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1.
Clin Nucl Med ; 44(12): 991-992, 2019 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-31689283

RESUMO

A 48-year-old woman with intermittent lower back pain for 9 months and known retroperitoneal neurofibroma underwent F-NaF PET/CT scan to assess possible bony lesions causing the pain. Incidentally, the images showed elevated NaF activity in the retroperitoneal neurofibroma. In addition, uterine leiomyoma with heterogeneous calcifications revealed increased NaF activity.


Assuntos
Calcinose/complicações , Radioisótopos de Flúor , Leiomioma/complicações , Neurofibroma/metabolismo , Neoplasias Retroperitoneais/metabolismo , Fluoreto de Sódio/metabolismo , Transporte Biológico , Humanos , Masculino , Pessoa de Meia-Idade , Neurofibroma/complicações , Neurofibroma/diagnóstico por imagem , Tomografia Computadorizada com Tomografia por Emissão de Pósitrons , Neoplasias Retroperitoneais/complicações , Neoplasias Retroperitoneais/diagnóstico por imagem
2.
Genes (Basel) ; 10(11)2019 11 05.
Artigo em Inglês | MEDLINE | ID: mdl-31694342

RESUMO

Non-coding RNAs (ncRNAs) are known to regulate gene expression at the transcriptional and post-transcriptional levels, chromatin remodeling, and signal transduction. The identification of different species of ncRNAs, microRNAs (miRNAs), circular RNAs (circRNAs), and long ncRNAs (lncRNAs)-and in some cases, their combined regulatory function on specific target genes-may help to elucidate their role in biological processes. NcRNAs' deregulation has an impact on the impairment of physiological programs, driving cells in cancer development. We here carried out a review of literature concerning the implication of ncRNAs on tumor development in neurofibromatosis type 1 (NF1), an inherited tumor predisposition syndrome. A number of miRNAs and a lncRNA has been implicated in NF1-associated tumors, such as malignant peripheral nerve sheath tumors (MPNSTs) and astrocytoma, as well as in the pathognomonic neurofibromas. Some authors reported that the lncRNA ANRIL was deregulated in the blood of NF1 patients with plexiform neurofibromas (PNFs), even if its role should be further elucidated. We here provided original data concerning the association of a specific genotype about ANRIL rs2151280 with the presence of optic gliomas and a mild expression of the NF1 phenotype. We also detected the LOH of ANRIL in different tumors from NF1 patients, supporting the involvement of ANRIL in some NF1-associated tumors. Our results suggest that ANRIL rs2151280 may be a potential diagnostic and prognostic marker, addressing early diagnosis of optic glioma and predicting the phenotype severity in NF1 patients.


Assuntos
Neurofibromatose 1/genética , Glioma do Nervo Óptico/genética , RNA Longo não Codificante/genética , Astrocitoma/complicações , Genes da Neurofibromatose 1 , Genótipo , Humanos , Perda de Heterozigosidade , MicroRNAs/genética , Neoplasias da Bainha Neural/complicações , Neurofibroma/complicações , Neurofibroma Plexiforme/complicações , Neurofibromatose 1/complicações , Glioma do Nervo Óptico/complicações , Glioma do Nervo Óptico/metabolismo , Fenótipo , RNA Longo não Codificante/metabolismo , RNA não Traduzido/genética , Transdução de Sinais/genética
3.
S Afr J Surg ; 57(3): 58, 2019 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-31392870

