion as intermediate-risk, and cases lacking all the aforementioned alterations as low-risk. The high-risk group showed significantly inferior survival compared to the low-risk group (both PFS and DSS p < 0.001). Subgroup analysis showed that among NF1-related MPNST, co-occurring somatic NF1 mutation or WT TP53 was associated with superior PFS. Collectively, genomic alterations detected by clinical NGS panels provide potential new biomarkers for risk stratification that can be integrated with conventional parameters to provide improved prognostication and guide therapeutic strategies. © 2026 The Pathological Society of Great Britain and Ireland.

Publicações recentes

O-Arm Navigated En Bloc Resection of a Paraspinal Malignant Peripheral Nerve Sheath Tumor Involving the Thoracic Spine: A Case Report.

Spine Surg Relat Res2026 Mar 27

Near-Bloodless Resection of a Giant Hypervascular NF1-Associated Malignant Peripheral Nerve Sheath Tumor Using Dual-Modality Preoperative Embolization.

Int J Dermatol2026 Apr 12

Malignant Peripheral Nerve Sheath Tumor of the Pancreas: A Case Report and Updated Review of Related Literature.

Acta Med Philipp2026

Defining Prognostic Markers and Clinical Outcomes in Epithelioid Malignant Peripheral Nerve Sheath Tumor.

Mod Pathol2026 Mar 23

Novel genomic risk stratification model for primary high-grade malignant peripheral nerve sheath tumor (MPNST).

J Pathol2026 Mar 20

Ver todas no PubMed

📚 EuropePMC991 artigos no totalmostrando 199

Novel genomic risk stratification model for primary high-grade malignant peripheral nerve sheath tumor (MPNST).

The Journal of pathology

bioRxiv : the preprint server for biology

Driving proteomic imbalance in malignancy provokes proteomic catastrophe and confers tumor suppression.

Trametinib and Fimepinostat Induce Malignant Peripheral Nerve Sheath Tumor Cell Death In Vitro.

Cancers

Clinical nuclear medicine

18F-FDG PET/CT in Epithelioid Malignant Peripheral Nerve Sheath Tumor of the Vagina.

Journal of orthopaedic case reports

Primary Intraosseous Malignant Nerve Sheath Tumor Of Humerus Presenting With Pathological Fracture: A Rare Case Report With Review Of Literature.

Journal of orthopaedic case reports

Challenging Limb Salvage in a Crural Malignant Peripheral Nerve Sheath Tumor with Pre-existing Long-Stem Implants: A Case Report.

Refractory Cancer Pain in a Pediatric Patient With Advanced Malignant Peripheral Nerve Sheath Tumor: A Total Pain-Oriented Palliative Care Approach.

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

Schwannoma: a spectrum from benign to malignant.

Heterologous Rhabdomyoblastic Differentiation in Dedifferentiated Liposarcoma Is Significantly Associated With Loss of H3K27 Trimethylation.

Pathology international

Surgical neurology international

Giant malignant peripheral nerve sheath tumor: Illustrative case and surgical technique.

Breathless and Beyond: Anterior Mediastinal Malignant Peripheral Nerve Sheath Tumor as a Rare Neurofibromatosis Type 1 Manifestation.

Clinical case reports

Single-cell transcriptomic profiling of malignant peripheral nerve sheath tumors.

Neuro-oncology advances

Clinical Outcomes Following Surgical Resection for Patients With Malignant Peripheral Nerve Sheath Tumors.

Neurosurgery

Molecular epidemiology of the expression of urokinase plasminogen activator receptor-associated protein (uPARAP) in mesenchymal malignancies.

Translational oncology

Case Report: Anlotinib combined with radiotherapy achieves sustained disease control in advanced malignant melanotic nerve sheath tumor.

Indocyanine green assists with sentinel lymph node mapping in pediatric and adolescent patients: A Pediatric Surgical Oncology Research Collaborative prospective observational study.

Cancer

Journal of translational medicine

Personalized medicine strategy for MPNSTs: using precision oncology on PDOX models to inform tumor boards.

