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1.
Radiat Oncol ; 17(1): 190, 2022 Nov 18.
Artigo em Inglês | MEDLINE | ID: mdl-36401312

RESUMO

BACKGROUND: Malignant peripheral nerve sheath tumors (MPNST) are sarcomas that arise from peripheral nerves. They generally have a poor prognosis which is exacerbated by high local recurrence rates. This case report discusses the treatment of a patient with a MPNST with local recurrence. This case report is novel due to the use of intraoperative Intrabeam™ (Zeiss, Dublin, CA) radiation therapy use in the protection of neurovascular structures such as the sciatic nerve. CASE PRESENTATION: The patient was a 65-year-old male who noticed a right posterior thigh mass slowly increasing in size over two months. A planned positive margin wide-resection excision was performed due to sciatic nerve abutment. The mass was determined to be a MPNST via postoperative pathology with positive margins along the sciatic nerve. The patient began adjuvant radiation therapy to the upper and lower thigh fields over a period of three months. Thirty-two months later, the patient was found to have a hypermetabolic mass with venous congestion and hyperemia at the prior surgical site which was confirmed by core needle biopsy to be local recurrence of the MPNST. Re-excision of the tumor was planned and performed followed by intraoperative Intrabeam™ radiation therapy. At two years of follow-up, the patient was doing well with minimal pain in his right buttock region with no new or recurrent neurological deficits. Radiologic imaging was negative for local recurrence of the MPNST. CONCLUSION: We believe this case report demonstrates a novel treatment strategy for sarcoma management. The unique use of intraoperative Intrabeam™ radiation therapy, which had not previously been used for this indication, may be efficacious in cases involving neurovascular structures. In this case, focal radiation from the intraoperative Intrabeam™ radiation device was used in a way to affect the recurrent tumor yet protect the sciatic nerve.


Assuntos
Neoplasias de Bainha Neural , Neurofibrossarcoma , Sarcoma , Humanos , Masculino , Idoso , Neurofibrossarcoma/complicações , Neurofibrossarcoma/patologia , Neoplasias de Bainha Neural/radioterapia , Neoplasias de Bainha Neural/cirurgia , Neoplasias de Bainha Neural/patologia , Nervo Isquiático/patologia , Dor , Sarcoma/radioterapia , Sarcoma/cirurgia
2.
Sci Adv ; 8(44): eabo5442, 2022 11 04.
Artigo em Inglês | MEDLINE | ID: mdl-36322658

RESUMO

Malignant peripheral nerve sheath tumor (MPNST), a highly aggressive Schwann cell (SC)-derived soft tissue sarcoma, arises from benign neurofibroma (NF); however, the identity, heterogeneity and origins of tumor populations remain elusive. Nestin+ cells have been implicated as tumor stem cells in MPNST; unexpectedly, single-cell profiling of human NF and MPNST and their animal models reveal a broad range of nestin-expressing SC lineage cells and dynamic acquisition of discrete cancer states during malignant transformation. We uncover a nestin-negative mesenchymal neural crest-like subpopulation as a previously unknown malignant stem-like state common to murine and human MPNSTs, which correlates with clinical severity. Integrative multiomics profiling further identifies unique regulatory networks and druggable targets against the malignant subpopulations in MPNST. Targeting key epithelial-mesenchymal transition and stemness regulators including ZEB1 and ALDH1A1 impedes MPNST growth. Together, our studies reveal the underlying principles of tumor cell-state evolution and their regulatory circuitries during NF-to-MPNST transformation, highlighting a hitherto unrecognized mesenchymal stem-like subpopulation in MPNST disease progression.


