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Plexiform Neurofibroma

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https://www.readbyqxmd.com/read/29670726/decayed-missing-and-restored-teeth-in-patients-with-neurofibromatosis-type-1
#1
Reinhard E Friedrich, Anika Reul
Background: NF1 is a relatively frequently occurring autosomal dominant inherited disease. There are conflicting reports about oral health status in NF1. The aim of this study was to analyze the dental status of patients with neurofibromatosis type 1 (NF1). Material and Methods: Radiographs of 179 patients with NF1 were analyzed for decayed, missing, and filled teeth (DMFT) in a cross-sectional, retrospective study. The results were compared to age- and sex-matched controls of individuals not affected by NF1...
February 2018: Journal of Clinical and Experimental Dentistry
https://www.readbyqxmd.com/read/29662612/exploiting-mitochondrial-and-metabolic-homeostasis-as-a-vulnerability-in-nf1-deficient-cells
#2
Robert J Allaway, Matthew D Wood, Sondra L Downey, Stephanie J Bouley, Nicole A Traphagen, Jason D Wells, Jaya Batra, Sir Norman Melancon, Carol Ringelberg, William Seibel, Nancy Ratner, Yolanda Sanchez
Neurofibromatosis type 1 is a disease caused by mutation of neurofibromin 1 ( NF1 ), loss of which results in hyperactive Ras signaling and a concomitant increase in cell proliferation and survival. Patients with neurofibromatosis type 1 frequently develop tumors such as plexiform neurofibromas and malignant peripheral nerve sheath tumors. Mutation of NF1 or loss of the NF1 protein is also observed in glioblastoma, lung adenocarcinoma, and ovarian cancer among other sporadic cancers. A therapy that selectively targets NF1 deficient tumors would substantially advance our ability to treat these malignancies...
March 23, 2018: Oncotarget
https://www.readbyqxmd.com/read/29651404/unusual-evolution-of-plexiform-neurofibroma-in-the-scalp-a-case-report
#3
Amine Rafik, Mounia Diouri, Naima Bahechar, Abdessamad Chlihi
The neurofibromatosis is a frequent and polymorphic genetic disorder. The severity is related to the complications. The degeneration of neurofibroma is a very rare complication of neurofibromatosis. In the literature, a few cases of solitary neurofibroma, which turned into a malignant tumor were reported. In our case, we described a very rare clinical case of neurofibrosarcoma in the scalp, and surgical treatment.
January 2018: World Journal of Plastic Surgery
https://www.readbyqxmd.com/read/29607552/pathophysiology-and-management-of-glaucoma-associated-with-phakomatoses
#4
REVIEW
Alisa T Thavikulwat, Deepak P Edward, Abdulrahman AlDarrab, Thasarat S Vajaranant
The phakomatoses, encephalotrigeminal angiomatosis (ETA; Sturge-Weber Syndrome), neurofibromatosis type 1 (NF1 or von Recklinghausen disease), Von Hippel-Lindau (VHL) disease, tuberous sclerosis (TSC), oculodermal melanocytosis (ODM), and phakomatosis pigmentovascularis are a group of neurocutaneous disorders that have characteristic systemic and ocular manifestations. Through many different mechanisms, they may cause glaucomatous damage of the optic nerve and subsequent vision loss varying from mild to severe...
April 1, 2018: Journal of Neuroscience Research
https://www.readbyqxmd.com/read/29599350/periapical-cemento-osseous-dysplasia-is-rarely-diagnosed-on-orthopantomograms-of-patients-with-neurofibromatosis-type-1-and-is-not-a-gender-specific-feature-of-the-disease
#5
Reinhard E Friedrich, Anika Reul
Several skeletal aberrations of the skull have been described for the tumor predisposition syndrome neurofibromatosis type 1 (NF1). Recently, periapical cemental/cemento-osseous dysplasia (COD) has been described in females affected with NF1. This reactive lesion of the hard tissues in tooth-bearing areas of the jaw has been proposed to represent a gender-specific radiological feature of NF1. The aim of this study was to investigate the prevalence of COD in patients with NF1. PATIENTS AND METHODS: The orthopantomograms (OPGs) of 179 patients with a confirmed diagnosis of NF1 were analyzed for COD...
