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vascularity of neurofibroma

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https://www.readbyqxmd.com/read/28405554/arteriovenous-malformation-underlying-a-plexiform-neurofibroma-an-unusual-presentation
#1
Zaheer Abbas, Sepideh Khani, Javad Zare
Vascular abnormalities associated with neurofibromatosis type 1 are well described in the literature, however, arteriovenous malformation is a very rare finding in neurofibromatosis type 1. We report the case of an 11-year-old girl who presented with a soft mass on the right flank. Provisional diagnosis of plexiform neurofibroma was made on the basis of clinical and histological observations. Because the lesion was warm on palpation, imaging studies were performed to evaluate further and arteriovenous malformation was detected underlying the plexiform neurofibroma...
March 2017: Indian Dermatology Online Journal
https://www.readbyqxmd.com/read/28398289/a-high-throughput-molecular-data-resource-for-cutaneous-neurofibromas
#2
Sara J C Gosline, Hubert Weinberg, Pamela Knight, Thomas Yu, Xindi Guo, Nripesh Prasad, Angela Jones, Shristi Shrestha, Braden Boone, Shawn E Levy, Salvatore La Rosa, Justin Guinney, Annette Bakker
Neurofibromatosis type 1 (NF1) is a genetic disorder with a range of clinical manifestations such as widespread growth of benign tumours called neurofibromas, pain, learning disorders, bone deformities, vascular abnormalities and even malignant tumours. With the establishment of the Children's Tumour Foundation biobank, neurofibroma samples can now be collected directly from patients to be analysed by the larger scientific community. This work describes a pilot study to characterize one class of neurofibroma, cutaneous neurofibromas, by molecularly profiling of ~40 cutaneous neurofibromas collected from 11 individual patients...
April 11, 2017: Scientific Data
https://www.readbyqxmd.com/read/28387148/malignant-triton-tumor-malignant-peripheral-nerve-sheath-tumor-with-rhabdomyoblastic-differentiation-occurring-in-a-vascularized-free-flap-reconstruction-graft
#3
Roopa Ram, Jerad Gardner, Sindhura Alapati, Kedar Jambhekar, Tarun Pandey, Corey Montgomery, Richard Nicholas
Malignant peripheral nerve sheath tumor (MPNST) is a rare form of sarcoma arising from Schwann cells or pluripotent cells of the neural crest. Malignant triton tumor (MTT) is a subtype of MPNST with a component of malignant rhabdomyoblasts in addition to malignant Schwann cells. MPNST and MTT are both aggressive malignancies that most commonly arise from large deep neurofibromas in patients with neurofibromatosis type 1 (NF-1). However, sporadic non-NF-1 cases of MTT have also been reported in the literature...
April 1, 2017: International Journal of Surgical Pathology
https://www.readbyqxmd.com/read/28163183/a-femoral-common-vein-aneurysm-in-a-patient-with-neurofibromatosis-syndrome-type-1
#4
Christophe Seinturier, Sophie Blaise, Frédéric Thony, Jean Luc Magne, Gilles Pernod
Neurofibromatosis type I (NFI), also called Von Recklinghausen disease, is an autosomal dominant disease secondary to a genetic mutation on the long arm of chromosome 17. This disorder affects neural crest cells. Cutaneous clinical forms are the most frequent with multiple benign skin neurofibromas, associated with café au lait skin spots and iris hamartomas. Vascular abnormalities in NF1 are rare but have also been well described. The most frequent abnormalities are characterized by arterial aneurysm degeneration, stenosis, and malformations...
February 3, 2017: Annals of Vascular Surgery
https://www.readbyqxmd.com/read/28145924/characteristics-of-pediatric-cheek-mass
#5
Ye Seul Eom, So Young Lim
PURPOSE: Patients with cheek mass are often referred to our medical center's department of plastic surgery. Most patients with deep cheek mass have the characteristic of a slowly growing, painless mass. We reviewed the lesion's pathology of this complex anatomic area in the pediatric population, focusing on differential diagnosis and the recent surgical strategies. METHODS: We retrospectively reviewed 56 patients visiting our department from 2009 to 2016. Data analysis included the patients' lesion characteristics, clinical presentation, presumptive diagnosis, results of preoperative investigations, pathological diagnosis, and treatment details...
