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1.
Medicine (Baltimore) ; 100(40): e27520, 2021 Oct 08.
Artigo em Inglês | MEDLINE | ID: mdl-34622886

RESUMO

ABSTRACT: There is no practical predictive model for the diagnosis of gastrointestinal stromal tumors (GISTs). To establish a practical predictive model for the diagnosis of subepithelial lesions in the stomach, we reviewed patients with GISTs (n = 89), schwannomas (n = 7), and leiomyomas (n = 28).The tumor was more frequently found along the gastric cardia in the leiomyoma group (57.1%) than in the GIST/schwannoma group (2.1%, P < .01). Contrast enhancement (57.3% vs 0%, P < .01) and intra-tumoral necrosis (34.4% vs 0.0%, P < .01) were more frequently observed in the GIST/schwannoma group than in the leiomyoma group. On endoscopic ultrasonography, 58.3% of GISTs/schwannomas showed uneven echogenicity, whereas the echogenicity was uneven in 21.4% of leiomyomas (P < .01). There were no differences between the tumor color and the presence or absence of ulcer formation, tumor bleeding, irregularity of the tumor margin, cystic spaces, and hyperechoic spots between the 2 groups. Based on these results, we developed a 2-step diagnostic algorithm for GISTs/schwannomas. The first step comprises 1 endoscopic feature: a cardiac or non-cardiac location. Tumors with a cardiac location were judged as leiomyomas and those with a non-cardiac location were judged as GISTs/schwannomas, with 96.9% sensitivity and 57.1% specificity for GIST/schwannoma diagnosis. The second step comprises a combination of endoscopic (non-cardiac location), radiologic (positive contrast enhancement and intra-tumoral necrosis), and endosonographic (uneven echogenicity) features for a total of 4 points. We assigned 1 point to each feature. Tumors with scores of 2 to 4 were judged as GISTs/schwannomas, with 81.3% sensitivity and 92.9% specificity for GIST/schwannoma diagnosis.Our predictive model will be a practical guide for the management of gastric subepithelial lesions.


Assuntos
Neoplasias Gastrointestinais/patologia , Tumores do Estroma Gastrointestinal/patologia , Leiomioma/patologia , Neurilemoma/patologia , Algoritmos , Cárdia/patologia , Diagnóstico Diferencial , Endossonografia , Neoplasias Gastrointestinais/diagnóstico por imagem , Tumores do Estroma Gastrointestinal/diagnóstico por imagem , Humanos , Leiomioma/diagnóstico por imagem , Neurilemoma/diagnóstico por imagem
2.
Int J Mol Sci ; 22(15)2021 Jul 23.
Artigo em Inglês | MEDLINE | ID: mdl-34360664

RESUMO

Peripheral nerve injuries are a common condition in which a nerve is damaged, affecting more than one million people every year. There are still no efficient therapeutic treatments for these injuries. Artificial scaffolds can offer new opportunities for nerve regeneration applications; in this framework, chitosan is emerging as a promising biomaterial. Here, we set up a simple and effective method for the production of micro-structured chitosan films by solvent casting, with high fidelity in the micro-pattern reproducibility. Three types of chitosan directional micro-grooved patterns, presenting different levels of symmetricity, were developed for application in nerve regenerative medicine: gratings (GR), isosceles triangles (ISO) and scalene triangles (SCA). The directional patterns were tested with a Schwann cell line. The most asymmetric topography (SCA), although it polarized the cell shaping less efficiently, promoted higher cell proliferation and a faster cell migration, both individually and collectively, with a higher directional persistence of motion. Overall, the use of micro-structured asymmetrical directional topographies may be exploited to enhance the nerve regeneration process mediated by chitosan scaffolds.


Assuntos
Quitosana/química , Membranas/química , Regeneração Nervosa , Neurilemoma/terapia , Células de Schwann/citologia , Cicatrização , Movimento Celular , Proliferação de Células , Humanos , Neurilemoma/patologia
3.
Ann R Coll Surg Engl ; 103(10): e314-e316, 2021 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-34448654

RESUMO

We report an intracerebral schwannoma originating in the angular gyrus of a 20-year-old female that was incidentally diagnosed after she presented with a post-traumatic seizure. After comprehensive investigations, including functional magnetic resonance imaging, she underwent a computed tomography-guided stereotactic resection of the lesion. Pathological examination confirmed features of a schwannoma. After six years of follow-up, she remains well, without any evidence of recurrence. Intracerebral schwannomas are extremely uncommon: fewer than 90 cases have been reported. We present a comprehensive summary of the literature and a discussion of novel theories on the pathogenesis of intracerebral schwannomas.