April 2018: Anticancer Research
https://www.readbyqxmd.com/read/29596064/contributions-of-inflammation-and-tumor-microenvironment-to-neurofibroma-tumorigenesis
#6
Chung-Ping Liao, Reid C Booker, Jean-Philippe Brosseau, Zhiguo Chen, Juan Mo, Edem Tchegnon, Yong Wang, D Wade Clapp, Lu Q Le
Neurofibromatosis type 1 associates with multiple neoplasms and the Schwann cell tumor neurofibroma is the most prevalent. A hallmark feature of neurofibroma is mast cell infiltration which is recruited by chemoattractant stem cell factor (SCF) that has been suggested to sustain neurofibroma tumorigenesis. In this study, using new genetically engineered Scf mice, we decipher the contributions of tumor-derived SCF and mast cells to neurofibroma development. We demonstrate that mast cell infiltration is dependent on SCF from tumor Schwann cells...
March 29, 2018: Journal of Clinical Investigation
https://www.readbyqxmd.com/read/29583067/neurofibromatous-neuropathy-an-ultrastructural-study
#7
Chiara Terracciano, Christa Pachatz, Emanuele Rastelli, Francesco Saverio Pastore, Mariarosa Anna Beatrice Melone, Roberto Massa
Plexiform neurofibroma is pathognomonic of neurofibromatosis 1 (NF1). An NF1-associated peripheral neuropathy has been described in a small minority of NF1 patients but its histopathological features are poorly characterized. We report the case of a 46-year-old woman presenting with bilateral supraclavicular painful masses without other stigmata of NF1. MRI showed bilateral plexiform lesions extending from cervical roots to the elbows. Nerve conduction studies documented a sensory motor polyneuropathy. Morphometric analysis of sural nerve biopsy showed a preferential loss of large-caliber myelinated fibers with a g ratio of 0...
March 27, 2018: Ultrastructural Pathology
https://www.readbyqxmd.com/read/29581693/large-solitary-plexiform-neurofibroma-of-the-penis-without-erectile-dysfunction-a-case-report-from-kerala
#8
Shanavas Cholakkal, Subhash V C, Gokul Nachiketh
Case Report: Solitary plexiform neurofibroma of the penis is an extremely rare peripheral nerve lesion with only about 20 cases reported all around the world. Here, we are reporting a case of solitary penile plexiform neurofibroma with sexual dysfunction due to difficulty in penetration. This 47-year-old gentleman presented with a swelling on the dorsal side of the penis. He was aware of the swelling for the last 15 years. The swelling was slow growing and there was no associated loss of sensation or sexual dysfunction...
February 2018: Indian Journal of Surgery
https://www.readbyqxmd.com/read/29570943/the-relationship-between-heart-rate-variability-psychological-flexibility-and-pain-in-neurofibromatosis-type-1
#9
Taryn M Allen, Kari L Struemph, Mary Anne Toledo-Tamula, Pamela L Wolters, Andrea Baldwin, Brigitte Widemann, Staci Martin
Individuals with neurofibromatosis type 1 (NF1) and plexiform neurofibromas (PNs) can experience chronic pain. Previous research has examined the relationship between heart rate variability (HRV) and persistent pain. HRV is an index of autonomic nervous system functioning, and reflects the variability in time elapsed between heartbeats. Patients with chronic pain tend to exhibit lower HRV, which has been associated with poor adaptability, or psychological flexibility, to stress. The aim of the current study was to examine relationships between HRV, psychological flexibility and pain in a sample of adolescents and young adults (AYA) with NF1 and PNs...