January 31, 2017: Journal of Craniofacial Surgery
https://www.readbyqxmd.com/read/27942480/diagnostic-imaging-of-benign-and-malignant-neck-masses-in-children-a-pictorial-review
#6
REVIEW
Ruth Elizabeth Brown, Srikrishna Harave
Neck masses are frequently encountered in pediatric medicine, and can present a diagnostic dilemma for the clinicians involved. There are several means by which neck masses in children can be subdivided, for example by age at presentation, anatomical location including compartments and fascia of the neck, their classical appearance when imaged, or by etiology. When imaging children the clinicians must be mindful of radiation exposure and as such ultrasound (US) is often attempted first. Cross sectional imaging can be helpful for problem solving with CT being particularly useful for assessing the patient in more acute scenarios, for example when there is airway compromise...
October 2016: Quantitative Imaging in Medicine and Surgery
https://www.readbyqxmd.com/read/27625246/evaluation-of-peripheral-nerve-lesions-with-high-resolution-ultrasonography-and-color-doppler
#7
Muhammed Afsal, Veena Chowdhury, Anjali Prakash, Sapna Singh, Neera Chowdhury
PURPOSE: Traditionally, peripheral nerve lesions are diagnosed on the basis of clinical history, physical examination, and electrophysiological studies, and the role of imaging studies has been limited. The purpose of the study was to assess the usefulness of sonography in diagnosing peripheral nerve lesions. MATERIALS AND METHODS: Thirty adult patients with peripheral nerve lesion/s and 30 healthy adult volunteers were included in the study, and sonography of the relevant peripheral nerve/s was done...
September 2016: Neurology India
https://www.readbyqxmd.com/read/27518244/ruptured-left-external-carotid-artery-aneurysm-presenting-as-upper-airway-obstruction-in-von-recklinghausen-s-disease
#8
Hajar Souldi, Mohammed Yahya Bajja, Meriem Chenguir, RedaLah Abada, Samy Rouadi, Mohammed Roubal, Mohammed Mahtar
INTRODUCTION: Neurofibromatosis type 1 (NF-1) is an autosomal dominant disorder characterized by café-au-lait macules, neurofibromas, and iris hamartomas. Carotid artery aneurysms rarely affect patients with NF-1 but may be associated with rupture. We report the first episode of a ruptured external carotid aneurysm with severe life-threatening airway obstruction in a NF-1 patient. PRESENTATION OF CASE: We report a case of NF-1 32-year-old woman admitted to our department for life-threatening upper airway obstruction caused by spontaneous expanding swelling in the left sided neck...
2016: International Journal of Surgery Case Reports
https://www.readbyqxmd.com/read/27497331/sudden-death-due-to-rupture-of-the-right-internal-carotid-artery-in-neurofibromatosis-type-1-a-case-report
#9
Yue Liang, Fang Tong, Lin Zhang, Wenhe Li, Yiwu Zhou
Vascular involvement is a well-recognized manifestation of neurofibromatosis type 1 (NF1) which has the potential to be fatal when disrupted. We here present a case of sudden death due to the fatal arterial rupture resulted from infiltration of the neurofibromas. A 42-year-old man who suffered from NF1 presented a 1-h history of sudden onset of pain in his right cervical region. His condition worsened and became unconscious on his way to the emergency room. Despite resuscitation efforts, he died 30min later without regaining consciousness...
July 2016: Legal Medicine
https://www.readbyqxmd.com/read/27476838/histopathological-examination-of-spine-tumors-after-treatment-with-radiosurgery
#10
Nathan T Zwagerman, Michael M McDowell, Ronald L Hamilton, Edward A Monaco, John C Flickinger, Peter C Gerszten
OBJECTIVE Increased survival time after diagnosis of neoplastic disease has resulted in a gradual increase in spine tumor incidence. Radiosurgery is frequently a viable alternative to operative management in a population with severe medical comorbidities. The authors sought to assess the histopathological consequences of radiosurgery in the subset of patients progressing to operative intervention. METHODS Eighteen patients who underwent radiosurgery for spine tumors between 2008 and 2014 subsequently progressed to surgical treatment...