Assuntos
Neoplasias Encefálicas/diagnóstico , Neurilemoma/diagnóstico , Lobo Parietal , Neoplasias Encefálicas/diagnóstico por imagem , Neoplasias Encefálicas/patologia , Neoplasias Encefálicas/cirurgia , Feminino , Humanos , Imageamento por Ressonância Magnética , Neurilemoma/diagnóstico por imagem , Neurilemoma/patologia , Neurilemoma/cirurgia , Neuroimagem , Lobo Parietal/diagnóstico por imagem , Lobo Parietal/patologia , Lobo Parietal/cirurgia , Radiocirurgia , Tomografia Computadorizada por Raios X , Adulto Jovem
4.
PLoS One ; 16(7): e0252048, 2021.
Artigo em Inglês | MEDLINE | ID: mdl-34264955

RESUMO

Neurofibromatosis Type 2 (NF2) is an autosomal dominant genetic syndrome caused by mutations in the NF2 tumor suppressor gene resulting in multiple schwannomas and meningiomas. There are no FDA approved therapies for these tumors and their relentless progression results in high rates of morbidity and mortality. Through a combination of high throughput screens, preclinical in vivo modeling, and evaluation of the kinome en masse, we identified actionable drug targets and efficacious experimental therapeutics for the treatment of NF2 related schwannomas and meningiomas. These efforts identified brigatinib (ALUNBRIG®), an FDA-approved inhibitor of multiple tyrosine kinases including ALK, to be a potent inhibitor of tumor growth in established NF2 deficient xenograft meningiomas and a genetically engineered murine model of spontaneous NF2 schwannomas. Surprisingly, neither meningioma nor schwannoma cells express ALK. Instead, we demonstrate that brigatinib inhibited multiple tyrosine kinases, including EphA2, Fer and focal adhesion kinase 1 (FAK1). These data demonstrate the power of the de novo unbiased approach for drug discovery and represents a major step forward in the advancement of therapeutics for the treatment of NF2 related malignancies.


Assuntos
Neoplasias Meníngeas/genética , Meningioma/genética , Neurilemoma/genética , Neurofibromina 2/deficiência , Neurofibromina 2/genética , Compostos Organofosforados/farmacologia , Proteínas Tirosina Quinases/antagonistas & inibidores , Pirimidinas/farmacologia , Proliferação de Células , Humanos , Mutação , Neurilemoma/patologia
5.
Neurology ; 97(7 Suppl 1): S111-S119, 2021 08 17.
Artigo em Inglês | MEDLINE | ID: mdl-34230200

RESUMO

OBJECTIVE: To assess imaging utilization practices across clinical specialists in neurofibromatosis type 1 (NF1) for the evaluation of symptomatic and asymptomatic children and adults with or without plexiform neurofibromas (PN). METHODS: An institutional review board-exempt survey was administered to medical practitioners caring for individuals with NF1 at the Response Evaluation in Neurofibromatosis and Schwannomatosis (REiNS) meeting in September 2019. The survey included questions on respondent demographic data (9 questions), type of imaging obtained for asymptomatic (4 questions) and symptomatic (4 questions) people with and without PN, and utilization of diffusion-weighted imaging (2 questions). RESULTS: Thirty practitioners participated in the survey. Most were academic neuro-oncologists at high-volume (>10 patients/week) NF1 centers. Of 30 respondents, 26 had access to whole-body MRI (WB-MRI). The most common approach to an asymptomatic person without PN was no imaging (adults: 57% [17/30]; children: 50% [15/30]), followed by a screening WB-MRI (adults: 20% [6/30]; children: 26.7% [8/30]). The most common approach to a person with symptoms or known PN was regional MRI (adults: 90% [27/30]; children: 93% [28/30]), followed by WB-MRI (adults: 20% [6/30]; children: 36.7% [11/30]). WB-MRI was most often obtained to evaluate a symptomatic child with PN (37% [11/30]). CONCLUSIONS: More than 90% of practitioners indicated they would obtain a regional MRI in a symptomatic patient without known or visible PN. Otherwise, there was little consensus on imaging practices. Given the high prevalence of PN and risk of malignant conversion in this patient population, there is a need to define imaging-based guidelines for optimal clinical care and the design of future clinical trials.