Journal of surgical case reports

Mammary malignant peripheral nerve sheath tumor in a 93-year-old male: case report.

Journal of surgical case reports

High-grade malignant peripheral nerve sheath tumor of the esophagus: a rare case highlighting the diagnostic value of immunohistochemistry.

Journal of cardiothoracic surgery

Intraoperative pafolacianin for nodule detection during VATS metastasectomy in extremity metastatic sarcoma surgery performed concurrently.

Pathologie (Heidelberg, Germany)

Protein kinase-related tumors in the pediatric population : Updated review on an emerging group with emphasis on the more rarely involved kinases.

Genes, chromosomes & cancer

Biallelic NF1 Inactivation and Widespread Loss of Heterozygosity in a Subset of Unclassified Fibromyxoid Mesenchymal Neoplasms.

Outcomes following definitive treatment of malignant peripheral nerve sheath tumor are significantly worse for patients with neurofibromatosis type 1: A Canadian Sarcoma Research and Clinical Collaboration study.

Cancer

Nagoya journal of medical science

A retrospective study of post-radiation sarcoma from three institutions in Japan with a review of the literature.

Veterinary clinical pathology

Clinical, Cytologic, Histopathologic, and Diagnostic Imaging of a Malignant Peripheral Nerve Sheath Tumor in the Renal Pelvis of a Border Collie Dog.

The American journal of surgical pathology

Pathologic Assessment of Retroperitoneal Sarcomas: A Position Paper By the Transatlantic Australasian Retroperitoneal Sarcoma Working Group.

Retroperitoneal malignant peripheral nerve sheath tumor in a patient with neurofibromatosis type 1: a case report.

BMC surgery

Research (Washington, D.C.)

Advances in Clinical Trials of Boron Neutron Capture Therapy.

Neurological sciences : official journal of the Italian Neurological Society and of the Italian Society of Clinical Neurophysiology

Misdiagnosed tuberculous myelitis turned out to be spinal malignant peripheral nerve sheath tumor: a case report.

Distinct coexisting pathologies of oral malignant peripheral nerve sheath tumor with melanin pigmentation and indolent small lymphocytic lymphoma.

Clinical cancer research : an official journal of the American Association for Cancer Research

SARC031: A Phase II Trial of Selumetinib and Sirolimus for Patients with Unresectable or Metastatic Malignant Peripheral Nerve Sheath Tumors (MPNST).

Cervical Intranodal Schwannoma and Its Malignant Transformation: A Case Report With Literature Review.

Clinical case reports

Biomedicine & pharmacotherapy = Biomedecine & pharmacotherapie

Midkine as a therapeutic node in NF1-driven neuro‑oncology: Biology, biomarkers, and translational strategies.

Journal of cardiothoracic surgery

Malignant peripheral nerve sheath tumor presenting as mitral stenosis: case report and literature review.

Malignant peripheral nerve sheath tumors in neurofibromatosis type 1 arise from distinct nodular lesions: A retrospective imaging analysis.

Neuro-oncology advances

Rapidly progressive spinal epithelioid malignant peripheral nerve sheath tumor following subtotal resection surgery: a case report.

Pathological and Immunohistochemical Features of Malignant Peripheral Nerve Sheath Tumor With Heterologous Rhabdomy Oblastic Differentiation (Malignant Triton Tumor) in the Context of the Malignant Peripheral Nerve Sheath Tumor Spectrum.

Clinical nuclear medicine

Intradural Spinal Malignant Peripheral Nerve Sheath Tumor on 18F-FDG PET/CT.

When the Rare Becomes Rarer: Metastasis of Epithelioid Malignant Peripheral Nerve Sheath Tumor (MPNST) to Lymph Node and Pleural Fluid.

Diagnostic cytopathology

Rinsho shinkeigaku = Clinical neurology

[Malignant peripheral nerve sheath tumor presenting with Pancoast syndrome in a ‍patient with neurofibromatosis type 1].

Malignant peripheral nerve sheath tumor mimicking post-traumatic hematoma: A case report.