Assuntos
Neoplasias de Bainha Neural , Neurofibroma , Neurofibrossarcoma , Humanos , Animais , Camundongos , Neoplasias de Bainha Neural/patologia , Nestina , Transformação Celular Neoplásica/genética
3.
Am J Dermatopathol ; 44(12): 943-947, 2022 Dec 01.
Artigo em Inglês | MEDLINE | ID: mdl-36395450

RESUMO

ABSTRACT: The seminal case report of plexiform melanocytic schwannoma, published a decade ago, indicated that this is a rare variant of schwannoma demonstrating immunohistochemical expression of melanocytic markers, electron microscopic evidence of melanosome formation, and genetic features of a benign schwannoma. We report herein, a second example of this entity. Of added interest, our case showed pseudoglandular features, as previously recorded in other variants of schwannoma. A 66-year-old man presented with a cutaneous papule on the abdomen. Histopathologically, a vertically oriented, exoendophytic, folliculocentric, dermal tumor with a plexiform architecture was observed. This was composed of nodules and diverging fascicles of bland spindle-shaped cells. Notable interstitial mucin deposition conveyed a pseudoglandular appearance to the lesion. The spindled cells co-expressed S100, SOX10, and HMB45. A minority of cells expressed Melan-A and MiTF. EMA and claudin-1 stained capsular and perifascicular perineurial cells. Melanin was absent. Plexiform melanocytic schwannoma represents one of several nerve sheath tumors that peculiarly display evidence of melanocytic differentiation. These include melanocytoneuroma, pigmented neurofibroma (or melanocytic neurofibroma), and malignant melanotic schwannian tumor. Of importance, these proliferations can be mistaken for melanocytic tumors, including melanoma. In expanding the literature on this topic, we discuss steps required to distinguish plexiform melanocytic schwannoma from melanoma and other nerve sheath tumors with melanocytic differentiation. The possible pathogenesis of these unusual neoplasms is also addressed.


Assuntos
Melanoma , Neoplasias de Bainha Neural , Neurilemoma , Neurofibroma , Lesões Pré-Cancerosas , Masculino , Humanos , Idoso , Neurilemoma/patologia , Melanoma/patologia , Neoplasias de Bainha Neural/patologia , Melanócitos/patologia
4.
Medicina (Kaunas) ; 58(10)2022 Oct 06.
Artigo em Inglês | MEDLINE | ID: mdl-36295565

RESUMO

Malignant peripheral nerve sheath tumour (MPNST) is an aggressive and uncommon cancer developing in the peripheral nerve sheath. Primary cardiac MPNST is an extremely rare finding, with no specific imaging and clinical characteristics. Only a handful of cases have been reported in the literature; thus, little is still known about them. Cardiac computed tomography (CT) and cardiac magnetic resonance imaging (CMRI) are important means of assessing cardiac morphology and function. The preferred course of treatment for this pathology is by full surgical resection of the tumour, with negative (clear) margins, followed by adjuvant radiotherapy and chemotherapy. We present the case of a 42-year-old woman with no significant cardiovascular symptoms who was incidentally diagnosed during routine transthoracic echocardiography (TTE) with a cardiac mass located in the left ventricle.


Assuntos
Neoplasias de Bainha Neural , Neurofibrossarcoma , Feminino , Humanos , Adulto , Neoplasias de Bainha Neural/diagnóstico por imagem , Neoplasias de Bainha Neural/patologia , Ventrículos do Coração/diagnóstico por imagem , Ventrículos do Coração/patologia , Imageamento por Ressonância Magnética , Radioterapia Adjuvante
5.
BMJ Case Rep ; 15(10)2022 Oct 03.
Artigo em Inglês | MEDLINE | ID: mdl-36192032

RESUMO

Malignant peripheral nerve sheath tumours are rare soft tissue sarcomas commonly seen in patients with neurofibromatosis type 1. They typically manifest in the fibrous sheaths of major nerve trunks in the extremities or in an axial location. Presenting symptoms are generally non-specific, including pain and weakness, and survival is dependent on size and location of the tumour. Surgical resection is the primary treatment modality followed by radiotherapy or chemotherapy; however, prognosis is poor. Medications such as tyrosine kinase inhibitors and mitogen-activated protein kinase (MAPK)/extracellular signal-regulated kinase (ERK) pathway inhibitors are increasingly being recognised as potentially effective therapy for these malignancies. We report a case of a patient with neurofibromatosis type 1 presenting with a malignant peripheral nerve sheath tumour along the tibial nerve that was initially diagnosed as a muscle strain. We discuss the utility of diagnostic imaging and pathology in correctly identifying this aggressive tumour as well as review the drugs used in her care.