RESUMO

BACKGROUND: Intradural extramedullary (IDEM) spinal masses are common lesions with varying histological diagnoses often associated with significant neurological deficits. This study aimed to describe the epidemiology, management and perioperative outcome of IDEM tumours seen at the teaching hospitals of the University of the Witwatersrand, Johannesburg, between 2014 and 2017. RESULTS: 92 patients were included in the study. The ages ranged from 21 to 87 years, sex ratio was M:F 1:1.4, and duration of symptoms prior to diagnosis ranged between 3 days to 18 months. Local and radicular type pain as well as motor weakness were the commonest symptoms. 67% had severe neurological deficit McCormick Grade III and IV. Schwannoma (26) Neurofibromas (21) and Meningiomas (16) and were the most frequent tumour types. Meningiomas predominantly occurred at the cranio-cervical and thoracic levels. Nerve sheath tumours were mostly found at the cervical and lumbar levels while filum terminale ependymomas occurred at the thoracolumbar area. Laminectomy was the commonest surgical approach employed, and the extent of resection varied, with total excision in half the cases. Neurological function was regained in 3 patients, deteriorated in two and was unchanged in the remainder. CONCLUSION: IDEM tumours are an important subset of spinal cord compressive lesions Presentation with severe neurological deficit is common and though resection is feasible neurological deficit remains in the vast majority. Earlier detection should improve the results of surgery.


Assuntos
Meningioma/cirurgia , Neurilemoma/cirurgia , Neurofibroma/cirurgia , Neoplasias da Medula Espinal/cirurgia , Adulto , Idoso , Idoso de 80 Anos ou mais , Dor nas Costas/etiologia , Feminino , Hospitais de Ensino , Humanos , Laminectomia , Masculino , Meningioma/complicações , Meningioma/epidemiologia , Pessoa de Meia-Idade , Debilidade Muscular/etiologia , Neurilemoma/complicações , Neurilemoma/epidemiologia , Neurofibroma/complicações , Neurofibroma/epidemiologia , Radiculopatia/etiologia , África do Sul/epidemiologia , Compressão da Medula Espinal/etiologia , Neoplasias da Medula Espinal/complicações , Neoplasias da Medula Espinal/epidemiologia , Adulto Jovem
4.
World Neurosurg ; 130: 1-6, 2019 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-31254713

RESUMO

BACKGROUND: Cases of multiple intracranial tumors are common; however, cases of multiple intraspinal tumors are rare. Except for cases of neurofibromatosis, it is very rare for tumors of different pathological types to exist concurrently at the same spinal level. Only 9 cases have been reported to date, with meningioma found with schwannoma in 7 cases and with neurofibroma in 2 cases. CASE DESCRIPTION: We have reported another rare case in which neurofibroma and meningioma were identified within a single dumbbell-shaped tumor at the same cervical level without neurofibromatosis. The preoperative magnetic resonance imaging findings indicated a single extra- and intradural extramedullary dumbbell-shaped neurogenic tumor on the left ventral side of the cervical spine. Intraoperatively, we found that the mass consisted of 2 pathologically different tumors. The results of surgical resection were mostly satisfactory. CONCLUSIONS: To the best of our knowledge, the present case is the first reported case of intradural neurofibroma (not meningioma) and extradural meningioma growing mixed together at the same spinal level without neurofibromatosis. The precise mechanism underlying the formation of the tumor is unknown, and multidirectional differentiation of a common progenitor cell is one possibility. Intra- and extradural exploration and component biopsies are useful for treatment planning, especially when the magnetic resonance imaging is not sufficiently sensitive for the diagnosis of coexisting tumor types.


Assuntos
Vértebras Cervicais/diagnóstico por imagem , Neoplasias Meníngeas/diagnóstico por imagem , Meningioma/diagnóstico por imagem , Neurofibroma/diagnóstico por imagem , Neurofibromatoses , Neoplasias da Coluna Vertebral/diagnóstico por imagem , Vértebras Cervicais/cirurgia , Feminino , Humanos , Neoplasias Meníngeas/complicações , Neoplasias Meníngeas/cirurgia , Meningioma/complicações , Meningioma/cirurgia , Pessoa de Meia-Idade , Neurofibroma/complicações , Neurofibroma/cirurgia , Neoplasias da Coluna Vertebral/complicações , Neoplasias da Coluna Vertebral/cirurgia
5.
J Neurosurg Spine ; 30(1): 126-132, 2018 10 19.
Artigo em Inglês | MEDLINE | ID: mdl-30485203