Radiology case reports

Combined Vacuum-assisted Closure Therapy and CACIPLIQ20 for Chronic Wound Management.

Journal of medical case reports

Malignant extrarenal rhabdoid tumor: a case report.

Diagnostic challenges of the neurofibroma-malignant peripheral nerve sheath tumor spectrum in neurofibromatosis type 1: illustrative case.

Malignant Peripheral Nerve Sheath Tumor Arising After Superficial Neurofibroma Excision: A Rare Sporadic Case in a Patient Without Neurofibromatosis Type 1.

Five cases report and literature review of malignant peripheral nerve sheath tumor.

Cytological Features of Spindle Cell Lesions of the Head and Neck and Review of the Literature.

Acta cytologica

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

Rigid sacropelvic fixation for lumbosacral instability following external hemipelvectomy for malignant peripheral nerve sheath tumor using CT navigation and virtual reality imaging: a case report and literature review.

bioRxiv : the preprint server for biology

PRC2 Restricts Malignant Peripheral Nerve Sheath Tumorigenesis in a Genetically Engineered Mouse Model of MPNST.

Clinical orthopaedics and related research

Periacetabular Resection for Bone Tumors: Is There Still a Role for Massive Allograft-prosthesis Composite Reconstructions?

Survival outcomes and prognostic factors in temporal bone malignancies: A retrospective cohort of 20 patients.

Oncology letters

Zhongguo xiu fu chong jian wai ke za zhi = Zhongguo xiufu chongjian waike zazhi = Chinese journal of reparative and reconstructive surgery

[Effectiveness evaluation of three-dimensional printed customized hemi-pelvic prosthesis for pelvic reconstruction after resection of massive pelvic tumor].

Malignant peripheral nerve sheath tumors in schwannomatosis: systematic review and meta-analysis.

Journal of neurosurgery

Malignant peripheral nerve sheath tumors in schwannomatosis: a case series.

Journal of neurosurgery

Diagnostics (Basel, Switzerland)

Perineurial Malignant Peripheral Nerve Sheath Tumor of the Cauda Equina: Diagnostic Challenge.

Archivos de bronconeumologia

Pulmonary Metastasis of Malignant Peripheral Nerve Sheath Tumor with Extensive Calcification.

The journal of medical investigation : JMI

The Diagnostic Ability of Texture Analysis in MR Imaging for Neurogenic Tumors.

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

Updates in Peripheral Nerve Sheath and Adipocytic Tumors.

Case Report: Malignant peripheral nerve sheath tumor of the kidney with a novel granular cell morphology.

Case Report: Malignant peripheral nerve sheath tumor of the thoracic spine with postoperative recurrence and intracranial metastasis-a review of the literature.

Virchows Archiv : an international journal of pathology

SOX11 is frequently expressed in ETV6::NTRK3-rearranged infantile fibrosarcoma and congenital mesoblastic nephroma.

Salvage of necrotic flap following malignant peripheral nerve sheath tumor excision using multimodal pharmacotherapy: a case report.

Orbit (Amsterdam, Netherlands)

Bilateral orbital metastasis from malignant peripheral nerve sheath tumor.

Physiotherapy enhances gait stability in a forelimb-amputated dog undergoing postoperative radiotherapy: a case report.

BMC veterinary research

Journal of orthopaedic science : official journal of the Japanese Orthopaedic Association

Epidemiology and prognosis of malignant peripheral nerve sheath tumor (MPNST) in Japan: A population-based analysis using the national cancer registry.

International forum of allergy & rhinology

Sinonasal Sarcomas Management: An International Consensus Statement.

Molecular medicine (Cambridge, Mass.)

Selective inhibition of BRAF and CRAF sensitizes NF1-deficient malignant peripheral nerve sheath tumors to MEK inhibitors.

Journal of cancer research and therapeutics

Cellular schwannoma of the base of tongue: A case report.

Circumferentially Exposed Achilles Tendon Wound Reconstruction Using Free Anterolateral Thigh Flap With Vascularized Fascia Lata Wrapping.