August 2016: Neurosurgical Focus
https://www.readbyqxmd.com/read/27376229/sympathetic-chain-schwannoma-resembling-carotid-body-tumour
#11
Tallat Najeeb, Musaddiq Khan
Schwannomas are rare, benign nerve sheath tumours of parapharyngeal space. Differential diagnosis should include salivary gland tumours, paragangliomas, neurofibromas, and metastatic lymph nodes. The tumours may arise from vagus nerve and cervical sympathetic chain (CSC). Diagnosis is usually made by imaging techniques: contrast CT, magnetic resonance imaging (MRI), and magnetic resonance angiography (MRA). Fine needle aspiration cytology (FNAC) is useful diagnostic procedure but poor results are seen in neurogenic tumours...
June 2016: Journal of the College of Physicians and Surgeons—Pakistan: JCPSP
https://www.readbyqxmd.com/read/27192529/isolated-plexiform-neurofibroma-mimicking-a-vascular-lesion
#12
Paola Cecilia Stefano, Sebastian Nicolas Apa, Agustina Maria Lanoël, Josefina Sala María, Sergio Sierre, Adrián Martin Pierini
Plexiform neurofibromas are benign tumors originating from peripheral nerve sheaths, generally associated with Neurofibromatosis Type 1 (NF1). They are diffuse, painful and sometimes locally invasive, generating cosmetic problems. This report discusses an adolescent patient who presented with an isolated, giant plexiform neurofibroma on her leg that was confused with a vascular lesion due to its clinical aspects. Once the diagnosis was confirmed by surgical biopsy, excision of the lesion was performed with improvement of the symptoms...
April 2016: Anais Brasileiros de Dermatologia
https://www.readbyqxmd.com/read/27127789/27-12%C3%A2-mhz-radiofrequency-ablation-for-benign-cutaneous-lesions
#13
Dong Hyun Kim, Dong Ju Hyun, Raymonde Piquette, Clément Beaumont, Lucie Germain, Danielle Larouche
As surgical and/or ablative modalities, radiofrequency (RF) has been known to produce good clinical outcomes in dermatology. Recently, 27.12 MHz RF has been introduced and has several advantages over conventional 4 or 6 MHz in terms of the precise ablation and lesser pain perception. We aimed to evaluate the clinical efficacy and safety of 27.12 MHz RF for the treatment of benign cutaneous lesions. Twenty female patient subjects were enrolled. Digital photography and a USB microscope camera were used to monitor the clinical results before one session of treatment with 27...
2016: BioMed Research International
https://www.readbyqxmd.com/read/27061148/a-rare-case-of-a-spontaneous-neck-hematoma-in-a-patient-with-type-1-neurofibromatosis
#14
Stamatia Dova, Kyriakos Ktenidis, Petros Karkos, Sarantis Blioskas, Georgios Psillas, Alexandros Iliadis, Konstantinos Markou
Neurofibromatosis type 1 (NF-1) is a genetic disorder that affects one in 3000 individuals. Although NF-1 notably involves nerves and connective tissue, vascular involvement in large series is estimated to range from 0.4% to 6.4%. Jugular vein involvement in these patients is rare. Spontaneous neck hematomas and hemorrhages are also unusual. We present a case of a NF-1 patient with a spontaneous neck hematoma with possible leakage from the left internal jugular vein, presenting as a lateral neck mass. The fragility of the vein wall and the surrounding tissue led patient to a severe intraoperative bleeding...
October 2016: Auris, Nasus, Larynx
https://www.readbyqxmd.com/read/26949362/peripheral-retinal-ischemia-in-a-young-indian-woman-with-neurofibromatosis-type-1
#15
Anisha Seth, Basudeb Ghosh, Anika Gupta, Neha Goel
Neurofibromatosis type 1 (NF-1) is an autosomal dominantly inherited disease characterized by café-au-lait spots, neurofibromas, axillary freckling, Lisch nodules of iris, gliomas and various systemic vascular ischemic manifestations mainly in the aorta, brain and kidney. Retinal vascular manifestations in patients with NF-1 are usually representative of retinal capillary hemangiomatosis. Few cases of NF-1 with retinal vascular occlusive disease have been described. We describe a young Indian woman with NF-1 with unilateral peripheral retinal ischemia but no vascular abnormality at the posterior pole...