Assuntos
Neurilemoma/patologia , Neurofibroma Plexiforme/patologia , Neurofibromatoses/patologia , Neurofibromatose 1/patologia , Neoplasias Cutâneas/patologia , Adolescente , Adulto , Criança , Humanos , Imageamento por Ressonância Magnética/métodos , Masculino , Pessoa de Meia-Idade , Neurofibroma Plexiforme/diagnóstico , Neurofibromatose 1/diagnóstico , Inquéritos e Questionários , Adulto Jovem
6.
APMIS ; 129(8): 524-532, 2021 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-34050984

RESUMO

A malignant peripheral nerve sheath tumor (MPNST) arising from a schwannoma is extremely rare, with limited literature on its clinicopathologic features. Here, we present a case series and literature review on patients with MPNSTs arising from schwannomas. We performed a retrospective review of patients from our institution's records to identify those with MPNSTs arising from schwannomas. We conducted a search for additional cases from the literature utilizing PubMed. 20 patients (including 2 at our institution and 18 from 16 prior publications) were identified. The patients aged 22-93 (mean 52) years, and 63% were females. Histologically, while most MPNSTs arising from schwannomas were of epithelioid-type, 7 tumors (including 2 at our institution) were of conventional spindle-cell type. All 20 patients underwent surgical excision, while a subset received additional radiotherapy and/or chemotherapy. In 17 patients with available follow-up, the overall survival was 2-72 (median 12) months. MPNSTs rarely arise from schwannomas and should be considered in patients with a clinical diagnosis of schwannoma, however, with atypical radiologic or clinical features. MPNSTs arising from schwannomas can show epithelioid or spindle-cell histology and harbor an aggressive course, even with surgical excision and adjuvant treatment.


Assuntos
Neoplasias da Bainha Neural/complicações , Neurilemoma/complicações , Adulto , Idoso , Idoso de 80 Anos ou mais , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Neoplasias da Bainha Neural/diagnóstico por imagem , Neoplasias da Bainha Neural/mortalidade , Neoplasias da Bainha Neural/patologia , Neurilemoma/diagnóstico por imagem , Neurilemoma/mortalidade , Neurilemoma/patologia , Estudos Retrospectivos , Adulto Jovem
7.
Ann R Coll Surg Engl ; 103(7): e216-e222, 2021 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-34019433

RESUMO

Schwannomas of the sinonasal tract are rare, accounting for <4% of head and neck schwannomas. We report the case of a 61-year-old male who presented with unilateral nasal symptoms. Examination and imaging revealed a unilateral polyp at the level of the middle turbinate, with an initial biopsy suggestive of an inflammatory polyp. Due to the persistence of the patient's symptoms and his polyp despite medical therapy, endoscopic nasal polypectomy was performed. The histology surprisingly showed a schwannoma. No further interventions were carried out, and the patient remains disease-free 6 months postoperatively. A review of the literature comprising 60 cases is included. An optimal clinical approach to the investigation and management of schwannomas of the sinonasal tract is subsequently discussed.


Assuntos
Endoscopia , Neurilemoma/diagnóstico , Neoplasias dos Seios Paranasais/diagnóstico , Seios Paranasais/patologia , Biópsia , Diagnóstico Diferencial , Humanos , Masculino , Pessoa de Meia-Idade , Neurilemoma/patologia , Neurilemoma/cirurgia , Neoplasias dos Seios Paranasais/cirurgia , Seios Paranasais/diagnóstico por imagem , Seios Paranasais/cirurgia , Pólipos/diagnóstico , Pólipos/imunologia , Tomografia Computadorizada por Raios X , Resultado do Tratamento
8.
Laryngoscope ; 131(12): 2729-2731, 2021 12.
Artigo em Inglês | MEDLINE | ID: mdl-34014564

RESUMO

OBJECTIVES/HYPOTHESIS: To report the first case of transoral excision of a paraglottic laryngeal schwannoma. METHODS: Case report. RESULTS: Laryngeal schwannomas are rare tumors, with approximately 75 cases reported to date worldwide. Most cases involve superficial supraglottic tumors excised transorally, and some have been found in deeper compartments and treated using transcervical approaches. This report describes the first excision of a schwannoma of the paraglottic space via endoscopic laser laryngotomy. The procedure allowed return to normal function, without recurrence within 8 months, after which the patient was lost to follow-up. CONCLUSIONS: Transoral excision of benign paraglottic tumors is safe and feasible. LEVEL OF EVIDENCE: NA Laryngoscope, 131:2729-2731, 2021.