The Intersection of a Rare Mass and Dual Primary Malignancies: A 73-Year-Old Male Patient's Cardiac Conundrum.

JACC. Case reports

Neurologia medico-chirurgica

Radiotherapy Offered Limited Additional Benefits on Cancer-specific Survival for Patients with Resectable Subcutaneous Malignant Peripheral Nerve Sheath Tumors: A Population-based Retrospective Cohort Analysis.

Malignant peripheral nerve sheath tumor of the right forearm: Case report.

Journal of experimental & clinical cancer research : CR

TRAP1 expression elicits pro-tumoral functions in macrophages associated to malignant peripheral nerve sheath tumor cells.

Malignant peripheral nerve sheath tumor of the sigmoid colon: A case report and comparison with published cases.

The Journal of veterinary medical science

Malignant peripheral nerve sheath tumor in the vagus nerve causing upper gastrointestinal signs in a dog.

Surgical Management of a Giant Mediastinal Schwannoma Associated with Neurofibromatosis Type 1: A Case Report.

Surgical case reports

Anais brasileiros de dermatologia

Recurrent metastatic malignant peripheral nerve sheath tumor associated with neurofibromatosis I: a rare case with atypical immunohistochemistry.

Frontiers in cellular neuroscience

Translocator protein deficiency blocks the ferroptosis of malignant peripheral nerve sheath tumors through glutathione peroxidase 4.

Inferior vena cava division with venous-venous bypass during en bloc lumbar spondylectomy for malignant peripheral nerve sheath tumor: illustrative case.

Regional lymph node invasion in pediatric non-rhabdomyosarcoma soft tissue sarcoma: an international cohort study from the International Soft Tissue Sarcoma Consortium.

EClinicalMedicine

Journal of cutaneous pathology

Diagnostic Utility of PRAME in Rare Melanoma Mimics: A Comparative Analysis Including GNET, MMNST, Epithelioid MPNST, and MITF-Rearranged Melanocytic Tumors.

Case reports in pathology

A Case of Myxoid Malignant Peripheral Nerve Sheath Tumor in a Patient With Carney Complex.

A Pediatric Case of Neurofibromatosis Type 1-Associated Malignant Peripheral Nerve Sheath Tumor of the Scalp.

Malignant Peripheral Nerve Sheath Tumor of the Rectum Characterized by Focal S100 Protein Expression.

Malignant Transformation of Tentorial Schwannoma.

Neurology India

Malignant peripheral nerve sheath tumor of the parotid gland.

Medicina clinica

Development of Malignant Peripheral Nerve Sheath Tumor After Keloid Treatment.

Malignant Transformation of Schwannoma to S-100 Negative Epithelioid Malignant Peripheral Nerve Sheath Tumor: A Case Report.

Malignant peripheral nerve sheath tumor in early childhood: a case report of a diagnostic challenge.

Case Report: Malignant transformation of ganglioneuroma in the rectovaginal septum to malignant peripheral nerve sheath tumor based on neurofibromas.

Prognostic factors and treatment outcomes of malignant peripheral nerve sheath tumors (MPNST) of the extremities: A tertiary cancer institutional analysis.

Journal of orthopaedics

Malignant peripheral nerve sheath tumor of the shoulder: A rare case highlighting the role of multimodal management.

Radiology case reports

Single-cell tumor microenvironment profiling informs a circulating proteome test for the interception of malignant transformation in NF1 nerve sheath tumors.

Research square

Ifosfamide, Carboplatin, and Etoposide as Neoadjuvant Chemotherapy in Patients With Neurofibromatosis Type I-related Malignant Peripheral Nerve Sheath Tumors.

In vivo (Athens, Greece)

Clinical cancer research : an official journal of the American Association for Cancer Research

MTA-Cooperative PRMT5 Inhibitors Are Efficacious in MTAP-Deleted Malignant Peripheral Nerve Sheath Tumor Models.

Malignant Triton Tumor of the Ileocolic Mesentery with Neurofibromatosis Type 1: A Case Report and Literature Review.