January 2016: Saudi Journal of Ophthalmology: Official Journal of the Saudi Ophthalmological Society
https://www.readbyqxmd.com/read/26868134/misdiagnosis-of-plexiform-neurofibroma-as-venous-malformation-in-pediatric-patients
#16
Robert K Clemens, Anna P Lillis, Jeannette Perez-Rossello, Raja Shaikh, Christoph Thalhammer, Beatrice R Amann-Vesti, Ahmad I Alomari
BACKGROUND: The aim of this study was to identify Magnetic Resonance Imaging (MRI) characteristics that reliably distinguish deep plexiform neurofibromas (PNFs) from venous malformations (VMs). METHODS: A database search was conducted for patients that were referred with a vascular anomaly but had a neurofibroma instead. Clinical and imaging features of patients with venous malformations as the most common referral diagnosis were compared to those with PNFs. The imaging features of deep PNFs recorded were: anatomical location, size, morphology, margins, signal intensity and post-contrast enhancement pattern...
December 2016: International Angiology: a Journal of the International Union of Angiology
https://www.readbyqxmd.com/read/26738373/pulmonary-hypertension-and-von-recklinghausen-s-disease-association-and-therapeutic-difficulties
#17
K Ennibi, I El Kassimi, I Asfalou, J Chaari, A Benyass
The neurofibromatosis type 1 (NF1) or Von Recklinghausen's disease is a genetic disorder. The café-au-lait spots and neurofibromas are the most common manifestations. Respiratory symptoms are rare in this disease, described as neurofibromas, infiltrative lesions, cysts, bubbles or emphysema. Pulmonary hypertension is rarely reported. It is due to the plexiform lesions in pulmonary arterioles or to parenchymal lung lesions reducing the vascular bed. We report a case of idiopathic precapillary pulmonary hypertension in a young patient with Von Recklinghausen's disease...
July 2015: Pneumologia: Revista Societății Române de Pneumologie
https://www.readbyqxmd.com/read/26690608/simultaneous-presentation-of-malignant-peripheral-nerve-sheath-tumor-and-moyamoya-disease-associated-with-neurofibromatosis-type-1-in-a-child
#18
Sun Young Yoo, Sun Mi Hwang, Min Kyung Lee, Dae Sun Jo, Pyoung Han Hwang
Neurofibromatosis type 1 (NF-1) is a rare hereditary disorder, which is inherited as an autosomal dominant trait. It is characterized by multiple caféau- lait spots of the skin, benign cutaneous neurofibromas, skeletal dysplasia and learning disability. The association of NF-1 with benign and malignant tumors is well established. The lifetime risk of patients with NF-1 developing malignant peripheral nerve sheath tumors (MPNSTs) has been estimated to be 8-13%. Such tumors can develop in any part of the body, but their occurrence in the gastrointestinal tract is rare...
March 2015: Turkish Journal of Pediatrics
https://www.readbyqxmd.com/read/26637864/vascular-innervation-in-benign-neurofibromas-of-patients-with-neurofibromatosis-type-1
#19
Reinhard E Friedrich, Christian-Alexander Behrendt, Markus Glatzel, Christian Hagel
UNLABELLED: Neurofibroma constitutes a heterogeneous group of solid tumours occurring sporadically or in association with syndromes. The aspect of these peripheral nerve sheath tumours may vary considerably, with disseminated tumours covering various parts of the body or nodular/diffuse plexiform neurofibroma that can grow to an impressive size. Although neurofibromas have vascular density comparable to that of normal tissue, they have tendency to bleed upon surgery which is poorly understood...
December 2015: Anticancer Research
https://www.readbyqxmd.com/read/26591676/large-neurofibroma-of-the-labia-majora-a-case-report
#20
Hussein L Kidanto, Joshua Garrison, Peter Wangwe
Neurofibromatosis is an autosomal dominant progressive disorder with an incidence of approximately 1 in 3000 live births. Its recognized features include hyper-pigmented skin lesions (cafe-au-lait spots), neurofibromas, iris hamartomas, macrocephaly, central nervous system tumours, defects of the skull and facial bones, and vascular lesions. Involvement of the external genitalia is extremely unusual. This report describes a case of a vulva neurofibroma in a 15-years old teenage girl with no history of trauma or features of Von Recklinghausen's disease...
January 2013: Tanzania Journal of Health Research
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