RESUMO

OBJECTIVEC2 nerve root neurofibromas have been reported frequently in patients with neurofibromatosis type 1 (NF1), although their genetic and imaging characteristics are unexplored. The aim of this study was to characterize genetic and spinal imaging findings in a large cohort of NF1 patients with C2 neurofibromas.METHODSThe authors performed a review of national NF1 referrals between 2009 and 2016. Inclusion criteria were at least 1 C2 root neurofibroma and cervical-spine or whole-spine MRI scans available for analysis. Blinded imaging review was performed by a neuroradiologist with an interest in NF1.RESULTSFifty-four patients with 106 C2 neurofibromas were included. The median age was 32.5 years (range 15-61 years), and there were slightly more male patients (33 vs 21 female patients). Splice-site (30%) and missense (20%) variants were frequent. Spinal neurofibromas were distributed in all spine regions (65%) or in the cervical spine alone (22%). Most (93%) C2 neurofibromas were visible on MRI scans of the head. Intradural invasion and cord compression in the cervical spine included the C2 level in 95% and 80% of patients, respectively. Compared with all other cervical spine neurofibromas in these patients, C2 neurofibromas had higher rates of intraspinal extension (75% vs 32%; OR 6.20, 95% CI 3.85-9.97; p < 0.001), intradural invasion (53% vs 26%; OR 3.20, 95% CI 2.08-4.92; p < 0.001), and cord compression (25% vs 13%; OR 2.26, 95% CI 1.35-3.79; p = 0.002). However, C2 neurofibromas had lower rates of extraforaminal growth beyond the transverse process (12% vs 62%; OR 0.09, 95% CI 0.05-0.16; p < 0.001).CONCLUSIONSC2 neurofibromas are associated with an aggressive intraspinal phenotype, limited growth outside the spinal canal, and an uncommon genetic profile. These observations require future study.


Assuntos
Neurofibroma/diagnóstico por imagem , Neurofibromatose 1/diagnóstico por imagem , Compressão da Medula Espinal/diagnóstico por imagem , Neoplasias da Medula Espinal/diagnóstico por imagem , Adolescente , Adulto , Feminino , Humanos , Imagem por Ressonância Magnética/métodos , Masculino , Neurofibroma/complicações , Neurofibromatose 1/complicações , Fenótipo , Compressão da Medula Espinal/complicações , Neoplasias da Medula Espinal/complicações , Coluna Vertebral/patologia , Adulto Jovem
6.
BMJ Case Rep ; 20182018 Oct 07.
Artigo em Inglês | MEDLINE | ID: mdl-30297491

RESUMO

An occurrence of vallecular neurofibroma with airway obstruction is extremely rare and to the best of our knowledge, no case report has been documented in the literature to date. Although microlaryngeal surgery with cold knife excision is the standard surgical procedure offered to patients of vallecular lesions, it can be successfully managed with the help of endoscopic coblation as later can provide a bloodless surgical field preventing the unmet need for the tracheostomy and reducing the postoperative morbidities.


Assuntos
Neoplasias Laríngeas/diagnóstico , Neurofibroma/diagnóstico , Adulto , Obstrução das Vias Respiratórias/etiologia , Transtornos de Deglutição/etiologia , Diagnóstico Diferencial , Endoscopia , Feminino , Humanos , Neoplasias Laríngeas/complicações , Neoplasias Laríngeas/diagnóstico por imagem , Neoplasias Laríngeas/cirurgia , Laringoscopia , Neurofibroma/complicações , Neurofibroma/diagnóstico por imagem , Neurofibroma/cirurgia , Tomografia Computadorizada por Raios X
7.
Neurology ; 91(2 Suppl 1): S1-S4, 2018 07 10.
Artigo em Inglês | MEDLINE | ID: mdl-29987129