Surgical case reports

Journal of surgical case reports

Malignant peripheral nerve sheath tumor of the nasal cavity: a rare diagnostic and therapeutic challenge.

Malignant Peripheral Nerve Sheath Tumor in Children and Adolescents: Local Treatment in a Retrospective Single-Center Experience.

Pediatric blood & cancer

Journal of cancer research and clinical oncology

Genomic profiling and pathological assessment of malignant peripheral nerve sheath tumors.

Multidisciplinary collaborative treatment of a giant malignant peripheral nerve sheath tumor on the top of the head: a case report.

Multimodal treatment with cisplatin for metastatic disease in malignant peripheral nerve sheath tumors: a case report and review.

Anti-cancer drugs

Epithelioid Malignant Peripheral Nerve Sheath Tumor of the Breast with Breast Cancer in the Contralateral Breast: A Case Report.

Case reports in oncology

Case Report: Two cases of atypical neurofibroma neoplasm of uncertain biological potential in the nasal cavity and a literature review.

Long-Term Outcomes of Radiation Therapy for Pediatric Brain Tumors: A Single-Center Study.

Pedicled Vascularized Common Peroneal Nerve Graft in Sciatic Nerve Reconstruction With Involvement of Inner Pelvic Lumbar and Sacral Nerve Roots: A Case Report and Literature Review.

Microsurgery

Clinical and translational radiation oncology

A pattern of local failure after preoperative 5 × 5 Gy in soft tissue sarcomas: A long-term real-world experience.

Galectin-1 Inhibition as a Strategy for Malignant Peripheral Nerve Sheath Tumor Treatment.

Cells

SLAS discovery : advancing life sciences R & D

Combinatorial screen with apoptosis pathway targeted agents alrizomadlin, pelcitoclax, and dasminapant in multi-cell type tumor spheroids.

Journal of neurological surgery reports

Malignant Transformation of a Vestibular Schwannoma Without Previous Radiation Exposure: Illustrative Case and Literature Review.

Geburtshilfe und Frauenheilkunde

Malignant Peripheral Nerve Sheath Tumor (MPNST) of the Uterine Cervix - A Systematic Literature Review and New Case Report with Possible New Therapeutic Approaches.

Wiener klinische Wochenschrift

Bone transport nails for reconstruction of lower limb diaphyseal defects in patients with bone sarcomas : A case series.

CD274/PD-L1 copy number gained malignant peripheral nerve sheath tumor: A case report and literature review.

Medicine

Rapidly Growing Malignant Peripheral Nerve Sheath Tumors Arising From Neurofibromatosis Type 1: A Case Report by Rehabilitation Physicians.

International cancer conference journal

Co-occurrence of breast cancer and malignant peripheral nerve sheath tumor in a patient with neurofibromatosis type 1.

Journal of the National Comprehensive Cancer Network : JNCCN

CRE25-048: A Rare Tumor Presentation in a Postpartum Female: Case Report of Malignant Peripheral Nerve Sheath Tumor Accompanying Neurofibromatosis Type I Diagnosis.

Malignant Peripheral Nerve Sheath Tumors in Children and Adolescents: A Population-Based Study.

World neurosurgery

The CoREST complex is a therapeutic vulnerability in malignant peripheral nerve sheath tumors.

Scientific reports

Malignant peripheral nerve sheath tumor presenting in the cauda equina: diagnostic and biological pearls. Illustrative case.

Clinical cancer research : an official journal of the American Association for Cancer Research

DLK1 Distinguishes Subsets of NF1-Associated Malignant Peripheral Nerve Sheath Tumors with Divergent Molecular Signatures.

Redefining radiologic responses in high-risk soft-tissue sarcomas treated with neoadjuvant chemotherapy: final results of ISG-STS 1001, a randomized clinical trial.

ESMO open

Cerebrospinal fluid dissemination of an intracranial malignant peripheral nerve sheath tumor in the sellar region: illustrative case.