Assuntos
Segunda Neoplasia Primária , Neoplasias de Bainha Neural , Neurofibromatose 1 , Neurofibrossarcoma , MAP Quinases Reguladas por Sinal Extracelular , Feminino , Humanos , Neoplasias de Bainha Neural/cirurgia , Neurofibromatose 1/complicações , Neurofibrossarcoma/diagnóstico por imagem , Inibidores de Proteínas Quinases
6.
Oral Oncol ; 135: 106211, 2022 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-36257181

RESUMO

Malignant peripheral nerve sheath tumor (MPNST) is a spindle cell sarcoma with poor prognosis. Although patients with neurofibromatosis type 1 (NF1) have a higher risk of MPNST, it can also occur in the sporadic setting and may rarely arise centrally within bone. In this study, we present an extremely rare case of intraosseous MPNST of the maxilla arising in a 38-year-old female with no history of NF1. Despite radical surgery and postoperative radiotherapy, the patient died due to multiple distant metastases 1 year after treatment. According to the results of the literature analysis performed in this study, maxillary MPNST cases have worse clinical outcomes than general MPNSTs. In addition, it seems that NF1 and histological necrosis are poor prognostic indicators in patients with maxillary MPNST.


Assuntos
Neoplasias de Bainha Neural , Neurofibromatose 1 , Neurofibrossarcoma , Feminino , Humanos , Adulto , Neurofibrossarcoma/complicações , Neurofibrossarcoma/patologia , Neoplasias de Bainha Neural/diagnóstico , Neoplasias de Bainha Neural/cirurgia , Maxila/patologia , Neurofibromatose 1/complicações , Neurofibromatose 1/patologia , Prognóstico
7.
J Ultrasound Med ; 41(12): 3091-3101, 2022 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-36082840

RESUMO

OBJECTIVES: The objectives were to identify the key features of malignant and benign peripheral nerve sheath tumors (PNSTs) and determine a strategy for differentiating them using sonography. METHODS: Forty-six malignant peripheral nerve sheath tumors (MPNSTs) and 83 benign peripheral nerve sheath tumors (BPNSTs) confirmed by pathology from April 2010 to July 2021 were included. The general data and grayscale and color Doppler ultrasonic manifestations were compared between the two groups. We used single factor, multifactor, and area under the receiver operating characteristic (ROC) curve analyses to extract significant malignant risk factors and then established a scoring system with these factors. RESULTS: The significant variables identified in univariate analysis (P < .05) were maximum diameter, location, shape, boundary, encapsulation, echogenicity, texture pattern, calcification, entering or exiting nerve, and vascularity. Shape, boundary and vascularity were significant risk factors, and a scoring system was established. The area under the ROC curve (0.925) confirmed the usefulness of the scoring system for differentiating MPNSTs and BPNSTs. CONCLUSIONS: Ultrasonography is an effective method for differentiating MPNSTs from BPNSTs.


Assuntos
Neoplasias de Bainha Neural , Humanos , Diagnóstico Diferencial , Neoplasias de Bainha Neural/diagnóstico por imagem , Neoplasias de Bainha Neural/patologia , Ultrassonografia , Curva ROC
9.
J Craniofac Surg ; 33(6): e644-e647, 2022 Sep 01.
Artigo em Inglês | MEDLINE | ID: mdl-35968945

RESUMO

BACKGROUND: Laryngeal neurilemmoma, especially recurrent laryngeal neurilemmoma, is a rare neural sheath tumor in head and neck. The most common symptom of laryngeal neurilemmoma is hoarseness or dysphonia, followed by dysphagia, dyspnea, and foreign body sensation. At present, surgical resection is the most effective treatment for this kind of tumor, thus making how to remove it become the most concerned problem of surgeons. CASE PRESENTATION: On February 18, 2021, a 64-year-old male presented to our clinic with recurrent sore throat and intermittent hoarseness for 3 years. The results of electronic laryngoscope and magnetic resonance imaging showed a 25×10×21 mm well-defined tumor in the left pyriform sinus without laryngeal cartilage destruction and enlarged lymph nodes. After the initial diagnosis of recurrent laryngeal neurilemmoma, to preserve the continuity of recurrent laryngeal nerve as much as possible, the authors determine to perform anatomical resection of recurrent laryngeal neurilemmoma with operating microscope under the monitoring of recurrent laryngeal nerve function. Finally, the patient recovered completely from hoarseness during postoperative follow-up. CONCLUSION: A complete diagnosis and treatment process of recurrent laryngeal neurilemmoma was presented by the case. Particularly, it shows the application of recurrent laryngeal nerve monitoring in the operation helps to protect the continuity of the recurrent laryngeal nerve, which lays a anatomical bases for the follow-up nerve repair.