March 23, 2018: Pain Practice: the Official Journal of World Institute of Pain
https://www.readbyqxmd.com/read/29503567/the-investigation-for-potential-modifier-genes-in-patients-with-neurofibromatosis-type-1-based-on-next-generation-sequencing
#10
Fan Yang, Song Xu, Renwang Liu, Tao Shi, Xiongfei Li, Xuebing Li, Gang Chen, Hongyu Liu, Qinghua Zhou, Jun Chen
Introduction: Neurofibromatosis type 1 (NF1) is a common Mendelian multi-system disorder that is characterized by café-au-lait spots (CLS), axillary freckling, optic glioma and plexiform neurofibroma. Various mutations of the NF1 gene are widely accepted to be the main cause of this disease, while whether there are still certain other modifier genes that could influence the phenotypes of NF1 is our concern. Patients and Methods: One proband and his father are involved, who are characterized by plexiform neurofibroma and cutaneous neurofibroma, respectively...
2018: OncoTargets and Therapy
https://www.readbyqxmd.com/read/29497706/plexiform-neurofibroma-of-the-posterior-tibial-nerve-misdiagnosed-as-proximal-tarsal-tunnel-syndrome-a-case-report
#11
Sang Hyun Nam, Jung Yeon Kim, Jaeki Ahn, Yongbum Park
Plexiform neurofibromas of the foot are rare, benign tumors of the peripheral nerves. Diagnosis can be challenging if they present with symptoms mimicking other peripheral nerve pathologies. Tarsal tunnel syndrome is an entrapment syndrome of the entire tibial nerve behind the medial malleolus and under the flexor retinaculum. The clinical presentation typically includes posteromedial pain, positive Tinel's sign, and neurogenic signs, including both the sensation of numbness and the actual hypoesthesia and clawing of the toes...
January 2018: The surgery journal
https://www.readbyqxmd.com/read/29478059/skin-examination-an-important-diagnostic-tool-in-renal-failure-patients
#12
Karen M Van de Velde-Kossmann
Renal failure is common in the United States with an estimated prevalence of 660,000 treated end-stage renal disease patients in 2015 [<xref ref-type="bibr" rid="ref1">1</xref>]. Causes of renal failure are many, and complications from renal failure, underlying disease, and treatment are not infrequent. Examples of common skin manifestations include xerosis, pigmentary change, and nail dystrophies. Frequent disease-specific skin changes may be helpful in the diagnosis of primary disorders leading to renal disease or severity of disease including bullosis diabeticorum, sclerodactyly, or leukoctoclastic vasculitis...
January 26, 2018: Blood Purification
https://www.readbyqxmd.com/read/29455242/heightened-cxcr4-and-cxcl12-expression-in-nf1-associated-neurofibromas
#13
Beren Karaosmanoglu, Çetin Y Kocaefe, Figen Söylemezoğlu, Banu Anlar, Ali Varan, İbrahim Vargel, Sükriye Ayter
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...
February 17, 2018: Child's Nervous System: ChNS: Official Journal of the International Society for Pediatric Neurosurgery
https://www.readbyqxmd.com/read/29449010/a-brief-report-of-plexiform-neurofibroma
#14
Mahdi Khajavi Md, Shahrokh Khoshsirat Md, Lida Ahangarnazari Md, Niloofar Majdinasab Md
Plexiform neurofibroma (PNF) is a rare variant of neurofibromatosis type1 (NF-1), which histopathologically, is a subtype of benign nerve sheath tumors, neurofibromas (NF). It develops as a result of proliferation in all parts of peripheral nervous system and can cause the functional damage, deformities, pain, considerable mortality, and morbidity and even the increasing risk of malignant transformation in some critical cases. Currently, the surgical intervention is the treatment of choice for PNF patients, which due to the tumor invasion, massive growth, and the chance of postoperative regrowth is not possible...
January 10, 2018: Current Problems in Cancer
https://www.readbyqxmd.com/read/29441226/plexiform-neurofibroma-in-the-axilla-with-intraosseous-neurofibroma-in-the-humerus-in-neurofibromatosis-5
#15
Sanjay Mhalasakant Khaladkar, Surbhi Chauhan, Raghav Kalra, Shishir Chauhan
Segmental neurofibromatosis type 5 (NF5) is a rare form of NF, in which the cutaneous and/or neural changes are confined to one region of the body. It is often underdiagnosed or undetected due to the absence of symptoms. Inheritance of NF is different from other types of NF. Intraosseous neurofibromas are rare tumors which develop from nerves supplying the periosteum or nerves following the intraosseous course. Plexiform neurofibroma with associated intraosseous neurofibroma is extremely rare. We report a case of plexiform neurofibroma in the left axilla of a 17-year-old female with associated intraosseous neurofibroma in adjoining left humerus...
