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https://www.readbyqxmd.com/read/28532923/radiation-induced-schwannomas-and-neurofibromas-a-systematic-review
#1
REVIEW
Ryuya Yamanaka, Azusa Hayano
OBJECTIVE: Radiation-induced benign peripheral nerve sheath tumors are uncommon late complications of irradiation. We conducted the largest systematic review of individual patient data. METHODS: We performed a systematic search of PubMed databases, and compiled a comprehensive literature review. Kaplan-Meier analysis was used to investigate survival, and statistical significance was assessed using a log-rank test. RESULTS: We analyzed 40 cases of radiation-induced benign peripheral nerve sheath tumors...
May 19, 2017: World Neurosurgery
https://www.readbyqxmd.com/read/28530162/radiation-induced-glandular-malignant-peripheral-nerve-sheath-tumor
#2
Ivy John, David L Bartlett, Uma N M Rao
Malignant peripheral nerve sheath tumors (MPNSTs) are aggressive soft-tissue tumors. They can occur in patients with neurofibromatosis type-1 (NF-1) or as sporadic tumors. Only 10% of MPNSTs are radiation induced. Divergent differentiation in MPNSTs can occur in 15% of cases and may include cartilage, bone, skeletal muscle, blood vessels, and very rarely well-formed glands, the latter typically described in NF-1-associated MPNSTs. We report an exceedingly rare case of radiation induced glandular MPNST arising in a neurofibroma of the femoral nerve in a patient previously irradiated for endometrial carcinoma...
May 1, 2017: International Journal of Surgical Pathology
https://www.readbyqxmd.com/read/28526004/hybrid-peripheral-nerve-sheath-tumors-report-of-five-cases-and-detailed-review-of-literature
#3
Nasir Ud Din, Zubair Ahmad, Jamshid Abdul-Ghafar, Rashida Ahmed
BACKGROUND: Hybrid peripheral nerve sheath tumors (PNSTs) have been recognized recently and were first included in the 4th edition of World Health Organization (WHO) Classification of Tumors of Soft tissue and Bone, published in 2013. These tumors show combined features of more than one type of conventional benign peripheral nerve sheath tumors. The most common combinations are those of schwannoma/perineurioma followed by combinations of neurofibroma/schwannoma and neurofibroma/perineurioma...
May 19, 2017: BMC Cancer
https://www.readbyqxmd.com/read/28524060/high-frequency-ultrasound-imaging-of-cutaneous-neurofibroma-in-patients-with-neurofibromatosis-type-i
#4
Delphine Raffin, Julia Zaragoza, Gabriella Georgescou, Youssef Mourtada, Annabel Maruani, Frédéric Ossant, Frédéric Patat, Loïc Vaillant, Laurent Machet
Neurofibromas (NFs) are benign tumours arising from a nerve sheath, which are present in nearly all patients with neurofibromatosis type 1 (NF1). High-frequency ultrasound (HFU) systems, using frequencies over 20 MHz, were developed to improve visualization of skin tumours by means of increased resolution. To describe NFs by using HFU in patients with NF1. Anonymized HFU (25-MHz) images of NFs were randomized. Initially, two dermatologist investigators, with experience in HFU imaging of the skin, together described the ultrasound images and established eight criteria for NFs...
May 19, 2017: European Journal of Dermatology: EJD
https://www.readbyqxmd.com/read/28523880/halolike-phenomenon-around-a-caf%C3%A3-au-lait-spot-superimposed-on-a-mongolian-spot
#5
Iria Neri, Martina Lambertini, Vera Tengattini, Beatrice Rivalta, Annalisa Patrizi
An 8-month-old Caucasian infant with neurofibromatosis type 1 presented with a congenital plexiform neurofibroma and multiple café au lait spots. A pale area surrounded one of the café au lait spots located on the left gluteus in the area of dermal melanocytosis. This halolike phenomenon results from the disappearance of the Mongolian spot around the café au lait spots, revealing normal pigmented skin. This sign has been described rarely in the literature and the pathogenic mechanism is unclear.