RESUMO

OBJECTIVE: Outside of procedural-based methods, there are currently no established medical treatments for cutaneous neurofibroma (cNF), which afflict up to 99% of patients with NF1. Further, adult patients often report cNF are the greatest burden of living with NF1. The Neurofibromatosis Therapeutic Acceleration Program (NTAP) launched a think tank to address core questions to facilitate development of effective therapeutics for cNF in people with NF1. METHODS: Experts (with and without explicit experience with NF1 or cNF) from multiple scientific and medical disciplines, representing the ranks of academia, industry, and government agencies, were invited to become a member of a team addressing a specific subset of questions pertinent to cNF. Teams met monthly to review published and unpublished materials, and created summaries about the material known and unknown that may influence therapeutic development for cNF. Teams prioritized questions and organized supporting data, which was presented to the entire body of experts by each team at a research summit. RESULTS: Four themes were identified as being relevant to creating a comprehensive research strategy for cNF: (1) establishing definitions of cNF, (2) determining the biology of cNF with respect to tumor initiation, progression, and maintenance, (3) outlining the factors that guide therapies development, and (4) defining core considerations for clinical trials design and optimization for cNF. CONCLUSION: Considerations and key questions for each of the thematic areas were identified and provided basis for a request for applications launched by NTAP focused on cNF and are described in the accompanying articles of this supplement.


Assuntos
Neurofibroma/terapia , Neurofibromatose 1/complicações , Neoplasias Cutâneas/terapia , Ensaios Clínicos como Assunto , Dermatologia , Humanos , Neurofibroma/complicações , Neurologia , Projetos de Pesquisa , Neoplasias Cutâneas/complicações
8.
Neurology ; 91(2 Suppl 1): S5-S13, 2018 07 10.
Artigo em Inglês | MEDLINE | ID: mdl-29987130

RESUMO

OBJECTIVE: To present the current terminology and natural history of neurofibromatosis 1 (NF1) cutaneous neurofibromas (cNF). METHODS: NF1 experts from various research and clinical backgrounds reviewed the terms currently in use for cNF as well as the clinical, histologic, and radiographic features of these tumors using published and unpublished data. RESULTS: Neurofibromas develop within nerves, soft tissue, and skin. The primary distinction between cNF and other neurofibromas is that cNF are limited to the skin whereas other neurofibromas may involve the skin, but are not limited to the skin. There are important cellular, molecular, histologic, and clinical features of cNF. Each of these factors is discussed in consideration of a clinicopathologic framework for cNF. CONCLUSION: The development of effective therapies for cNF requires formulation of diagnostic criteria that encompass the clinical and histologic features of these tumors. However, there are several areas of overlap between cNF and other neurofibromas that make distinctions between cutaneous and other neurofibromas more difficult, requiring careful deliberation with input across the multiple disciplines that encounter these tumors and ultimately, prospective validation. The ultimate goal of this work is to facilitate accurate diagnosis and meaningful therapeutics for cNF.


Assuntos
Neurofibroma/diagnóstico , Neurofibroma/patologia , Neurofibromatose 1/patologia , Neoplasias Cutâneas/diagnóstico , Neoplasias Cutâneas/patologia , Humanos , Neurofibroma/classificação , Neurofibroma/complicações , Neurofibromatose 1/complicações , Qualidade de Vida , Neoplasias Cutâneas/classificação , Neoplasias Cutâneas/complicações
9.
Neurology ; 91(2 Suppl 1): S14-S20, 2018 07 10.
Artigo em Inglês | MEDLINE | ID: mdl-29987131

RESUMO

OBJECTIVE: A group of experts in dermatology, genetics, neuroscience, and regenerative medicine collaborated to summarize current knowledge on the defined factors contributing to cutaneous neurofibroma (cNF) development and to provide consensus recommendations for future research priorities to gain an improved understanding of the biology of cNF. METHODS: The group members reviewed published and unpublished data on cNF and related diseases via literature search, defined a set of key topic areas deemed critical in cNF pathogenesis, and developed recommendations in a series of consensus meetings. RESULTS: Five specific topic areas were identified as being relevant to providing an enhanced understanding of the biology of cNF: (1) defining the human cells of origin; (2) understanding the role of the microenvironment, focusing on neurons, mast cells, and fibroblasts; (3) defining the genetic and molecular differences between the cNFs, focusing on size and number; (4) understanding if sex hormones are critical for cNF development or progression; and (5) identifying challenges in establishing in vitro and in vivo models representing human cNF. CONCLUSIONS: The complexity of cNF biology stems from its heterogeneity at multiple levels including genetic, spatial involvement, temporal development, and cellular composition. We propose a unified working model for cNF that builds a framework to address the key questions about cNF that, when answered, will provide the necessary understanding of cNF biology to allow meaningful development of therapies.