Assuntos
Neoplasias Laríngeas/cirurgia , Cirurgia Endoscópica por Orifício Natural/métodos , Neurilemoma/cirurgia , Epiglote/patologia , Epiglote/cirurgia , Humanos , Neoplasias Laríngeas/diagnóstico , Neoplasias Laríngeas/patologia , Masculino , Pessoa de Meia-Idade , Neurilemoma/diagnóstico , Neurilemoma/patologia , Resultado do Tratamento
9.
Indian J Pathol Microbiol ; 64(2): 379-381, 2021.
Artigo em Inglês | MEDLINE | ID: mdl-33851640

RESUMO

WHO classification of adrenal tumors. Only a handful of cases have been reported so far. A 30-year-old lady presented with cerebrovascular accident. CT scans of the abdomen and pelvis revealed a 3.5-cm well-defined, smooth margined, heterogeneously enhancing, mass lesion in the right adrenal gland. She had no endocrine symptoms and urinary metanephnines were normal. She underwent right adrenalectomy for incidentaloma. Histopathology of the excised mass showed features of an adrenal schwannoma. Diagnosis of adrenal schwannoma on imaging studies is difficult preoperatively and raises suspicion of other adrenal tumors. Surgical excision followed by histopathology confirms the diagnosis.


Assuntos
Neoplasias das Glândulas Suprarrenais/diagnóstico , Neoplasias das Glândulas Suprarrenais/cirurgia , Glândulas Suprarrenais/patologia , Neurilemoma/diagnóstico , Neurilemoma/patologia , Neoplasias das Glândulas Suprarrenais/patologia , Glândulas Suprarrenais/cirurgia , Adulto , Feminino , Humanos , Achados Incidentais , Neurilemoma/cirurgia , Acidente Vascular Cerebral/patologia , Tomografia Computadorizada por Raios X
10.
Sci Rep ; 11(1): 8608, 2021 04 21.
Artigo em Inglês | MEDLINE | ID: mdl-33883565

RESUMO

In vestibular schwannoma (VS) patients hearing outcome and surgery related risks can vary and depend on the nerve of origin. Preoperative origin differentiation between inferior or superior vestibular nerve may influence the decision on treatment, and the selection of optimal treatment and counselling modalities. A novel scoring system based on functional tests was designed to predict the nerve of origin for VS and was applied to a large number of consecutive patients. A prospective, double blind, cohort study including 93 patients with suspected unilateral VS was conducted at a tertiary referral centre. Preoperatively before tumor resection a functional test battery [video head-impulse test (vHIT) of all semicircular canals (SCC)], air-conducted cervical/ocular vestibular evoked myogenic potential tests (cVEMP, oVEMP), pure-tone audiometry, and speech discrimination was applied. Sensitivity and specificity of prediction of intraoperative finding by a preoperative score based on vHIT gain, cVEMP and oVEMP amplitudes and asymmetry ratios was calculated. For the prediction of inferior vestibular nerve origin, sensitivity was 73% and specificity was 80%. For the prediction of superior vestibular nerve origin, sensitivity was 60% and specificity was 90%. Based on the trade-off between sensitivity and specificity, optimized cut-off values of - 0.32 for cVEMP and - 0.11 for oVEMP asymmetry ratios and vHIT gain thresholds of 0.77 (anterior SCC), 0.84 (lateral SCC) and 0.80 (posterior SCC) were identified by receiver operator characteristic curves. The scoring system based on preoperative functional tests improves prediction of nerve of origin and can be applied in clinical routine.


Assuntos
Neurilemoma/patologia , Nervo Vestibular/patologia , Adolescente , Adulto , Idoso , Audiometria de Tons Puros/métodos , Método Duplo-Cego , Feminino , Teste do Impulso da Cabeça/métodos , Audição/fisiologia , Humanos , Masculino , Pessoa de Meia-Idade , Neuroma Acústico/patologia , Estudos Prospectivos , Canais Semicirculares/patologia , Potenciais Evocados Miogênicos Vestibulares/fisiologia , Adulto Jovem
11.
Pan Afr Med J ; 38: 128, 2021.
Artigo em Francês | MEDLINE | ID: mdl-33912298

RESUMO

Intraspinal tumors are uncommon. Positive diagnosis is based on medical imaging exams, in particular MRI. Anatomopathological examination allows for definitive diagnosis. Surgery is the treatment of choice for most of them. Prognosis is related to the histological type and patient´s initial clinical condition. We here report the histological profile and progression of intraspinal tumors in our department. We conducted a retrospective study over a period of 10 years. All operated patients with histology-confirmed intraspinal tumor (23 cases) were enrolled. Four unworkable records were excluded. The median time from symptom onset and first consultation was 79 days. Patients presented with spinal cord compression. In 11 cases this was characterized by slow onset. Seven CT scan and 14 MRI were performed, which showed 4 intramedullary tumors, 9 intradural, 1 extradural, and 5 of unknown site. Histological examination showed meningioma in 11 cases, neurinoma in 3 cases. In 7 cases, it confirmed the diagnosis based on Imaging tests (4 CT and 3 MRI). Macroscopically complete resection was performed in 14 cases; it was partial in 5 cases. After a 6-month follow-up period 6 patients had fully recovered, 9 partially. This study highlights diagnosis delays. MRI better defined the lesion, but its histological approach was limited. Meningiomas dominated. Complete resection was most often performed. The postoperative course was uneventful.