Assuntos
Laringe , Neoplasias de Bainha Neural , Neurilemoma , Rouquidão/etiologia , Rouquidão/cirurgia , Humanos , Laringe/patologia , Masculino , Pessoa de Meia-Idade , Neurilemoma/diagnóstico por imagem , Neurilemoma/cirurgia , Nervo Laríngeo Recorrente/patologia , Nervo Laríngeo Recorrente/cirurgia
11.
Ann Diagn Pathol ; 60: 151997, 2022 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-35777330

RESUMO

Neurofibromatosis type 1 (NF1) is a common autosomal dominant disorder. The role of angiogenesis and VEGF pathway in the pathogenesis of neurofibromas and malignant peripheral nerve sheath tumors (MPNSTs) remains poorly understood. We assessed the expression of VEGF and VEGFR family members in cohorts of plexiform neurofibromas (pNF), MPNSTs and MPNST cell lines at transcript [pNF, n = 49; MPNST, n = 34] and protein levels [pNF, n = 21; MPNST, n = 9]. VEGF and VEGFR members were variably expressed in cell lines. VEGFA (p = 3.10-5), VEGFR1 (p = 0.08), and VEGFR2 (p = 2.10-4) mRNAs were overexpressed in MPNSTs in comparison with pNFs. Both VEGFA and VEGFR1 proteins were expressed by spindle tumor cells of pNFs and MPNSTs. VEGFA was expressed more in MPNSTs than in pNFs (p = 9.10-6) and a trend for VEGFR1 overexpression was observed (p = 0.06). VEGFR2 was not found at the protein level. The microvascular density was significantly reduced in MPNSTs as compared to pNFs (p = 0.0025), with no differences regarding the expression of the activated phosphorylated forms of ERK (P-ERK [p = 0.63]) and AKT (P-AKT [p = 0.41]) in endothelial cells, suggesting that VEGF-dependant angiogenesis may not be critical for MPNST oncogenesis. Altogether, these results indicate that the VEGF-VEGFR pathway may play a role in the development of pNFs and MPNSTs, independently of angiogenesis. Whether or not it drives an oncogenic autocrine/paracrine loop in neoplastic cells, participating in an increased activation of signaling pathways downstream of tyrosine kinase receptors, including VEGFRs, is a tempting hypothesis. Nevertheless, the specific targeting of angiogenesis in MPNSTs may not be sufficient to slow down tumor growth.


Assuntos
Neoplasias de Bainha Neural , Neurofibromatose 1 , Neurofibrossarcoma , Humanos , Carcinogênese , Células Endoteliais/metabolismo , Neovascularização Patológica , Neoplasias de Bainha Neural/patologia , Neurofibromatose 1/patologia , Proteínas Proto-Oncogênicas c-akt , Receptores de Fatores de Crescimento do Endotélio Vascular , Fator A de Crescimento do Endotélio Vascular/metabolismo , Comunicação Autócrina
13.
Ophthalmic Plast Reconstr Surg ; 38(6): e180-e183, 2022.
Artigo em Inglês | MEDLINE | ID: mdl-35793663

RESUMO

Myxomas are a heterogeneous group of mesenchymal tumors. Soft tissue myxomas are divided into myocardial, intramuscular, juxta-articular, superficial, aggressive, and nerve sheath myxomas. Although benign, myxomas have site-specific biologic behavior and syndromic associations, which can influence prognosis and management. In addition, myxomas need to be distinguished from malignant neoplasms, such as myxofibrosarcomas, low-grade fibromyxoid sarcomas, myxoid liposarcomas, and peripheral nerve sheath tumors. While myxomas can occur throughout the body, these tumors arise more commonly in the thigh, shoulder, buttocks, and upper extremity, and less often in the head and neck. Rarely, myxomas can arise in a periocular location, typically in the conjunctiva and eyelid skin. In this case report, we present a patient with recurrent intramuscular myxoma of the eyelid and discuss the differential diagnosis and syndromic associations of this neoplasm. To our knowledge, there have been no prior reports of intramuscular myxoma of the eyelid and orbit.