2018: Journal of Clinical Imaging Science
https://www.readbyqxmd.com/read/29435207/supernumerary-molars-and-wisdom-tooth-shape-alterations-in-patients-with-neurofibromatosis-type-1
#16
Reinhard E Friedrich, Anika Reul
Objectives: The purpose of the present study was the systematic investigation of numerical and morphological aberrations of teeth in the wisdom teeth region in neurofibromatosis type 1 patients. Material and Methods: The panoramic radiographs of 179 patients were analysed for supernumerary molars and dysplastic wisdom teeth with special reference to facial tumour type. The results were compared to radiographic findings obtained in panoramic radiographs of age- and sex-matched controls...
October 2017: Journal of Oral & Maxillofacial Research
https://www.readbyqxmd.com/read/29427150/programmed-death-ligand-1-expression-and-tumor-infiltrating-lymphocytes-in-neurofibromatosis-type-1-and-2-associated-tumors
#17
Shiyang Wang, Benjamin Liechty, Seema Patel, Jeffrey S Weber, Travis J Hollmann, Matija Snuderl, Matthias A Karajannis
Immune checkpoint inhibitors targeting programmed cell death 1 (PD-1) or its ligand (PD-L1) have been shown to be effective in treating patients with a variety of cancers. Biomarker studies have found positive associations between clinical response rates and PD-L1 expression on tumor cells, as well as the presence of tumor infiltrating lymphocytes (TILs). It is currently unknown whether tumors associated with neurofibromatosis types 1 and 2 (NF1 and NF2) express PD-L1. We performed immunohistochemistry for PD-L1 (clones SP142 and E1L3N), CD3, CD20, CD8, and CD68 in NF1-related tumors (ten dermal and six plexiform neurofibromas) and NF2-related tumors (ten meningiomas and ten schwannomas) using archival formalin-fixed paraffin-embedded tissues...
February 9, 2018: Journal of Neuro-oncology
https://www.readbyqxmd.com/read/29409029/the-characteristics-of-76-atypical-neurofibromas-as-precursors-to-neurofibromatosis-1-associated-malignant-peripheral-nerve-sheath-tumors
#18
Christine S Higham, Eva Dombi, Aljosja Rogiers, Sucharita Bhaumik, Steven Pans, Steve E J Connor, Markku Miettinen, Raf Sciot, Roberto Tirabosco, Hilde Brems, Andrea Baldwin, Eric Legius, Brigitte C Widemann, Rosalie E Ferner
Background: Neurofibromatosis 1 (NF1) leads to the development of benign and malignant peripheral nerve sheath tumors (MPNST). MPNST have been described to develop in pre-existing 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 three institutions...
February 2, 2018: Neuro-oncology
https://www.readbyqxmd.com/read/29382000/endovascular-management-of-renal-artery-aneurysms-induced-by-neurofibromatosis-type-1-a-case-report
#19
Bihui Zhang, Yinghua Zou, Min Yang, Guochen Niu
RATIONALE: Neurofibromatosis type 1 (NF-1) is an autosomal dominant disorder characterized by cafe au lait macules and neurofibromatosis. Renal artery aneurysms are relatively uncommon. Endovascular techniques are effective in treating renal aneurysms but successful cases are rarely reported in NF-1 adults. PATIENT CONCERNS: The patient was one 23-year-old female presented with hypertension, multiple café-au-lait spots ≥15 mm, and plexiform neurofibroma. Renal artery aneurysms were found by ultrasound...
November 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/29356283/intrahepatic-plexiform-neurofibroma-with-neurofibromatosis-type-1
#20
Yoshiki Kusama, Yo Matsui
No abstract text is available yet for this article.
January 2018: Pediatrics International: Official Journal of the Japan Pediatric Society
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