Assuntos
Neoplasias Meníngeas/patologia , Meningioma/patologia , Neurilemoma/patologia , Neoplasias da Coluna Vertebral/patologia , Adolescente , Adulto , Idoso , Criança , Progressão da Doença , Feminino , Humanos , Imageamento por Ressonância Magnética , Masculino , Neoplasias Meníngeas/diagnóstico por imagem , Neoplasias Meníngeas/cirurgia , Meningioma/diagnóstico por imagem , Meningioma/cirurgia , Pessoa de Meia-Idade , Neurilemoma/diagnóstico por imagem , Neurilemoma/cirurgia , Estudos Retrospectivos , Compressão da Medula Espinal/etiologia , Neoplasias da Coluna Vertebral/diagnóstico por imagem , Neoplasias da Coluna Vertebral/cirurgia , Tomografia Computadorizada por Raios X , Adulto Jovem
12.
Medicine (Baltimore) ; 100(16): e25517, 2021 Apr 23.
Artigo em Inglês | MEDLINE | ID: mdl-33879689

RESUMO

RATIONALE: Primary schwannoma of the thyroid gland is very rare, and its preoperative diagnosis is difficult. PATIENT CONCERNS: We report the case of a thyroid nodule in an 18-year-old woman, who presented with a mass in her left neck with stiffness and normal thyroid function. However, the patient complained of numbness in her left upper extremity, and ultrasound (US) features were suspicious of malignancy. DIAGNOSES: Multimodal US imaging was performed using B-mode, color doppler, ultrasonic elastography (UE), and contrast-enhanced ultrasound (CEUS). CEUS revealed heterogeneous enhancement and "target sign" within the tumor. The nodule was suspicious for malignancy and classified as TI-RADS 4b, while the elasticity values measured by UE indicated a benign lesion. Fine needle aspiration (FNA) was subsequently performed in the markedly contrast-enhanced area for biopsy. Cytological results revealed a benign schwannoma. INTERVENTION: The patient underwent left lobe resection. Postoperative pathology confirmed it to be a primary benign schwannoma of the thyroid. OUTCOMES: After thyroidectomy, the patient was followed-up with US. At present, all laboratory tests and thyroid imaging are normal, and the numbness of the left upper limb has disappeared. LESSONS: The combination of different US modalities is useful for the diagnosis of thyroid lesions. FNA performed under CEUS guidance improves the accuracy of biopsy sampling.


Assuntos
Técnicas de Imagem por Elasticidade/métodos , Neurilemoma/diagnóstico , Glândula Tireoide/diagnóstico por imagem , Neoplasias da Glândula Tireoide/diagnóstico , Ultrassonografia Doppler em Cores/métodos , Adolescente , Biópsia por Agulha Fina , Meios de Contraste/administração & dosagem , Feminino , Humanos , Imagem Multimodal/métodos , Neurilemoma/patologia , Neurilemoma/cirurgia , Glândula Tireoide/patologia , Glândula Tireoide/cirurgia , Neoplasias da Glândula Tireoide/patologia , Neoplasias da Glândula Tireoide/cirurgia , Tireoidectomia , Resultado do Tratamento
13.
J Surg Oncol ; 124(1): 33-40, 2021 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-33831232

RESUMO

INTRODUCTION: Advances in the care of soft-tissue tumors, including imaging capabilities and adjuvant radiation therapy, have broadened the indications and opportunities to pursue surgical limb salvage. However, peripheral nerve involvement and femoral nerve resection can still result in devastating functional outcomes. Nerve transfers offer a versatile solution to restore nerve function following tumor resection. METHODS: Two cases were identified by retrospective review. Patient and disease characteristics were gathered. Preoperative and postoperative motor function were assessed using the Medical Research Council Muscle Scale. Patient-reported pain levels were assessed using the numeric rating scale. RESULTS: Nerve transfers from the obturator and sciatic nerve were employed to restore knee extension. Follow up for Case 1 was 24 months, 8 months for Case 2. In both patients, knee extension and stabilization of gait without bracing was restored. Patient also demonstrated 0/10 pain (an average improvement of 5 points) with decreased neuromodulator and pain medication use. CONCLUSION: Nerve transfers can restore function and provide pain control benefits and ideally are performed at the time of tumor extirpation. This collaboration between oncologic and nerve surgeons will ultimately result in improved functional recovery and patient outcomes.