Assuntos
Neurofibroma/fisiopatologia , Neurofibromatose 1/fisiopatologia , Neoplasias Cutâneas/fisiopatologia , Animais , Conferências de Consenso como Assunto , Humanos , Neurofibroma/complicações , Neurofibroma/genética , Neurofibromatose 1/complicações , Neurologia , Pesquisa , Neoplasias Cutâneas/complicações , Neoplasias Cutâneas/genética , Microambiente Tumoral
10.
Neurology ; 91(2 Suppl 1): S21-S30, 2018 07 10.
Artigo em Inglês | MEDLINE | ID: mdl-29987132

RESUMO

OBJECTIVE: The only therapies currently available for cutaneous neurofibromas (cNF) are procedural. The goals of the Therapies Development Working Group were to (1) summarize currently available treatment options for cNF, (2) define key considerations for drug discovery and development generally, and specifically for cNF, and (3) outline recommendations for the successful development of medical therapies for cNF. METHODS: The subgroup reviewed published and unpublished data on procedural, drug/device, and medical treatment approaches utilized for cNFs via literature search. The team defined disease- and patient-specific factors to consider for therapies development in a series of consensus meetings. RESULTS: The team identified 5 approaches entailing procedural and drug/device methods currently under study. There have been 4 clinical studies exploring various interventional therapies, from which outcomes were highly variable. The team identified 4 key factors to prioritize during the development of products for the treatment for cNF: safety, anatomic distribution of cNF, numbers of tumors to be treated, and route of administration. CONCLUSIONS: The number, size, and distribution of cNF is highly variable among patients with NF1 and it is possible that different phenotypes will require different drug development paths. The nonfatal nature of the disease and relatively limited patient numbers suggest that for any product to have a higher likelihood of acceptance, it will have to (1) demonstrate an effect that is clinically meaningful, (2) have a safety profile conducive to long-term dosing, and (3) have a low manufacturing cost.


Assuntos
Desenvolvimento de Medicamentos , Neurofibroma/tratamento farmacológico , Neurofibromatose 1/complicações , Neoplasias Cutâneas/tratamento farmacológico , Descoberta de Drogas , Humanos , Neurofibroma/complicações , Neoplasias Cutâneas/complicações
13.
Childs Nerv Syst ; 34(5): 877-882, 2018 05.
Artigo em Inglês | MEDLINE | ID: mdl-29455242

RESUMO

BACKGROUND: Neurofibromatosis type 1 (NF1) is a common autosomal dominantly inherited disorder that affects both the skin and the nervous system. NF1 occurs due to the mutations in the NF1 gene. Neurofibromas are the most common Schwann cell-based tumors in NF1 patients, which are mainly categorized into dermal and plexiform neurofibromas. Studies on different tumor types demonstrate that CXCR4 expression increases in tumor tissues and is linked to metastasis and cancer progression. PURPOSE: In the present study, we aimed to analyze the gene expression of CXCR4, and its ligand CXCL12, in human neurofibromas. METHODS: Eight NF1 patients aged between 5 and 37 (2 males, 6 females) were selected. The patient group comprised 1 plexiform neurofibroma, 1 pheochromocytoma, and 6 dermal neurofibromas. Following pathological examination and diagnosis, tumors were co-stained with antibodies against Schwann cell marker S100 and target molecule CXCR4. CXCR4 expression in Schwann cell-based tumors was detected at the protein level. RNA isolated from the same tumors was used for RT-PCR-based studies to measure the quantitative expression of CXCR4 and CXCL12. RESULTS: CXCR4 gene expression increased 3- to 120-fold and CXCL12 gene expression increased 33- to 512-fold in all human Schwann cell-based tumors. CONCLUSION: In order to validate the role of CXCR4 and its relationship with CXCL12 in NF1, future studies should be performed with additional tumors and different tumor types.