May 2017: Pediatric Dermatology
https://www.readbyqxmd.com/read/28502567/myelopathy-secondary-to-a-spinal-cord-compression-induced-by-c2-nerve-tumors-during-head-rotation
#6
P Finet, C Raftopoulos
INTRODUCTION: It is well known that nerve root tumors developing in the spinal canal can be responsible for spinal cord compression leading to myelopathy and neurological deficits. We report the case of a patient with bilateral C2 neurofibromas presenting with a myelopathy of the upper cervical spine with no spinal cord compression on standard magnetic resonance imaging. The spinal cord compression occurred between the cervical neurofibromas only during head rotation. CASE REPORT: A patient with bilateral neurofibromas of the C2 nerve roots showed a progressive neurological deterioration with an intramedullary MRI hypersignal without visible compression...
May 11, 2017: Neuro-Chirurgie
https://www.readbyqxmd.com/read/28502478/the-cellular-retinoic-acid-binding-protein-2-promotes-survival-of-malignant-peripheral-nerve-sheath-tumor-cells
#7
Susan Fischer-Huchzermeyer, Anna Dombrowski, Christian Hagel, Victor Felix Mautner, Jens Schittenhelm, Anja Harder
Malignant peripheral nerve sheath tumors (MPNSTs) are aggressive neoplasms that commonly occur in patients with neurofibromatosis type 1 (NF1). Effective chemotherapy is not available. To characterize a therapeutic target for treatment, we investigated the role of cellular retinoic acid binding protein 2 (CRABP2) in MPNST in vitro. CRABP2 is a transcriptional co-activator of retinoic acid signaling. Although overexpression of CRABP2 is described in several cancers, it has not yet been studied in MPNSTs. We investigated CRABP2 expression in cultured Schwann cells and formalin-fixed, paraffin-embedded specimens of human peripheral nerve sheath tumors...
May 11, 2017: American Journal of Pathology
https://www.readbyqxmd.com/read/28491213/-the-diffuse-plexiform-neurofibroma-of-the-left-thigh-in-a-patient-aged-78-years-in-dermatological-environment-in-bamako
#8
Békaye Traoré, Youssouf Fofana
No abstract text is available yet for this article.
2017: Pan African Medical Journal
https://www.readbyqxmd.com/read/28489286/sustained-activation-of-erk1-2-mapk-in-schwann-cells-causes-corneal-neurofibroma
#9
Paola Bargagna-Mohan, Akihiro Ishii, Ling Lei, Daniel Sheehy, Saagar Pandit, Grace Chan, Rashmi Bansal, Royce Mohan
Recent studies have shown that constitutive activation of extracellular signal-regulated kinases 1 and 2 (ERK1/2) in Schwann cells (SCs) increases myelin thickness in transgenic mice. In this secondary analysis, we report that these transgenic mice develop a postnatal corneal neurofibroma with the loss of corneal transparency by age six months. We show that expansion of non-myelinating SCs, under the control of activated ERK1/2, also drive myofibroblast differentiation that derives from both SC precursors and resident corneal keratocytes...
May 10, 2017: Journal of Neuroscience Research
https://www.readbyqxmd.com/read/28488063/subgemmal-neurogenous-plaque-of-the-tongue-a-report-of-three-cases
#10
Amanda Katarinny Goes Gonzaga, Deborah Gondim Lambert Moreira, Dáurea Adília Cóbe Sena, Maria Luiza Diniz de Sousa Lopes, Lélia Batista de Souza, Lélia Maria Guedes Queiroz
Subgemmal neurogenous plaque (SNP) is a biphasic neural structure associated with the taste buds. Clinically, SNP usually presents as an asymptomatic, normally colored, papule located in the posterior lateral border of the tongue. Accurate diagnosis is based only on histopathological examination, which shows a superficial neurofibroma-like pattern and a neuroma-like in the deep zone. Appropriate recognition of clinical and morphological aspects of SNPs can avoid their misdiagnosis as neural neoplasms. We report three cases of SNP with detailed clinical, histopathological, and immunohistochemical features...