Assuntos
Nervo Femoral/lesões , Lipossarcoma/cirurgia , Transferência de Nervo/métodos , Neurilemoma/cirurgia , Traumatismos dos Nervos Periféricos/cirurgia , Neoplasias de Tecidos Moles/cirurgia , Adulto , Idoso , Feminino , Humanos , Lipossarcoma/patologia , Masculino , Neurilemoma/patologia , Traumatismos dos Nervos Periféricos/etiologia , Traumatismos dos Nervos Periféricos/patologia , Estudos Retrospectivos , Neoplasias de Tecidos Moles/patologia
14.
Ann R Coll Surg Engl ; 103(4): e124-e126, 2021 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-33682474

RESUMO

This case report discusses a cervical psammomatous melanotic schwannoma - a rare form of peripheral nerve sheath tumour - which may be highly vascular and is often associated with the Carney complex. Significant intraneural bleeding, which was encountered intraoperatively, was controlled successfully with a gelatine-based thrombin haemostatic agent (Floseal®, Baxter International, Deerfield, IL, USA) without complication.


Assuntos
Neoplasias de Cabeça e Pescoço/diagnóstico , Neurilemoma/diagnóstico , Perda Sanguínea Cirúrgica/prevenção & controle , Feminino , Esponja de Gelatina Absorvível/uso terapêutico , Neoplasias de Cabeça e Pescoço/patologia , Neoplasias de Cabeça e Pescoço/cirurgia , Hemostasia Cirúrgica/métodos , Humanos , Pessoa de Meia-Idade , Neurilemoma/patologia , Neurilemoma/cirurgia
16.
Medicine (Baltimore) ; 100(8): e24803, 2021 Feb 26.
Artigo em Inglês | MEDLINE | ID: mdl-33663098

RESUMO

RATIONALE: Melanotic schwannoma (MS) is an unusual variant of a nerve sheath neoplasm that accounts for less than 1% of all primary peripheral nerve sheath tumors. Fluorine-18 fluorodeoxyglucose positron emission tomography/computed tomography (18F-FDG PET/CT) has unique value in detecting malignant MS lesions. To date, only 4 cases of MS with hepatic metastasis have been reported. Herein, we report the fifth case, which is the first reported patient with MS of Asian ethnicity with hepatic metastasis. PATIENT CONCERNS: A 29-year-old woman with a 1-day history of backache was admitted to our hospital. PET/CT showed a paravertebral heterogeneous soft tissue mass along the spinal nerve at the L2-L3 level with strong FDG uptake, and a nodule with increased FDG uptake in the lateral lobe of the left liver. DIAGNOSIS: A puncture biopsy of the L3 bony destruction and surrounding soft tissue mass was performed. The final diagnosis was spinal MS with hepatic metastasis. INTERVENTIONS: The patient underwent 6 courses of systemic chemotherapy. OUTCOMES: The patient did not receive further treatment for half a year after the end of chemotherapy and recovered well. LESSONS: Unlike conventional schwannomas, which are completely benign, MS has an unpredictable prognosis. It is thought to have low malignant potential, and the malignant type tends to metastasize. FDG PET/CT has a unique and important value in the differential diagnosis of benign and malignant lesions, in detecting occult metastases, monitoring the treatment response, and assessing the prognosis of MS.


Assuntos
Fluordesoxiglucose F18/administração & dosagem , Neurilemoma/diagnóstico , Tomografia por Emissão de Pósitrons combinada à Tomografia Computadorizada/métodos , Compostos Radiofarmacêuticos/administração & dosagem , Neoplasias da Coluna Vertebral/diagnóstico , Adulto , Antineoplásicos/uso terapêutico , Feminino , Humanos , Neoplasias Hepáticas/secundário , Vértebras Lombares/fisiologia , Neurilemoma/diagnóstico por imagem , Neurilemoma/tratamento farmacológico , Neurilemoma/patologia , Neoplasias da Coluna Vertebral/diagnóstico por imagem , Neoplasias da Coluna Vertebral/tratamento farmacológico , Neoplasias da Coluna Vertebral/patologia , Raízes Nervosas Espinhais/patologia
17.
World Neurosurg ; 149: e766-e779, 2021 05.
Artigo em Inglês | MEDLINE | ID: mdl-33540091