Assuntos
Fibrossarcoma , Mixoma , Neoplasias de Bainha Neural , Adulto , Humanos , Mixoma/diagnóstico , Mixoma/cirurgia , Mixoma/patologia , Fibrossarcoma/diagnóstico , Diagnóstico Diferencial , Neoplasias de Bainha Neural/diagnóstico , Pálpebras/patologia
14.
Semin Roentgenol ; 57(3): 232-240, 2022 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-35842244

RESUMO

Tumors of the peripheral nervous system can range from benign, such as neurofibroma or schwannoma, to malignant peripheral nerve sheath tumors (MPNSTs). Magnetic resonance neurography (MRN) enables the distinction of benign peripheral nerve sheath tumors (PNSTs) from MPNSTs. In addition, MRN allows for the assessment of anatomical extent if operative management is planned and can help determine a surveillance strategy. Occasionally, tumor mimics such as traumatic neuromas can masquerade as peripheral nerve tumors. This review will illustrate the spectrum of peripheral nerve tumors and their mimics, emphasizing key distinguishing features to provide optimal MRN interpretation that enhances diagnostic thinking and therapeutic management.


Assuntos
Neoplasias de Bainha Neural , Neurilemoma , Neurofibroma , Neoplasias do Sistema Nervoso Periférico , Humanos , Imageamento por Ressonância Magnética , Neoplasias de Bainha Neural/diagnóstico por imagem , Neoplasias de Bainha Neural/patologia , Neurilemoma/diagnóstico por imagem , Neurilemoma/patologia , Neurofibroma/diagnóstico por imagem , Neoplasias do Sistema Nervoso Periférico/diagnóstico por imagem
17.
Korean J Gastroenterol ; 79(6): 265-269, 2022 Jun 25.
Artigo em Inglês | MEDLINE | ID: mdl-35746841

RESUMO

Gastric malignant peripheral nerve sheath tumors (MPNSTs) are extremely rare spindle cell sarcomas that arise within the peripheral nerves of the gastrointestinal tract. MPNST can present as a mass that may or may not be accompanied by obstruction or bleeding. Type 1 neurofibromatosis (NF) is an autosomal dominant genetic disorder with an incidence of 1 in 2,500-3,000. Plexiform neurofibromas in Type 1 NF can undergo a malignant transformation to MPNSTs. Approximately half of the incidence of MPNST is associated with the NF-1 gene. MPNST behaves aggressively, and radical excisional surgery is important for treatment. Recurrence and metastasis are significant, even after a radical excision. Despite multidisciplinary treatment, the five-year survival rate is only 30-50%. This paper reports the case of a 47-year-old man with Type 1 NF who presented with hemorrhage of a gastric subepithelial lesion. He underwent surgery under the suspicion of a gastrointestinal stromal tumor, but it was diagnosed as MPNST after confirming the histopathological appearance and immunohistochemical profiles. In addition, the large mass invaded the spleen and diaphragm. Radical surgery was performed, and additional chemotherapy was administered. This paper reports the experience of a patient with NF 1 with advanced MPNST discovered due to a subepithelial lesion.


Assuntos
Neoplasias de Bainha Neural , Neurofibromatose 1 , Neurofibrossarcoma , Humanos , Masculino , Pessoa de Meia-Idade , Neoplasias de Bainha Neural/diagnóstico , Neoplasias de Bainha Neural/cirurgia , Neurofibromatose 1/complicações , Neurofibromatose 1/diagnóstico , Neurofibrossarcoma/complicações , Taxa de Sobrevida
18.
Handb Clin Neurol ; 186: 245-255, 2022.
Artigo em Inglês | MEDLINE | ID: mdl-35772889