May 10, 2017: Oral and Maxillofacial Surgery
https://www.readbyqxmd.com/read/28484547/solitary-giant-diffuse-neurofibroma-of-the-scalp-with-calvarial-defect
#11
Sanjeev Kumar, Pramod Chaurasia, Daljit Singh, Vineeta V Batra, Rajendra Aher
Neurofibromas of the scalp can have protean presentations. Most of the swellings are small, solitary and are easily diagnosed clinically. Diffuse swellings on the other hand are rare and are commonly seen in adults. The skull defects with these swellings are also rarely reported in the absence of neurofibromatosis. There is only one report of child having diffuse neurofibroma with skull defect. We report a second case in literature in a child with progressive, painful, diffuse neurofibroma along with calvarial defect...
April 2017: Asian Journal of Neurosurgery
https://www.readbyqxmd.com/read/28483239/the-efficacy-and-safety-of-the-flexible-fiber-co2-laser-delivery-system-in-the-endoscopic-management-of-pediatric-airway-problems-our-long-term-experience
#12
Gi Soo Lee, Alexandra Irace, Reza Rahbar
OBJECTIVE: To report the use of flexible fiber CO2 laser in the endoscopic management of pediatric airway cases. METHODS: A retrospective review was conducted of patients who underwent CO2 laser-assisted airway procedures between September 2007 and January 2014 at a tertiary pediatric hospital. RESULTS: Sixty-eight patients underwent 80 procedures utilizing flexible fiber CO2 laser. Procedures included supraglottoplasty (n = 32), laryngeal cleft repair (type I [n = 10], type II [n = 7], type III [n = 6]), suprastomal granuloma excision (n = 6), cordotomy (n = 4), laryngeal neurofibroma excision (n = 4), laryngeal granulomatous mass excision (n = 1), subglottic stenosis excision (n = 6), division of glottic web (n = 2), subglottic cyst excision (n = 1), and supraglottic biopsy (n = 1)...
June 2017: International Journal of Pediatric Otorhinolaryngology
https://www.readbyqxmd.com/read/28469964/comprehensive-pharmacological-profiling-of-neurofibromatosis-cell-lines
#13
Jianman Guo, Michael R Grovola, Hong Xie, Grace E Coggins, Patrick Duggan, Rukhsana Hasan, Jiale Huang, Danny W Lin, Claire Song, Gabriela M Witek, Simon Berritt, David C Schultz, Jeffrey Field
Patients with Neurofibromatosis type 1 (NF1) and Neurofibromatosis type 2 (NF2) are predisposed to tumors of the nervous system. NF1 patients predominantly develop neurofibromas, and Malignant Peripheral Nerve Sheath Tumors (MPNST) while NF2 patients develop schwannomas and meningiomas. Here we quantified the drug sensitivities of NF1 and NF2 tumor cell lines in a high throughput platform. The platform contained a comprehensive collection of inhibitors of MEK, RAF, RAS, farnesyl transferase, PAK and ERK, representative drugs against many other cancer pathways including Wnt, Hedgehog, p53, EGF, HDAC, as well as classical cytotoxic agents recommended for treating MPNST, such as doxorubicin and etoposide...
2017: American Journal of Cancer Research
https://www.readbyqxmd.com/read/28468226/characteristics-of-pediatric-cheek-mass
#14
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...
May 2017: Journal of Craniofacial Surgery
https://www.readbyqxmd.com/read/28456002/multiple-spinal-nerve-enlargement-and-sos1-mutation-further-evidence-of-overlap-between-neurofibromatosis-type-1-and-noonan-phenotype
#15
Claudia Santoro, Teresa Giugliano, Mariarosa Anna Beatrice Melone, Mario Cirillo, Carla Schettino, Pia Bernardo, Giovanni Cirillo, Silverio Perrotta, Giulio Piluso
Neurofibromatosis type 1 (NF1) has long been considered a well-defined, recognizable monogenic disorder, with neurofibromas constituting a pathognomonic sign. This dogma has been challenged by recent descriptions of patients with enlarged nerves or paraspinal tumors, suggesting that neurogenic tumors and hypertrophic neuropathy may be a complication of Noonan syndrome with multiple lentigines or RASopathy phenotype. We describe a 15-year-old boy, whose mother previously received clinical diagnosis of NF1 due to presence of bilateral cervical and lumbar spinal lesions resembling plexiform neurofibromas and features suggestive of Noonan syndrome...