Cancer diagnosis & prognosis

Malignant Peripheral Nerve Sheath Tumor (MPNST) Arising from Orbital Plexiform Neurofibroma in a Small Child With Neurofibromatosis Type 1.

Discoidin domain receptor inhibitor DDR1-IN-1 induces autophagy and necroptotic cell death in malignant peripheral nerve sheath tumor.

Cell death discovery

Annals of surgical oncology

Neuropathic Pain Following Surgery for Malignant Peripheral Nerve Sheath Tumors.

Primary intraosseous malignant peripheral nerve sheath tumor of clavicle: Report of a rare entity.

Indian journal of cancer

Archives of pathology & laboratory medicine

Loss of H3K27me3 Is Not Specific to Malignant Triton Tumor: Immunohistochemical Analysis of 23 Cases of Embryonal Rhabdomyosarcoma.

Journal of clinical medicine

Indocyanine Green as a Marker for Tissue Ischemia in Spinal Tumor Resections and Extended Revisions: A Technical Note.

Zhonghua zhong liu za zhi [Chinese journal of oncology]

[Artemin promotes proliferation and invasion of malignant peripheral nerve sheath tumor cells through the PI3K/Akt pathway].

Primary Malignant Peripheral Nerve Sheath Tumor of the Cauda Equina: A Case Report and Literature Review.

Retroperitoneal malignant peripheral nerve sheath tumor treated with laparotomy approach: A case report.

Annals of diagnostic pathology

The diagnostic utility of SOX11 Immunohistochemical expression in malignant peripheral nerve sheath tumors and their potential mimickers.

Difficulties in the diagnosis and treatment of axillary malignant triton tumors: A case report.

Oncology letters

Chirurgie (Heidelberg, Germany)

[Plastic surgical treatment of neurofibromatosis type 1].

Knee Desmoplastic Melanoma, an Uncommon Tumor, Disguised as a Malignant Peripheral Nerve Sheath Tumor: A Case Report and Review of the Literature.

Importance of the multidisciplinary approach in the surgical treatment of giant mediastinal neoplasms: a case series.

AME case reports

UBR5 in Tumor Biology: Exploring Mechanisms of Immune Regulation and Possible Therapeutic Implications in MPNST.

Cancers

Alternative driver pathways in peripheral nerve sheath tumors - including DICER1 and/or KRAS alterations.

The Journal of pathology

Clinical cancer research : an official journal of the American Association for Cancer Research

Triple Combination of MEK, BET, and CDK Inhibitors Significantly Reduces Human Malignant Peripheral Nerve Sheath Tumors in Mouse Models.

Life (Basel, Switzerland)

Surgical Treatment and Targeted Therapy for a Large Metastatic Malignant Peripheral Nerve Sheath Tumor: A Case Report and Literature Review.

Assessing the Utility of DNA Methylation Profiling in the Classification of Myogenic Sarcomas of Bone.

Malignant Peripheral Nerve Sheath Tumor of the Small Bowel: A Case Report and Review of the Literature.

Journal of dentistry (Shiraz, Iran)

Cellular Schwannoma of the Palate Simulating as Malignant Peripheral Nerve Sheath Tumor: A Diagnostic Marathon.

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

Spinal intradural malignant peripheral nerve sheath tumor at the foramen magnum in a non-neurofibromatosis child.

Distinguishing Characteristics of Benign Versus Malignant Intraosseous Schwannomas: A Comparative Study.

Orthopaedic surgery

Trigeminal malignant peripheral nerve-sheath tumor: Systematic review, and case report treated by proton-beam therapy.

Neuro-Chirurgie

Ear, nose, & throat journal

Malignant Degeneration of a Peripheral Nerve Sheath Tumor in the Neck Region.

Brachial plexopathy and intradural cord compression caused by malignant peripheral nerve sheath tumor a case report and literature review.

Plasma DNA Methylation-Based Biomarkers for MPNST Detection in Patients With Neurofibromatosis Type 1.

Molecular carcinogenesis

Perioperative Observations and Outcome in Surgical Treatment of Malignant Peripheral Nerve Sheath Tumors.

