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vascularity of neurofibroma

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
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
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
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
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
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
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
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...
April 6, 2016: Auris, Nasus, Larynx
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
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
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
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
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
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
Jacqueline L Anderson, David H Gutmann
Neurofibromatosis type 1 (NF1), previously known as von Recklinghausen disease, is a neurogenetic disorder distinct from neurofibromatosis type 2 (NF2). Approximately 1:2500 to 1:3500 individuals worldwide are affected, regardless of ethnicity or race. The classic manifestations of NF1 include café-au-lait macules, skinfold freckling, neurofibromas, brain tumors, iris hamartomas, and characteristic bony lesions. In addition, patients with NF1 are at increased risk for learning and intellectual disabilities, aqueductal stenosis, pheochromocytoma, vascular dysplasia, scoliosis, and cancer...
2015: Handbook of Clinical Neurology
Jeong Ah Ryu, Sang Hoon Lee, Eun-Young Cha, Tae Yeob Kim, Sung Moon Kim, Myung Jin Shin
OBJECTIVES: The purpose of this study was to determine key features and define a strategy for differentiation between schwannomas and neurofibromas using sonography. METHODS: This retrospective study was approved by the Institutional Review Board at our hospital, and informed consent was waived. We reviewed sonograms of pathologically proven schwannomas and neurofibromas of the extremities and body wall. On grayscale images, tumors were evaluated on the basis of their size, maximum-to-minimum diameter ratio, shape, contour, margin, location, encapsulation, echogenicity, echo texture, cystic changes, presence of intratumoral calcifications, presence of a target sign, and presence of an entering or exiting nerve...
December 2015: Journal of Ultrasound in Medicine: Official Journal of the American Institute of Ultrasound in Medicine
Tushar B Patil, Maneesh Kumar Singh, Rakesh Lalla
Type 1 neurofibromatosis (NF1), which mainly involves ectodermal tissue arising from the neural crest, can increase the risk of developing malignant peripheral nerve sheath tumors (MPNSTs), soft tissue sarcomas and subarachnoid hemorrhage. We describe a patient with neurofibromatosis type 1 who developed soft tissue sarcoma, MPNST, and subarachnoid hemorrhage. A 22-year-old male reported right focal seizures consequence to severe headache. He had a weakness in both legs, could walk only with the support of a stick for the last 3 months and suffered from constipation and intermittent urinary retention for the past 1 week...
July 2015: Journal of Natural Science, Biology, and Medicine
Reinhard E Friedrich, Urs Naber, Markus Glatzel, Christian Hagel
BACKGROUND/AIM: Peripheral nerve sheath (PNS) tumors constitute a heterogeneous group of solid tumors. Neurofibroma and schwannoma are the most frequently diagnosed entities. Both tumor types occur sporadically and are associated with syndromes. Current strategies to fight PNS progression by means of pharmaceuticals aim to specifically interfere with vascular growth factors identified in PNS. Furthermore, malignant transformation of PNS tumors is known to be associated with a change in vascularization...
September 2015: Anticancer Research
Peter B Bartline, Stephen H McKellar, Daniel V Kinikini
BACKGROUND: Venous aneurysms are exceedingly rare manifestations of neurofibromatosis type 1 (NF1). There are only a handful of cases reported, and no prior cases describing treatment of mediastinal venous aneurysms in this patient population exist. CASE REPORT: A 58-year-old woman with NF1 presented with a right neck mass. The mass had recently doubled in size and was associated with cough, hoarseness of voice, and pain. Her pertinent medical history included untreated obstructive sleep apnea, severe pulmonary hypertension, and a recent hospital admission for pneumonia...
January 2016: Annals of Vascular Surgery
Kevin J Kovatch, Michael T Purkey, Maria Martinez-Lage, Roberta E Gausas, Laurie A Loevner, M Sean Grady, Bert W O'Malley, Christopher H Rassekh
Neurofibromatosis types 1 (NF-1) and 2 are significant entities to otolaryngologists because they frequently involve head and neck skin, brain, skull base, orbits, and surrounding neural and vascular structures. We present the case of a 52-year-old male with progressive, marked unilateral proptosis due to a multilobulated orbital mass, secondary to biopsy-proven plexiform neurofibroma (PN). Acute worsening of proptosis leading to corneal abrasion, diplopia, and pain required debulking surgery, for which an orbitozygomatic approach was utilized...
November 2015: Laryngoscope
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