April 29, 2017: Clinical Genetics
https://www.readbyqxmd.com/read/28454108/telomere-erosion-in-nf1-tumorigenesis
#16
Rhiannon E Jones, Julia W Grimstead, Ashni Sedani, Duncan Baird, Meena Upadhyaya
Neurofibromatosis type 1 (NF1; MIM# 162200) is a familial cancer syndrome that affects 1 in 3,500 individuals worldwide and is inherited in an autosomal dominant fashion. Malignant Peripheral Nerve Sheath Tumors (MPNSTs) represent a significant cause of morbidity and mortality in NF1 and currently there is no treatment or definite prognostic biomarkers for these tumors. Telomere shortening has been documented in numerous tumor types. Short dysfunctional telomeres are capable of fusion and it is considered that the ensuing genomic instability may facilitate clonal evolution and the progression to malignancy...
April 9, 2017: Oncotarget
https://www.readbyqxmd.com/read/28448720/mosaic-neurofibromatosis-type-1-in-children-a-single-institution-experience
#17
Irene Lara-Corrales, Mitra Moazzami, Maria Teresa García-Romero, Elena Pope, Patricia Parkin, Andrea Shugar, Peter Kannu
BACKGROUND: Neurofibromatosis type 1 (NF1) is a neurocutaneous disorder caused by loss-of-function mutation in the NF1 gene. Segmental or mosaic NF1 (MNF) is an uncommon presentation of the NF1 result of postzygotic mutations that present with subtle localised clinical findings. OBJECTIVES: Our study's objectives were to describe the clinical characteristics of children with MNF. METHODS: We conducted a cross-sectional study of children diagnosed with MNF at the Hospital for Sick Children in Toronto, Canada, from January 1992 to September 2012...
April 1, 2017: Journal of Cutaneous Medicine and Surgery
https://www.readbyqxmd.com/read/28442864/multiple-plexiform-neurofibroma-of-the-hand-misdiagnosed-as-ganglion-cyst
#18
Daniel Dreyfuss, Ido Stahl, Edward Calif
No abstract text is available yet for this article.
April 2017: Journal of Hand and Microsurgery
https://www.readbyqxmd.com/read/28429332/the-effect-of-post-injection-18-f-fdg-pet-scanning-time-on-texture-analysis-of-peripheral-nerve-sheath-tumours-in-neurofibromatosis-1
#19
Eitan Lovat, Musib Siddique, Vicky Goh, Rosalie E Ferner, Gary J R Cook, Victoria S Warbey
BACKGROUND: Texture features are being increasingly evaluated in (18)F-fluorodeoxyglucose positron emission tomography ((18)F-FDG PET) as adjunctive imaging biomarkers in a number of different cancers. Whilst studies have reported repeatability between scans, there have been no studies that have specifically investigated the effect that the time of acquisition post-injection of (18)F-FDG has on texture features. The aim of this study was to investigate if texture features change between scans performed at different time points post-injection...
December 2017: EJNMMI Research
https://www.readbyqxmd.com/read/28413392/t-cell-lymphoma-in-a-patient-with-neurofibromatosis-type-1-and-aids
#20
Izana Junqueira de Castro, Esther Botelho Soares da Silva, Talita Rezende Dos Santos, Amanda Barroso de Freitas, Inara Junqueira de Castro, Alessandra Santos Portela, Marilza Campos de Magalhães, Karina Lebeis Pires, Guilherme Almeida Rosa da Silva, Marcelo Costa Velho Mendes de Azevedo
Neurofibromatosis type 1 (NF1) and AIDS are risk factors for the development of malignant neoplasms, including hematological malignancies, such as non-Hodgkin lymphoma. NF1 is an autosomal dominant disease that primarily manifests as café-au-lait spots, dermal neurofibromas, axillary and/or inguinal ephelides or freckles, plexiform neurofibromas, Lisch nodules, and bone deformities. In this report, we present a 38-year-old female patient with NF1 from childhood and AIDS who developed peripheral T-cell lymphoma with good response to chemotherapeutic treatment...
January 2017: Case Reports in Oncology
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