RESUMO

Meningiomas are the most common intradural extramedullary tumors, followed by nerve sheath tumors that can also grow extradurally. Metastases are the most frequent extradural tumors and most commonly affect the thoracic vertebrae. Spinal fractures with column dislocation and/or instability require surgical fixation. Spine surgery for an extramedullary tumor or fracture usually involves decompression of neural elements and instrumentation for stabilization. These procedures risk spinal cord and nerve root injury. The incidence of nerve root deficits after resection of nerve sheath tumors is particularly high since the tumor grows from the rootlets. Intraoperative neurophysiologic monitoring and mapping techniques have been introduced to prevent iatrogenic neurologic deficits. These include motor and sensory evoked potentials, electromyography, compound muscle action potentials, and the bulbocavernosus reflex. The combination of techniques chosen for a particular procedure depends on the surgical level and the character of the lesion.


Assuntos
Neoplasias Meníngeas , Neoplasias de Bainha Neural , Neoplasias da Medula Espinal , Potencial Evocado Motor/fisiologia , Potenciais Somatossensoriais Evocados/fisiologia , Humanos , Neoplasias de Bainha Neural/cirurgia , Estudos Retrospectivos , Medula Espinal/cirurgia , Neoplasias da Medula Espinal/cirurgia
19.
J Vet Med Sci ; 84(8): 1051-1055, 2022 Aug 01.
Artigo em Inglês | MEDLINE | ID: mdl-35732442

RESUMO

A 12-year-old mixed-breed dog presented with a 2-month history of abdominal distension. Radiographic examination, abdominal ultrasonography, and computed tomography revealed a mass in the cecum (15.0 × 11.9 × 4.5 cm). The cecal mass was surgically removed and examined histopathologically. Immunohistochemically, the neoplastic cells expressed S-100 and neuron specific enolase but not α-smooth muscle actin and CD117 (c-kit). These histologic and immunohistochemical features indicated that the mass was consistent with a malignant peripheral nerve sheath tumor (MPNST). In dogs, most MPNSTs arise from the brachial plexus, spinal nerve root, and skin of the extremities. However, gastrointestinal MPNSTs in dogs have not been described previously. To the best of our knowledge, this is the first report to describe cecal MPNST in a dog.


Assuntos
Doenças do Cão , Neoplasias de Bainha Neural , Neurofibrossarcoma , Animais , Ceco/cirurgia , Doenças do Cão/diagnóstico por imagem , Doenças do Cão/cirurgia , Cães , Neoplasias de Bainha Neural/cirurgia , Neoplasias de Bainha Neural/veterinária , Neurofibrossarcoma/veterinária , Proteínas S100
20.
Adv Ther ; 39(8): 3449-3471, 2022 08.
Artigo em Inglês | MEDLINE | ID: mdl-35689724

RESUMO

Benign peripheral non-cranial nerve sheath tumors are rare lesions, including both schwannomas and neurofibromas. These tumors arise from Schwann cells, and may originate from any peripheral, cranial, or autonomic nerve. Most of them are localized and sporadic but multifocal systemic forms can occur. Cervical sympathetic chain, brachial plexus, cervical plexus and spinal roots and nerves are the major nerve systems commonly affected. Dumbbell-shaped intra- and extradural tumors occur most commonly in the cervical spine, as well as purely extradural and paravertebral tumors. The management of these tumors has improved greatly owing to the developments in imaging techniques and surgical innovations such as endoscopically assisted approaches and robotic surgery. Microsurgical intracapsular excision of the tumor helped by the use of intraoperative fluorescent dyes and intraoperative neurophysiological monitoring minimize postoperative neural deficit, since most schwannomas are encapsulated. Most tumors can be removed with a low rate of complications and recurrence. Radiotherapy should be considered for growing lesions that are not amenable to surgery. In asymptomatic patients, observation and serial scans is an option for elderly infirm patients.


Assuntos
Neoplasias de Bainha Neural , Neurilemoma , Neurofibroma , Idoso , Humanos , Neoplasias de Bainha Neural/diagnóstico por imagem , Neoplasias de Bainha Neural/patologia , Neoplasias de Bainha Neural/cirurgia , Neurilemoma/diagnóstico por imagem , Neurilemoma/patologia , Neurilemoma/cirurgia , Neurofibroma/patologia , Neurofibroma/cirurgia , Procedimentos Neurocirúrgicos
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