Assuntos
Quimiocina CXCL12/metabolismo , Regulação Neoplásica da Expressão Gênica/genética , Neurofibroma/patologia , Neurofibromatose 1/patologia , Receptores CXCR4/metabolismo , Células de Schwann/metabolismo , Adolescente , Adulto , Quimiocina CXCL12/genética , Criança , Pré-Escolar , Feminino , Humanos , Masculino , Neurofibroma/complicações , Neurofibroma/metabolismo , Neurofibromatose 1/complicações , Neurofibromatose 1/metabolismo , Receptores CXCR4/genética , Proteínas S100/metabolismo , Adulto Jovem
14.
Neuro Oncol ; 20(6): 818-825, 2018 05 18.
Artigo em Inglês | MEDLINE | ID: mdl-29409029

RESUMO

Background: Neurofibromatosis 1 (NF1) leads to the development of benign and malignant peripheral nerve sheath tumors (MPNST). MPNST have been described to develop in preexisting benign plexiform neurofibromas (PN) and have a poor prognosis. Atypical neurofibromas (ANF) were recently described as precursor lesions for MPNST, making early detection and management of ANF a possible strategy to prevent MPNST. We aimed to clinically characterize ANF and identify management approaches. Methods: We analyzed clinical, imaging, and pathology findings of all patients with NF1 and ANF at 3 institutions. Results: Sixty-three patients had 76 ANF (32M/31F; median age 27.1 y). On MRI, most ANF appeared as distinct nodular lesions and were 18F-fluorodeoxyglucose (FDG) avid. Forty-six ANF were associated with pain, 19 with motor weakness, 45 were palpable or visible, and 13 had no clinical signs. Completely resected ANF (N = 57) have not recurred (median follow-up, 4.1 y; range, 0-14 y). Four ANF transformed into MPNST and 17 patients had a history of MPNST in a different location than was their ANF. Conclusions: Growth of distinct nodular lesions, pain, and FDG-PET avidity should raise concern for ANF in NF1. Patients with ANF are at greater risk for development of MPNST. Complete resection of ANF may prevent development of MPNST.


Assuntos
Imagem por Ressonância Magnética/métodos , Neurofibroma/patologia , Neurofibromatose 1/patologia , Neurofibrossarcoma/patologia , Tomografia por Emissão de Pósitrons/métodos , Tomografia Computadorizada por Raios X/métodos , Adolescente , Adulto , Criança , Pré-Escolar , Feminino , Humanos , Lactente , Masculino , Pessoa de Meia-Idade , Neurofibroma/complicações , Neurofibroma/diagnóstico por imagem , Neurofibromatose 1/diagnóstico por imagem , Neurofibromatose 1/etiologia , Neurofibrossarcoma/diagnóstico por imagem , Neurofibrossarcoma/etiologia , Prognóstico , Adulto Jovem
17.
World J Surg Oncol ; 15(1): 160, 2017 Aug 23.
Artigo em Inglês | MEDLINE | ID: mdl-28835241

RESUMO

BACKGROUND: Neurofibromatosis type 1 (NF1) is an autosomally dominant inherited disorder characterized by multiple pigmented skin spots (café-au-lait spots) and neurofibroma. NF1 is associated with a wide variety of benign or malignant tumors. We report a NF1 patient who received surgical treatment for rectal carcinoma and multifocal small intestinal gastrointestinal stromal tumors (GISTs). CASE PRESENTATION: A 70-year-old female patient with NF1 was referred to our hospital after a positive fecal occult blood test. Locally advanced rectal carcinoma was detected in the upper rectum using colonoscopy. A submucosal tumor 20 mm in diameter was detected in the duodenal bulb during the upper gastrointestinal endoscopy. The biopsy specimen from the duodenum was GIST with positive immunostaining of KIT and CD34 microscopically. Laparoscopic low anterior resection for rectal carcinoma and local excision of the duodenal GIST were performed successfully. During the operation, five white small nodules were found on the serosa of the jejunum. One nodule was excised for histological examination. The resected rectal tumor was a well-differentiated adenocarcinoma with multiple lymph nodes metastases according to the histology. The duodenal tumor was found to be low-risk GIST. Moreover, the nodule from the jejunum was very low risk GIST. An excised skin wart was neurofibroma according to the histology. CONCLUSIONS: GIST or carcinomas have been reported to occasionally occur in the digestive tract of the patients with NF1. We present a rare case of a NF1 patient with GISTs and colorectal carcinoma.