Cancers

Dysregulation of miRNAs in Soft Tissue Sarcomas.

Cells

Laboratory investigation; a journal of technical methods and pathology

Spatial Lipidomics Reveals Myelin Defects and Protumor Macrophage Infiltration in Malignant Peripheral Nerve Sheath Tumor Adjacent Nerves.

European journal of case reports in internal medicine

Malignant Peripheral Nerve Sheath Tumour Presenting as Sciatica in a Patient with Neurofibromatosis Type 1: Is Magnetic Resonance Imaging an Effective Screening Tool?

Laboratory investigation; a journal of technical methods and pathology

Genomic Landscape of Superficial Malignant Peripheral Nerve Sheath Tumor.

Profiling the cancer-prone microenvironment in a zebrafish model for MPNST.

Oncogene

Genes, chromosomes & cancer

Malignant Peripheral Nerve Sheath Tumor (MPNST) With Smooth Muscle Differentiation of the Uterus-A Case Report With Emphasis on Diagnostic Pitfalls and Value of DNA Methylation Analysis.

Consensus recommendations for an integrated diagnostic approach to peripheral nerve sheath tumors arising in the setting of Neurofibromatosis Type 1.

Neuro-oncology

The journal of obstetrics and gynaecology research

Malignant peripheral nerve sheath tumor of the cervix in an adolescent with neurofibromatosis type 1: A case report and review of literature.

TGF-β superfamily-induced transcriptional activation pathways establish the RAD52-dependent ALT machinery during malignant transformation of MPNSTs.

Scientific reports

Malignant Triton Tumor of the Distal Femur: A Case Report and Review of the Literature.

In vivo (Athens, Greece)

Ear, nose, & throat journal

A Rare Case Report of Malignant Peripheral Nerve Sheath Tumor in the Infratemporal Fossa.

Zhongguo xiu fu chong jian wai ke za zhi = Zhongguo xiufu chongjian waike zazhi = Chinese journal of reparative and reconstructive surgery

[Expression and its clinical significance of cell-cycle dependent kinase 1 in malignant peripheral nerve sheath tumors].

Zhongguo xiu fu chong jian wai ke za zhi = Zhongguo xiufu chongjian waike zazhi = Chinese journal of reparative and reconstructive surgery

[Analysis of clinical features, treatment methods, and prognostic influence factors in patients with malignant peripheral nerve sheath tumor].

Zhongguo xiu fu chong jian wai ke za zhi = Zhongguo xiufu chongjian waike zazhi = Chinese journal of reparative and reconstructive surgery

[Clinical features and surgical treatments of neurofibromas associated with neurofibromatosis type 1].

Zhongguo xiu fu chong jian wai ke za zhi = Zhongguo xiufu chongjian waike zazhi = Chinese journal of reparative and reconstructive surgery

[Progress in neurosurgical treatment of neurofibromatosis type 1].

Current state of spinal nerve sheath tumor management and future advances.

Neuro-oncology advances

Cauda equina malignant peripheral nerve sheath tumor presenting with subarachnoid hemorrhage: a case report.

Neurocirugia

International journal of molecular sciences

Proof of Concept for Genome Profiling of the Neurofibroma/Sarcoma Sequence in Neurofibromatosis Type 1.

Seminars in musculoskeletal radiology

Inflammatory Pseudotumor of the Ulnar Nerve Mimicking a Malignant Peripheral Nerve Sheath Tumor: A Radiologic Puzzle.

Clinical cancer research : an official journal of the American Association for Cancer Research

Histology-Specific Clinical Trial of Lenvatinib and Pembrolizumab in Patients with Sarcoma.

A Rare Case of Malignant Triton Tumor Without Associated Neurofibromatosis.

Radiation-Induced Intraosseous Malignant Peripheral Nerve Sheath Tumor: A Case Report.

Malignant peripheral nerve sheath tumor mimicking carotid body tumor, a rare case report and review of literature.

Annals of medicine and surgery (2012)

Neurofibromatosis to neoplasia transition: a rare case report of spindle cell malignant peripheral nerve sheath tumor with literature review.

