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Plexiform neurofibroma [PDF]

Cleveland Clinic Journal of Medicine, 2016
Plexiform neurofibromas represent an uncommon variant (30%) of neurofibromatosis type 1 (NF-1) in which neurofibromas arise from multiple nerves as bulging and deforming masses involving also connective tissue and skin folds.We report a rare case of a 30-year-old man who presented with a progressive facial deformity that began in early childhood.
Tchernev, Georgi, Chokoeva, Anastasiya Atanasova, Patterson, James W., Bakardzhiev, Ilko, Wollina, Uwe, Tana, Claudio   +5 more
  +9 more sources

Imaging diagnosis of plexiform neurofibroma- unravelling the confounding features: A report of two cases

Radiology Case Reports, 2021
Peripheral nerve sheath tumors such as neurofibroma, comprise 5% of all benign soft tissue tumors and usually occur due to an underlying neurofibromatosis.
Dr. Shabnam Bhandari Grover, MD, DNBE, FICR, MNAMS, MRCR, FICMU, Dr. Rohit Kundra, MBBS, Dr. Hemal Grover, MBBS, MD, Dr. Vishal Gupta, MBBS, MD, Dr. Rishab Gupta, MBBS   +4 more
doaj   +2 more sources

Plexiform Neurofibroma: A Case Report

SAS Journal of Surgery, 2021
Plexiform neurofibroma is a characteristic lesion of Von's disease Recklinghausen. Currently, surgery is the only effective treatment. This is difficult form due to its hemorrhagic nature and the pervasive appearance of the lesions.
Dehhaze Adil, Diher Issam, Ahmar-Rass Nour El Imane, Daghouri Nada- Imane, Taybi Otmane, Labbaci Rim   +5 more
semanticscholar   +2 more sources

Exuberant Plexiform Neurofibroma

Acta Médica Portuguesa, 2023
N/a.
Catarina Correia, Miguel Reis, Paulo Filipe   +2 more
doaj   +3 more sources

A Fibroblast-Derived Secretome Stimulates the Growth and Invasiveness of 3D Plexiform Neurofibroma Spheroids. [PDF]

Cancers (Basel)
Simple Summary Plexiform neurofibromas in neurofibromatosis type 1 (pNF1), characterized by aggressive growth and local invasiveness, are comprised primarily of Schwann cell-derived tumor cells (Nf1−/−) and the tumor microenvironment (TME).
Ji K, Schwenkel GJ, Mattingly RR, Sundararaghavan HG, Zhang ZG, Chopp M.   +5 more
europepmc   +2 more sources

Safety and efficacy of selumetinib in pediatric and adult patients with neurofibromatosis type 1 and plexiform neurofibroma. [PDF]

Neuro Oncol
Background The MEK inhibitor, selumetinib, reduces plexiform neurofibroma (PN) in pediatric patients with neurofibromatosis type 1 (NF1). Its safety and efficacy in adults with PN and effectiveness in other NF1 manifestations (eg, neurocognitive function,
Kim H, Yoon HM, Kim EK, Ra YS, Kim HW, Yum MS, Kim MJ, Baek JS, Sung YS, Lee SM, Lim HS, Lee BJ, Lim HT, Kim D, Yoon J, Bae H, Hwang S, Choi YH, Kim KA, Choi IH, Lee SW, Park SJ, Lee BH.   +22 more
europepmc   +2 more sources

Excision of Solitary Non-syndromic Oral Plexiform Neurofibroma Utilizing a Diode Laser: A Case Report. [PDF]

Cureus
Plexiform neurofibroma is a benign peripheral nerve sheath tumor known to be pathognomonic for neurofibromatosis type 1. However, solitary plexiform neurofibroma in the oral cavity is extremely rare.
Al-Ali MM, Al-Otaibi LM, Al-Bakr I.
europepmc   +2 more sources

A Rare Tumor in the Neck of a Child: Plexiform Neurofibroma

Turkish Archives of Otorhinolaryngology, 2021
Plexiform neurofibroma represents an uncommon variant of neurofibromatosis type 1, constituting only 5%–30% of all cases. Plexiform neurofibroma is usually diagnosed during childhood and arises from multiple nerves, manifesting as bulging and deforming ...
Siti Sarah Mohd Ramli, Goh Bee See, Faizah Mohd Zaki, Suria Hayati Md Pauzi, Mohamad Fariz Bin Amri   +4 more
doaj   +2 more sources

ReNeu: A Pivotal, Phase IIb Trial of Mirdametinib in Adults and Children With Symptomatic Neurofibromatosis Type 1-Associated Plexiform Neurofibroma. [PDF]

J Clin Oncol
PURPOSE Pharmacologic therapies for neurofibromatosis type 1-associated plexiform neurofibromas (NF1-PNs) are limited; currently, none are US Food and Drug Administration–approved for adults.
Moertel CL, Hirbe AC, Shuhaiber HH, Bielamowicz K, Sidhu A, Viskochil D, Weber MD, Lokku A, Smith LM, Foreman NK, Hajjar FM, McNall-Knapp RY, Weintraub L, Antony R, Franson AT, Meade J, Schiff D, Walbert T, Ambady P, Bota DA, Campen CJ, Kaur G, Klesse LJ, Maraka S, Moots PL, Nevel K, Bornhorst M, Aguilar-Bonilla A, Chagnon S, Dalvi N, Gupta P, Khatib Z, Metrock LK, Nghiemphu PL, Roberts RD, Robison NJ, Sadighi Z, Stapleton S, Babovic-Vuksanovic D, Gershon TR, ReNeu Trial Investigators, ReNeu Study Investigators.   +41 more
europepmc   +2 more sources

Revisiting the NPcis mouse model: A new tool to model plexiform neurofibroma. [PDF]

PLoS ONE
Neurofibromatosis Type I (NF1) is a rare genetic disorder. NF1 patients frequently develop a benign tumor in peripheral nerve plexuses called plexiform neurofibroma.
Camille Plante, Teddy Mohamad, Dhanushka Hewa Bostanthirige, Michel Renaud, Harsimran Sidhu, Michel ElChoueiry, Jean-Paul Sabo Vatasescu, Mikael Poirier, Sameh Geha, Jean-Philippe Brosseau   +9 more
doaj   +2 more sources