Assuntos
Adenocarcinoma/complicações , Neoplasias Duodenais/complicações , Tumores do Estroma Gastrointestinal/complicações , Neoplasias Intestinais/complicações , Neoplasias Primárias Múltiplas/complicações , Neurofibroma/complicações , Neurofibromatose 1/complicações , Neoplasias Retais/complicações , Neoplasias Cutâneas/complicações , Adenocarcinoma/diagnóstico , Adenocarcinoma/patologia , Adenocarcinoma/terapia , Idoso , Biópsia , Quimioterapia Adjuvante/efeitos adversos , Quimioterapia Adjuvante/métodos , Meios de Contraste/administração & dosagem , Diarreia/induzido quimicamente , Procedimentos Cirúrgicos do Sistema Digestório/métodos , Neoplasias Duodenais/diagnóstico , Neoplasias Duodenais/patologia , Neoplasias Duodenais/terapia , Duodeno/diagnóstico por imagem , Duodeno/patologia , Duodeno/cirurgia , Endoscopia Gastrointestinal/métodos , Feminino , Tumores do Estroma Gastrointestinal/diagnóstico , Tumores do Estroma Gastrointestinal/patologia , Tumores do Estroma Gastrointestinal/terapia , Humanos , Neoplasias Intestinais/diagnóstico , Neoplasias Intestinais/patologia , Neoplasias Intestinais/terapia , Intestino Delgado/diagnóstico por imagem , Intestino Delgado/patologia , Intestino Delgado/cirurgia , Jejuno/diagnóstico por imagem , Jejuno/patologia , Jejuno/cirurgia , Laparoscopia/métodos , Metástase Linfática , Estadiamento de Neoplasias , Neoplasias Primárias Múltiplas/diagnóstico , Neoplasias Primárias Múltiplas/patologia , Neoplasias Primárias Múltiplas/terapia , Neurofibroma/diagnóstico , Neurofibroma/patologia , Neurofibroma/terapia , Neurofibromatose 1/diagnóstico , Neurofibromatose 1/patologia , Neurofibromatose 1/terapia , Sangue Oculto , Prognóstico , Neoplasias Retais/diagnóstico , Neoplasias Retais/patologia , Neoplasias Retais/terapia , Neoplasias Cutâneas/diagnóstico , Neoplasias Cutâneas/patologia
20.
Masui ; 66(4): 412-414, 2017 Apr.
Artigo em Japonês | MEDLINE | ID: mdl-30382644

RESUMO

Von Recklinghausen disease is an autosomal domi- nant neurocutaneous disorder, characterized by cuta- neous neurofibromas, accompanied by café-au-lait spots. We report a case of a 51-year-old man with von Recklinghausen disease who had been operated on for thoracic and spinal neurofibroma. Exploratory thoracotomy was performed under general anesthesia combined with epidural anesthesia. After tracheal intubation with spiral tube, one lung ventilation was performed using blocker tube. General anesthesia was maintained by inhalation of oxygen, air, and desflurane and the continuous infusion of remifentanil. The opera- tive course was uneventful. The patient emerged from general anesthesia smoothly, and was extubated safely. There was no neurological abnormality after operation. Preoperative evaluation of airway status and neuro- logical findings are essential in the anesthetic management of the patient with von Recklinghausen disease. This case suggests that we must take the complication into account for anesthetic management and select the appropriate anesthetic method by routine preoperative estimation. It is of great importance that anesthesiologists evaluate the airway status and neurological find- ings of patients with von Recklinghausen disease.


Assuntos
Neurofibroma/cirurgia , Neurofibromatose 1/complicações , Anestesia Geral , Anestésicos , Humanos , Intubação Intratraqueal , Masculino , Pessoa de Meia-Idade , Neurofibroma/complicações
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