Vascular and endovascular surgery

Primary Aortic Malignant Peripheral Nerve Sheath Tumor.

Malignant Transformation of Previously Radiated Vestibular Schwannoma: Two-Dimensional Operative Video.

World neurosurgery

Clinical cancer research : an official journal of the American Association for Cancer Research

MEK Inhibitors Lead to PDGFR Pathway Upregulation and Sensitize Tumors to RAF Dimer Inhibitors in NF1-Deficient Malignant Peripheral Nerve Sheath Tumor.

A Case Report and Review of Diagnostic and Therapeutic Challenges of a Malignant Peripheral Nerve Sheath Tumor in the Foot.

NSD3::NUTM1 Fusion Sarcoma Mimicking Malignant Peripheral Nerve Sheath Tumor with Prolonged Survival.

Biomedicines

Journal of clinical ultrasound : JCU

Imaging findings of type I neurofibromatosis with outcome of malignant peripheral nerve sheath tumor in the right lower extremity.

The American journal of surgical pathology

Immunohistochemical Evaluation of Schlafen 11 (SLFN11) Expression in Cancer in the Search of Biomarker-Informed Treatment Targets: A Study of 127 Entities Represented by 6658 Tumors.

Contemporary surgical management of pediatric non-rhabdomyosarcoma soft tissue sarcoma.

Pediatric blood & cancer

Oman journal of ophthalmology

Orbital malignant peripheral nerve sheath tumor: A case report and review.

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

A rare Encounter with Pediatric Malignant Peripheral Nerve Sheath Tumor in Nasal Sinuses and Orbit.

Denosumab combined with chemotherapy followed by anlotinib in the treatment of multiple metastases of malignant peripheral nerve sheath tumor: a case report and literature review.

European journal of human genetics : EJHG

Late-onset tumors in rhabdoid tumor predisposition syndrome type-1 (RTPS1) and implications for surveillance.

Multiparametric whole-body MRI of patients with neurofibromatosis type I: spectrum of imaging findings.

Skeletal radiology

Malignant Transformation of Plexiform Neurofibroma Due to Neglected Giant Soft Tissue Swelling of the Back: A Case Report.

TRPS1 expression in MPNST is correlated with PRC2 inactivation and loss of H3K27me3.

Human pathology

Asia Oceania journal of nuclear medicine & biology

A rare presentation of thyroid malignant peripheral nerve sheath tumor in neurofibromatosis type 1 delineated by FDG PET/CT: A case report and literature review.

Rare primary intrapulmonary malignant peripheral nerve sheath tumor showing significant response to sintilimab: A case report and literature review.

Oncology letters

Presumed myxosarcoma in the eyelid of a cat: Case report.

Veterinary ophthalmology

Revista brasileira de ortopedia

Painless Growing Swelling on the Palm of a 4-year-old Boy: Malignant Peripheral Nerve Sheath Tumor.

Pathology, research and practice

ISLET-1 expression in soft tissue neoplasms reveals high sensitivity but moderate specificity for desmoplastic small round cell tumors and potential utility as a diagnostic biomarker.

Journal of pediatric hematology/oncology

A Pediatric Case of Intraocular Malignant Peripheral Nerve Sheath Tumor.

Zhonghua jie he he hu xi za zhi = Zhonghua jiehe he huxi zazhi = Chinese journal of tuberculosis and respiratory diseases

[A case of pleural metastasis of malignant peripheral nerve sheath tumor].

Malignant Peripheral Nerve Sheath Tumor, a Heterogeneous, Aggressive Cancer with Diverse Biomarkers and No Targeted Standard of Care: Review of the Literature and Ongoing Investigational Agents.

Targeted oncology

The Journal of veterinary medical science

Forelimb lameness caused by malignant peripheral nerve sheath tumors of the median nerve in a dog: a case report.

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

Brachial plexus peripheral nerve sheath tumors (PNSTs): clinical and surgical management in the pediatric population.

Outcomes of Head and Neck Neurogenic Tumors.

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