RESUMO

BACKGROUND: Surveillance imaging is a valid management option for selected vestibular schwannomas (VS). An ideal protocol for radiologic monitoring would highlight growth-related risk factors and tailor management accordingly. This study aims to identify variables associated with the growth of sporadic VS to enhance surveillance imaging, enable early intervention, and optimize outcomes. METHODS: The review was conducted according to the PRISMA guidelines. A systematic review of 5 databases (PubMed, Ovid, Cochrane Library, Web of Science, and Google Scholar) was performed to identify negative and positive growth predictors of sporadic vestibular schwannomas. The search was limited to studies reported between January 2015 and January 2020. We conducted an individual patient data meta-analysis using a 1-stage multivariate mixed-effect logistic regression model. RESULTS: A total of 437 studies were identified, of which 25 met our criteria for full-text analysis. Articles that measured VS with comparable methods were determined eligible for meta-analysis inclusion. The selected articles were highly heterogeneous in their use of grading scales and assessment of tumor size. Our review showed that size at diagnosis (odds ratio, 1.15; 95% confidence interval, 1.11-1.18; P < 0.0001) and intracanalicular localization (odds ratio, 0.49; 95% confidence interval, 0.26-0.90; P = 0.023) were associated with VS growth. CONCLUSIONS: The factors most frequently reported as being associated with growth within the literature were size of VS at diagnosis and localization of an intracanalicular component. Greater attention should be placed on these criteria within the surveillance imaging algorithm for VS.


Assuntos
Neurilemoma/patologia , Neurilemoma/cirurgia , Neuroma Acústico/patologia , Neuroma Acústico/cirurgia , Idoso , Idoso de 80 Anos ou mais , Feminino , Humanos , Masculino , Pacientes , Qualidade de Vida , Radiocirurgia/métodos
18.
Medicine (Baltimore) ; 100(7): e24801, 2021 Feb 19.
Artigo em Inglês | MEDLINE | ID: mdl-33607842

RESUMO

RATIONALE: Orbital schwannoma is a relatively rare orbital tumor, and calcification of the lesion is rarely found in the orbit. We report a case of orbital schwannoma which was characterized by calcification in the orbital muscle cone, and was cured by intracapsular excision. PATIENT CONCERNS: A 54-year-old female with a complaint of a mass in the left orbit during a magnetic resonance imaging examination and symptom of dizziness 6 months before, presented with painless exophthalmos and vision decline in the left eye. DIAGNOSES: According to clinical manifestations, imaging examinations and postoperative immunohistochemical examinations, the diagnosis was orbital schwannoma, with calcification in the muscle cone. INTERVENTIONS: The patient was treated by intracapsular excision of the left orbit. We removed the intracapsular mass and most part of the cyst wall in order to prevent orbital apex syndrome. OUTCOMES: The diagnosis of schwannoma with calcification was confirmed finally through histological and immunohistochemical exam. The patient was followed up for 28 months and the orbital CT scan showed that there were no significant lesions found in the orbital muscle cone. LESSONS: Understanding clinical, imaging diagnostic, and histopathological features of rare orbital schwannoma with calcification will facilitate timely diagnosis and treatment of this condition. The intracapsular excision can help in avoiding complications.


Assuntos
Calcinose/cirurgia , Neurilemoma/cirurgia , Neoplasias Orbitárias/cirurgia , Calcinose/complicações , Calcinose/diagnóstico por imagem , Calcinose/patologia , Feminino , Humanos , Imageamento por Ressonância Magnética , Pessoa de Meia-Idade , Neurilemoma/complicações , Neurilemoma/diagnóstico por imagem , Neurilemoma/patologia , Músculos Oculomotores/diagnóstico por imagem , Músculos Oculomotores/patologia , Neoplasias Orbitárias/complicações , Neoplasias Orbitárias/diagnóstico por imagem , Neoplasias Orbitárias/patologia
19.
Cancer Imaging ; 21(1): 23, 2021 Feb 15.
Artigo em Inglês | MEDLINE | ID: mdl-33588954

RESUMO

BACKGROUND: There is a paucity of existing literature centering on the magnetic resonance (MR) imaging features of pancreatic schwannomas, due to the neoplasm's nonspecific presentation and its rarity. We aimed to identify the characteristic imaging features of pancreatic schwannoma. METHODS: This retrospective search was conducted for histologically confirmed pancreatic schwannoma in multi-institutional database of pathology. Abdominal magnetic resonance imaging (MRI) was performed before histologic examination and their MR imaging studies were independently reviewed. The search yielded six adults (mean age, 46 years) with a definitive histologic postoperative diagnosis of single pancreatic schwannoma each. Additionally, a comprehensive English and Chinese literature review for pancreatic schwannoma and reported MR-imaging findings since 1961 was also conducted. MR imaging features of those cases in the literature were analyzed, summarized and compared with our case series. RESULTS: This rare entity appeared to be a well-circumscribed, exophytic, oval or round pancreatic mass with a mean greatest diameter of 3.7 cm. Five schwannomas were located in the pancreatic head-neck and one in the pancreatic tail. On MRI, all cases appeared hypointense on T1-weighted images, inhomogeneous hyperintense on T2-weighted images, and hyperintense on diffusion-weighted images. The mean apparent diffusion coefficient (ADC) values of pancreatic schwannoma were 1.11 ± 0.29 × 10- 3 mm2/s and significantly lower than the surrounding pancreas. The lesion-to-pancreas signal intensity ratio (SIR) at unenhanced T1-weighted images was 0.53 ± 0.07. On dynamic contrast-enhanced MRI, most of the lesions (67%, 4/6) showed homogeneously iso- or hypointense on arterial and portal venous phases, and hyperenhancement on delayed phase compared with the surrounding pancreas. In our analysis of the time intensity curves, all cases exhibited a gradual enhancement pattern. CONCLUSIONS: A well-circumscribed mass displaying inhomogeneous hyperintensity on T2, marked hypointensity on T1, hyperintensity on DWI, and with early slight enhancement at arterial phase and progressive enhancement at portal venous and delayed phase, may suggest the diagnosis of pancreatic schwannoma.


Assuntos
Imageamento por Ressonância Magnética/métodos , Neurilemoma/diagnóstico por imagem , China , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Neurilemoma/patologia , Estudos Retrospectivos
20.
Neurosurgery ; 88(3): 443-456, 2021 02 16.
Artigo em Inglês | MEDLINE | ID: mdl-33588442

RESUMO

Neoplasms of the peripheral nervous system represent a heterogenous group with a wide spectrum of morphological features and biological potential. They range from benign and curable by complete excision (schwannoma and soft tissue perineurioma) to benign but potentially aggressive at the local level (plexiform neurofibroma) to the highly malignant (malignant peripheral nerve sheath tumors [MPNST]). In this review, we discuss the diagnostic and pathologic features of common peripheral nerve sheath tumors, particularly those that may be encountered in the intracranial compartment or in the spine and paraspinal region. The discussion will cover schwannoma, neurofibroma, atypical neurofibromatous neoplasms of uncertain biological potential, intraneural and soft tissue perineurioma, hybrid nerve sheath tumors, MPNST, and the recently renamed enigmatic tumor, malignant melanotic nerve sheath tumor, formerly referred to as melanotic schwannoma. We also discuss the diagnostic relevance of these neoplasms to specific genetic and familial syndromes of nerve, including neurofibromatosis 1, neurofibromatosis 2, and schwannomatosis. In addition, we discuss updates in our understanding of the molecular alterations that represent key drivers of these neoplasms, including neurofibromatosis type 1 and type 2, SMARCB1, LZTR1, and PRKAR1A loss, as well as the acquisition of CDKN2A/B mutations and alterations in the polycomb repressor complex members (SUZ12 and EED) in the malignant progression to MPNST. In summary, this review covers practical aspects of pathologic diagnosis with updates relevant to neurosurgical practice.


Assuntos
Nervos Periféricos/diagnóstico por imagem , Neoplasias do Sistema Nervoso Periférico/diagnóstico por imagem , Neoplasias do Sistema Nervoso Periférico/genética , Animais , Humanos , Neoplasias da Bainha Neural/diagnóstico por imagem , Neoplasias da Bainha Neural/genética , Neoplasias da Bainha Neural/patologia , Neurilemoma/diagnóstico por imagem , Neurilemoma/genética , Neurilemoma/patologia , Neurofibroma/diagnóstico por imagem , Neurofibroma/genética , Neurofibroma/patologia , Neurofibromatoses/diagnóstico por imagem , Neurofibromatoses/genética , Neurofibromatoses/patologia , Nervos Periféricos/patologia , Neoplasias do Sistema Nervoso Periférico/patologia , Neoplasias Cutâneas/diagnóstico por imagem , Neoplasias Cutâneas/genética , Neoplasias Cutâneas/patologia , Fatores de Transcrição/genética
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